List of studies that reviewed herpes zoster infection and motor paresis of only upper limbs.
Abstract
Segmental zoster paresis (SZP) is a rare complication of varicella zoster infection that occurs due to the spread of the infection from the posterior horn of spinal cord to the anterior horn and the motor nerve root. As recognizing segmental zoster paresis is important in the differential diagnosis of muscle weakness of other origin, information about demographic (gender and age), clinical presentation, diagnosis, treatment, and course about published patients with SZP was extracted from PubMed database. SZP is classified into several categories: paresis of upper extremity, lower limb involvement, diaphragmatic involvement, and abdomen involvement. Published experiences have shown that clinical course and electromyoneurography of paretic muscle are the most important in the diagnosis; physical therapy is the most common therapy in these patients and their prognosis is generally good except diaphragmatic paresis, where there is no significant recovery in most number of patients.
Keywords
- segmental paresis
- varicella zoster
- clinical presentation
- diagnosis
- treatment
- course
1. Introduction
Herpes zoster (HZ) is an infection of dorsal root ganglion characterized by a painful cutaneous rash. It is believed that reactivated varicella zoster virus (VZV) migrate from dorsal root ganglion in retrograde direction along the sensory nerve to the skin, where it makes the characteristic dermatomal rash [1].
The initial infection caused by varicella or chickenpox occurs during childhood. After that, the virus then exists as a latent infection of sensory ganglia from which it may reactivate many years later and cause herpes zoster.
The virus can be recovered from skin lesion and can cause an inflammatory reaction in sensory ganglion, dorsal root, and posterior horn of spinal cord [2].
Postherpetic neuralgia is the most common complication associated with extracranial HZ. Pain persisting 90 days or longer after the onset of the shingles rash is called postherpetic neuralgia [3, 4]. Postherpetic neuralgia usually remits spontaneously, but some patients could have pain for all their life.
Beside postherpetic neuralgia, segmental herpetic paresis is another complication of HZ, and they often occur together.
Flaccid muscle paralysis rarely occurs due to the spread of the infection from the posterior horn of spinal cord to the anterior horn and the motor nerve root (Figure 1) when weakness generally corresponding to the dermatomes in which cutaneous lesions develop [5].
This finding supports the enhancement of spinal nerve roots on MRI that was clinically symptomatic [6], attributed to autoimmune inflammation or vasculitis [7]; brachial plexus inflammation; and myelin destruction with intact axons found postmortem [8].
The association of muscle paralysis and herpes zoster was first reported by Broadbent in 1866. When he described the case to zoster described as “frozen shoulder” [9], it is noted rarely between 0.5% [10] and 0.8% of segmental motor paralysis between all patients with HZ [11].
In patients with dissociation between motor and dermatomal involvement, a possible explanation could be viral spread to anterior roots without corresponding axonal transport through the sensory nerves [6, 12].
Some patients may have zoster infection without vesicular eruptions, which is called “zoster sine herpete.” This diagnose is safer by a rise VZV antibodies. These patients may have the same neurologic manifestations, including muscular paralysis [13].
As there are no certain standards regarding diagnosis and treatment of segment zoster paresis, we have listed in this paper experiences from literature related to the topic.
The aim of this study was to summarize the experiences related to its clinical manifestation, applied diagnostics, treatment, and patient outcome.
2. Methods
We searched the PubMed database for literature on herpes zoster infection and extracranial motor paresis in adults (last search on September 2019).The search was limited to full-length articles written in English and a study population that included adults aged 18 years and older. A combination of the following search terms was used “herpes zoster, paresis, complications.” As a result, 74 articles were retrieved.
The reference list was also searched for relevant manuscripts not retrieved from PubMed.
Studies included in the final review met the following criteria: (1) infection with herpes zoster virus, (2) motor paresis, and (3) adult study population.
They encompass original articles, technical reports, clinical observations, and single case reports.
3. Results
3.1 Data extraction and synthesis
For comparison across the reports, the sample size was extracted along with demographic information (gender and age), clinical presentation, laboratory confirmation of herpes zoster, electromyographic (EMG) findings, imagining, treatment, and course.
For clarity, segmental zoster paresis is classified into several categories: paresis of upper extremity, lower limb involvement, diaphragmatic involvement, and abdomen involvement.
Upper extremity involvement: In 37 previously published papers about patients with segmental zoster paresis of arms, there are 19 papers that are processed only as arms paresis (Table 1), while the rest of the 18 papers describe patients with segmental zoster paresis of arms and legs (Table 2). They include a total of 101 patients with segmental paresis of arms with a mean age of 68.56 ± 11.97 and with 55 women and 45 men and one patient who did not specify gender.
Literature | Demographics, medical history | Subjective complaints | Neurological impairments | EMG findings | Other tests | Treatment | Course/Outcome | |
---|---|---|---|---|---|---|---|---|
[14] | 58-year old. female | Rash in left shoulder, lateral part of left arm and hand; pain in neck and interscapular region | Weakness of left shoulder, wasting of left | Exercises | After 2 months, power and mass of M. deltoideus increase | |||
[15] | #1: 82-year old female | Pain and rash in right shoulder and upper arm | Weakness in proximal arm mild elbow flexion contracture | Denervation in deltoid, biceps, supraspinatus; polyphasia and decreased number of MUAP | Moist hot packs, ultrasound, exercise | Incomplete recovery | ||
#2: 72-year old female, breast carcinoma with mastectomy | Weakness in biceps, deltoid, wrist flexors/ extensors | Denervation in almost all arm muscles; ulnar and median conduction velocities reduced | Not reported | B12 injections, heat, paraffin baths, exercises | Incomplete recovery | |||
#3: 65-year old female with knee amputation for vascular disease, chronic leukemia | Pain and rash in right arm | Weakness in deltoid, biceps, triceps, wrist extensors, thenar muscles | Marked denervation | Not reported | Heat, electrical stimulation, strengthening exercises | Functional recovery within 6 weeks | ||
#4: 62-year old female, rheumatoid arthritis | Pain and rash in right arm | Weakness in deltoid, infraspinatus, supraspinatus, biceps | Denervation; polyphasic MUAP | Not reported | Heat, electrical stimulationsling | Some recovery of strength and less pain | ||
#5: 71-year old female | Pain and rash in right shoulder and forearm | Weakness in deltoid, biceps, wrist flexors/extensors | Denervation in C7–8/T1 muscles | Not reported | Codeine, whirlpool, paraffin baths | Nearly complete recovery after unknown time period | ||
[16] | #1: 70-year old female | Pain in left shoulder with radiation to arm, hyperesthesia in shoulder and neck; rash in C4–5 dermatomes | Weakness in arm; reflexes absent in biceps, supinator | Denervation in deltoid and biceps muscles | Unremarkable chest X-ray and laboratory work-up | Physical therapy | Full recovery after 3 weeks | |
#2. 60-year old man, lymphatic leukemia | Rash and hypersensitivity in right C5 dermatome after weeks of coughing; enlarged liver | Not reported | Chest X-ray: elevated right hemidiaphragm, paralysis confirmed on fluoroscopy | Complete recovery after 1 month | ||||
[17] | 25-year old female | Rash over shoulder | Weakness in deltoid | Not reported | X-ray: shoulder subluxation | Not reported | Full recovery after unspecified number of months | |
[18] | 59-year old man, diabetes, mild hypertension | Pain and rash in left C7 dermatome; hyperalgesia in inner half of left hand | Weakness in whole arm; marked atrophy in almost all arm muscles, reflexes diminished | Fibrillations, reduced interference pattern in atrophied muscles; conduction velocities normal | X-ray: spine normal; CSF: normal | Not reported | Unknown | |
[13] | #1. 81-year old man | Pain in left arm and left lateral chest wall; rash in C5–7 dermatomes | Complete flaccid weakness of left arm | Not reported | Not reported | Physical therapy | Complete recovery after 3 months | |
#2. 75-year old female, diabetes | Pain and rash in right shoulder and over back of the head | Marked weakness in deltoid, biceps, infraspinatus; reflexes absent in biceps | Denervation in infraspinatus, deltoid, biceps; conduction velocities normal | Not reported | Not reported | Minimal recovery after 2 months | ||
[5] | 64-year old female | Pain and rash in left inner arm and fingers after 4 months of an episode of shingles | Severe weakness in wrist and hand intrinsic muscles | Lesion of anterior interosseus nerve | Surgical exploration‑no compression | Not reported | No recovery after 8 months | |
[19] | 73-year old man, left biceps rupture 10 yrs. earlier with full recovery | Pain in left shoulder; rash over biceps, brachioradialis | Moderate atrophy of deltoid; weakness in biceps and infraspinatus; reflexes diminished in biceps, brachioradialis muscle | Acute motor axonal lesion in C5–6 dermatomes | Neck MRI: no spinal cord, root compression | Acyclovir, opioid analgesics, amitriptyline, physical therapy, home exercises | Slight deltoid weakness after 2 years | |
[7] | 72-year old man with oral prednisolone due to myasthenia gravis | Rash in left C3–5 dermatomes | 10 days before the rash, weakness of the left arm limited to muscles controlled by the C5 myeloma | T2-weighted MRI | ||||
[20] | 73-year old man | Right shoulder pain and herpes zoster eruptions over the C5 dermatome | Two days later, he found it impossible to lift up the right arm | |||||
[21] | A 48-year old female | Rash and vesicles over her left C5–7 dermatomes | Weakness in the left deltoids and biceps muscles, and a diminished left biceps reflex; dyspnea with paradoxical abdominal wall movement | Chest CT normal, MRI-hyperintensity in the left anterolateral aspect of the spinal cord at C5 level | 1000 mg valacyclovir orally three times daily for 7 days. | The patient’s pain resolved three months later, and the patient underwent outpatient follow-up for 2 years without further complications | ||
[22] | A 72-year old female | Burning pain and rash in the left shoulder and its weakness | Hyperalgesia skin in left C4–T2 dermatomes, left shoulder abduction weakness | EMG-denervation in left biceps, deltoid, brachioradialis and C5–6 paraspinal muscles; sensory and motor nerve-conduction, and somatosensory evoked potentials are normal | MRI of cervical spine-degenerative changes with foraminal stenosis at C3–T1 | 750 mg/d famciclovir per os, cervical epidural blocks with 20 mg triamcinolone twice during 4 weeks, 450 mg pregabalin and 10 mg nortriptyline, physiotherapy | After 2 months, pain and weakness were significantly retracted | |
[23] | 88-year old man with corticosteroids in therapy because of polymyalgia rheumatica | Rash in the right C3–5 dermatome | Paresis in the right shoulder 2 days after the rash | Oral valaciclovir, acyclovir iv after paresis and methylprednisolone | Paresis has gradually improved | |||
[24] | 59-year old-female with carcinoma mammae dextri and lung metastasis, chemotherapy and radiotherapy | Pain and rash in whole right arm and scapula | Patient was able to adduct shoulder, flexor and extensor wrist and passively extend elbow | EMNG-low amplitude action potentials in left superior radial and median, SNAPs and MUAPs in left deltoid muscle | Positive direct fluorescent antibody test for VZV, neck MRI-C4-C5-C6 levels protruded disk | Acyclovir 10 mg/8 hours i.v. for 7 days | Motor activities recovered after 5 months with physiotherapy but postherpetic neuritis had a poor decrement |
Literature | Demographics, medical history | Subjective complaints | Neurological impairments | EMG findings | Other tests | Treatment | Course/outcome |
---|---|---|---|---|---|---|---|
[25] | #1: 89-year old man, transient facial weakness 1 year earlier, conjunctivitis 6 months earlier | Pain and rash in right thigh and knee | Weakness and atrophy in right thigh, decreased sensation; reflexes diminished in knees and ankles | Denervation in | X-ray: degenerative changes in LS spine | Physical therapy, galvanic electrical stimulation | Incomplete recovery after 3 months |
#2. 44-year old female | Pain and rash over right distal arm in C7–8 dermatome | Weakness of proximal and distal muscles of right arm | Fibrillations in right upper arm in C8 myotome | Not reported | Physical therapy | Incomplete recovery | |
#3. 74-year old woman | Pain and rash in T12-L1 dermatomes | Bulging of right abdomen and decreased muscle tone | Not reported | Not reported | Corset | Not reported | |
#4. 58-year old woman, diabetes | Pain in left leg and rash over S1–2 dermatomes | Weakness in left knee extensors, ankle dorsiflexors and plantar flexors; reflexes absent in left leg | Not reported | Not reported | Not reported | Able to walk after 5 weeks | |
[26] | #1. 71-year old female | Rash along right ulnar border of forearm and hand | Weakness in forearm and hand muscles | Not reported | Not reported | Physical therapy | Return of strength but impaired fine movements |
#2. 76-year old female | Rash over right shoulder and arm | Weakness in complete right arm, reflex absent in biceps | Not reported | Not reported | Physical therapy | Full recovery except in deltoid muscle, neuralgia over 3 years | |
#3. 83-year old female | Pain and rash over right shoulder | Weakness in deltoid, biceps; reflexes absent in biceps | Not reported | Not reported | Hydrotherapy, exercises | Weakness in deltoid and biceps; neuralgia over 8 months | |
#4. 85-year old woman | Pain and rash in left shoulder | Weakness in deltoid, supraspinatus biceps; reflex diminished in triceps | Not reported | Not reported | Physical therapy | Incomplete recovery, residual neuralgia died after 7 months | |
#5. 73-year old man | Pain and rash in right shoulder and in upper arm | Weakness in shoulder with deltoid fasciculations | Not reported | Not reported | Intensive physical therapy | Full recovery after 7 months | |
#6. 74-year old woman | Rash over left thigh | Weakness of hip flexors, adductors, knee extensors | Not reported | Not reported | Not reported | Full recovery after 4 months | |
#7. 73-year old man | Rash over left shoulder and upper third of arm | Weakness in left arm | Not reported | Not reported | Hydrotherapy, physical therapy | Full recovery | |
#8. 81-year old female | Pain and rash over C1–7 dermatomes | Weakness of right serratus anterior, scapula winging | Not reported | Not reported | Intensive physiotherapy | Full recovery in arm, residual pain after 5 months | |
#9. 75-year old female | Rash of outer part of right arm | Weakness in shoulder flexors and abductors, and in elbow flexors | Not reported | Not reported | Intensive physiotherapy | Full recovery after 5 months; some pain persisted | |
[27] | #1. 73-year old female | Rash in right C4–7 dermatomes; segmental severe paresis in C6, 7, 8 | Denervation | CSF normal; myelogram normal | Supportive treatment | Full recovery after 7 months | |
#2. 78-year old female, diabetes | Rash in right C5–7 dermatomes | Moderate weakness in C5–7 myotomes | Denervation | CSF normal; myelogram normal | Supportive treatment | Full recovery after 9 months | |
#3. 67-year old man | Rash in left C5–6 dermatomes | Moderate weakness in C5–6 myotomes | Denervation | Not reported | Supportive treatment | Full recovery after 3 months | |
[28] | #1. 84-year old man | Pain and rash in right L2–3 dermatomes; impaired sensation in L2–3 dermatomes | Weakness in quadriceps and hip adductors, and flexors; reflexes absent in knee | Fibrillations in hip flexors, vastus medialis | Not reported | Not reported | No recovery, died 6 years later |
# 2. 66-year old man | Rash and pain in left shoulder and arm in C5 dermatome | Weakness in deltoid and spinatus muscles | Fibrillation, polyphasia and reduced interference patterns MUAP | Not reported | Not reported | Complete recovery after 4 months | |
#3. 85-year old female | Rash, pain and impaired sensation in left in C4–5 dermatomes | Weakness in deltoid, spinatus, biceps; reflexes absent | Fibrillations and reduced interference pattern in deltoid | Not reported | Not reported | Minimal weakness after 4 years, residual neuralgia | |
#4. 83-year old. female | Pain in outer part of right arm and digitis III-V, rash on posterior forearm and digitis | Weakness in biceps, in triceps, fingers, hand intrinsics; absent reflex in triceps, depressed in biceps, supinator | Fibrillations polyphasia and reduced interference patterns, motor median velocity reduced; absent median sensory neurogram | Not reported | Not reported | Incomplete recovery of hand after 1 year, full recovery in triceps wrist extensors | |
#5. 64-year old man | Pain in right shoulder, rash in C5 distribution | Weakness in deltoideus, supraspinatus; impaired sensation in C5 dermatome; reflexes absent in whole arm | Fibrillations in deltoid, moderately reduced interference patterns in deltoid and supraspinatus | Not reported | Not reported | Full motor recovery after 2 years | |
C#6. 52-year old female | Pain and rash in left arm (C5–6 distribution); numbness in left thumb | Weakness in left deltoid, spinatus, biceps; reflexes absent in biceps, supinator | Reduced interference pattern in left deltoideus with polyphasic units | Not reported | Not reported | Incomplete recovery after 7 months | |
#7. 77-year old man | Pain in right groin, rash in anterior and medial thigh | Weakness in hip flexors, adductors, knee extensors; reflex absent at knee | Fibrillations in thigh muscles, reduced patterns, polyphasic units | Not reported | Not reported | Full recovery after 4 months | |
#8. 73-year old man | Pain around knee, rash in medial aspect of left thigh | Wasting and moderate weakness in quadriceps; reflex diminished at the knee | Fibrillations slightly reduced interference pattern, polyphasic units | Not reported | Not reported | Full recovery after 4 months | |
#9.69-year old man | Pain in right axilla, rash in medial arm and medial aspects of two fingers | Weakness in hand intrinstics, sensory impairment in C8, T1 and T2 dermatomes; absent reflexes | Fibrillations polyphasia and reduced interference pattern, reduced motor velocity in ulnar nerve, absent sensory neurograms | Not reported | Not reported | Marked wasting and weakness in thenar, hypothenar, and intrinsic muscles after 1 year | |
[29] | #1. 77-year old man | Pain and rash in left shoulder | Weakness in shoulder, biceps, triceps, and wrist extensors muscles | Denervation in deltoid and biceps, reduced MUAP recruitment | Routine lab normal; CSF increased protein, normal cell count; VZV antibodies IgG positive IgM negative; cervical spine MRI normal | Valacyclovir 3 g for 7 days, Acyclovir iv 750 mg for 7 days, Methylprednisolone iv 500 mg for 3 days, Prednisol 60 mg and tapering | Full recovery after 1 year |
#2. 57-year old man with diabetes | Pain and rash over right wrist and groin unable to walk | Weakness in right hip, thigh muscles, ankle dorsiflexors, patellar reflex diminished | Denervation in iliopsoas, quadriceps, tibial, F waves reduced in frequency | Blood tests normal; CSF increased protein and cell count; VZV antibodies IgG and IgM positive; lumbar spine MRI normal | Acyclovir 750 mg for 7 days, Acyclovir iv 750 mg for 7 days, Methylprednisolone iv 1000 mg for 3 days, Prednisolone 60 mg | Full recovery after 3 months | |
#3. 65-year old female | Pain and rash in left shoulder and arm C5–6 dermatomes | Weakness in shoulder, reflexes diminished | Reduced interference with denervation in deltoid and C5 paraspinals muscles | Cervical spine MRI normal | Valacyclovir 3 g | Marked recovery after 3 months | |
[11] | #1.84-year old man with myelodysplastic syndrome | R C5,6 | Weakness in C5,6 and atrophia | Denervation, polyphasia, normal conduction velocity | Moderate recovery after 6 months | ||
#2. 85-year old female | Pain and rash in left C5 | Weakness and atrophia in left C5,6 distribution | Denervation, polyphasia, normal conduction velocity | Poor recovery after 1 year and 7 months | |||
#3. 79-year old female | Pain and rash in right C 5,6 | Weakness in 5, 6 distribution | Denervation, polyphasia | Moderate recovery after 2 months | |||
#4. 67-year old female | Pain and rash in right C5,7 dermatome | Weakness in C5–8 | Denervation, polyphasia | Good outcome after 6 y + 8 m | |||
#5. 82/F | Pain and rash in dermatome L1-S2 bilateral | Weakness in projection L1-S1 bilateral | Moderate recovery after 3y + 8 m | ||||
#6. 80-year old man | Pain and rash in left C6,7 | Weakness in left C5-Th1 | Good recovery after 1y + 8 m | ||||
#7. 76-year old female | Pain and rash in right C6,7 dermatome | Weakness in C6-Th1 distribution | Good recovery after 7y | ||||
#8. 83-year old male | Pain and rash in right C8-Th1 dermatome | Weakness in C7, 8 distribution | Good recovery after 5y + 9 m | ||||
#9. 7 year old female | Pain and rash in right L4-S1 dermatome | Weakness in right L1,S1 distribution | Good recovery after 5y + 4 m | ||||
#10. 72-year old female | Pain and rash in right C5–7 dermatome | Weakness in right C5-Th1 distribution | Moderate recovery after 2 years and 10 month | ||||
#11. 43-year old male bone marrow transplantation | Rash and pain in right C8-Th1 dermatome | Weakness in C5-Th1 distribution | Uncertain 6y + 8 m | ||||
[30] | #1. 69-year old female with diabetes | Pain and rash in right arm, rash over face | Weakness and impaired sensation in shoulder, reflexes diminished in right arm | Not reported | Not reported | Steroid, procaine, physical therapy | Almost full recovery |
Pain and rash in low back and right leg in distribution of the sciatic nerve | Weakness in right hip flexors; knee jerk absent | Not reported | Not reported | Paravertebral sympathetic block | Unknown | ||
[31] | #1. 63-year old man | Pain and rash in right shoulder, arm, and hand | Weakness in shoulder, elbow flexors, extensors, in hand muscles; reflexes absent | Denervation in hand, motor amplitudes decreased, velocity normal; absent sensory neurograms | Complement fixing for antibodies VZV in sera was elevated | Physical therapy | Significant recovery except hand intrinsics |
#2. 80-year old woman | Pain from left knee to foot, rash in L5-S2 dermatomes, urinary frequency and incontinence | Weakness in knee flexors, ankle flexors and extensors; reflexes in ankles absent | Not reported | CSF: hyperproteinorachia | Not reported | Incomplete recovery: foot drop and urinary retention remained | |
[32] | 15 patients (9 females and 6 males, mean age 66 years, range 48–80) with rheumatoid arthritis in 3, lymphosarcoma in 1, and lymphatic leukemia in 1 | Pain-to-rash interval 2–3 days in 5 patients, 4–6 days in 6, 7 days in 2, unknown in 2; Rash-to-weakness interval < 10 days in 1 patient, 10 to 28 days in 12, 42 days in 1, unknown in 1 (similar in upper and lower limbs) | Distribution of motor paresis: C5–6 in 5, C5–7 in 1, C7-C8-T1 in 2; L3-S1 in 7, L3-S1. Weakness severe in 10 patients, moderate in 3, mild in 2; sensory abnormalities in half of the patients; reflexes diminished | Denervation in 12 patients | Increased protein and cell count in CSF of one patient | Not reported | Full recovery in 11 patients (5 arms and 6 legs). Mean recovery time in arms 9 months, 7 months in legs. 2 improved arms and 1 in legs. Postherpetic neuralgia in 3 patients |
#1. 70-year old man | Pain and rash in C5 myotome | Not reported | Not reported | Not reported | Not reported | Marked recovery | |
#2. 56-year old man | Pain and rash in L3 myotome | Diminished knee reflexes | Not reported | Not reported | Not reported | Marked recovery | |
#3. 59-year old man | Pain and rash in C5 myotome | Absent reflexes | Not reported | Not reported | Not reported | Full recovery | |
#4. 70-year old man | Pain and rash in C5 myotome | Absent reflexes | Not reported | Not reported | Not reported | Marked recovery | |
#5. 71-year old man | Pain and rash in C5–6 myotome | Absent reflexes | Not reported | Not reported | Not reported | Full recovery | |
#6. 67-year old female | Pain and rash in C7 myotome | Not reported | Not reported | Not reported | Not reported | Full recovery | |
#7. 91-year old woman | Pain and rash in T1 myotome | Not reported | Not reported | Not reported | Not reported | No recovery | |
#8. 65-year old female | Pain and rash in C5 myotome | Absent reflexes | Not reported | Not reported | Not reported | Full recovery | |
#9. 72-year old man | Pain and rash in C5 myotome | Absent reflexes | Not reported | Not reported | Not reported | Unknown | |
#10. 65-year old man | Pain and rash in L2–3 myotome | Absent knee reflexes | Not reported | Not reported | Not reported | Marked recovery | |
#11. 56-year old man | Pain and rash in L5 myotome | Not reported | Not reported | Not reported | Not reported | No recovery | |
#12. 76-year old man | Pain and rash in C5 myotome | Absent SJ and BJ | Not reported | Not reported | Not reported | Modest recovery | |
#13. 62-year old woman | Pain and rash in L3 myotome | Absent knee reflex | Not reported | Not reported | Not reported | Modest recovery | |
#14. 70-year old woman | Pain and rash in L3 myotome | Absent knee reflex | Not reported | Not reported | Not reported | Full recovery | |
[33] | 61 patients (39 men and 22 women), mean age 62 yrs. (range 18–87); lymphoma in 6, chronic lymphocytic leukemia in 1, diabetes in 3, histoplasmosis in 1 | Rash-to-weakness interval in 51 patients: <3 days in 6, 3–6 days in 12, 7–10 days in 14, 11–14 days in 11, 15–20 days in 5, 21–28 days in 2 patients, 29–35 days in 1 | Weakness in upper limbs in 16 (C5 to T1 segments at about equal frequency), lower limbs in 15 patients (L2 to S1 at about similar frequency); abdominal weakness in 2 patients | EMG in 18 patients (9 of them are within legs): fibrillations present in all affected muscles, MUAPs decreased in number in 2 with large amplitudes; sensory and motor nerve conduction studies normal in all but 3 cases | CSF proteins and cell count increased in 2 patients | Not reported | Limb recovery full in 55%, marked in 25%; residual pain in 8 patients (4 of them are with leg distribution) |
[34] | #1. 45-year old woman | Pain in right thigh, rash along the sciatic nerve | Decreased sensation in L2-S1-S2 dermatomes, reflexes diminished; weakness in right ankle dorsi/plantar flexors | 7 years later- high amplitude and polyphasia on MUAP | Lumbar/pelvic X-ray and CSF normal | Not reported | Full recovery at 3 months after the first episode; moderate after each of next relapses over 7 years |
#2. 80-year old man | Pain in all right arm, rash over anterolateral part of arm | Weakness in all right arm, reflexes diminished, shoulder atrophy and subluxation of humeral head | Fasciculations, rare action potentials in deltoid, supraspinatus and biceps muscles | Neck and spine X-ray: spondyloarthritic changes | Not reported | Incomplete recovery in shoulder muscles after 1.5 years | |
[35] | #1. 77-year old woman with hypertension and cardiomegaly | Pain and rash in right thigh, decreased sensation in L1–3 dermatomes | Weakness and atrophy in quadriceps; knee reflex absent | Not reported | Not reported | Analgesics, physical therapy | Full recovery after 3–4 months |
#2. 65-year old man | Pain in left chest radiating in left arm, rash in left C5–6 dermatomes | Weakness in proximal and distal muscles of left arm; reflexes absent in arm | Not reported | Not reported | Symptomatic treatment | Full recovery | |
#3. 74-year old female with osteoarthritis | Pain and rash in right shoulder (C5–7) | Weakness in proximal and distal muscles of right arm | Denervation in upper and middle trunks of the brachial plexus | Chest X-ray normal | Not reported | Full recovery after 6 months | |
#4. 67-year old female | Pain and rash in distal right arm, sensory loss in C6–8/T1 segments | Weakness and atrophy in hand muscles, reflexes diminished | Severe median and ulnar neuropathy | CSF increased protein, no cells | Analgesics, physical therapy | Full recovery after 8 months | |
#5. 64-year old man | Rash over lower lateral chest on left side, T9–10 dermatome | Weakness in left rectus abdominis and oblique muscles | Denervation in external oblique muscles | Not reported | Mild analgesia | ||
#6. 80-year old man with DM, myocardial infarction, CVI | Rash and burning pain over right lower abdomen | Bulging of lateral and anterior abdominal wall | Fibrillations and positive waves in right abdominal muscles with later reinnervation | Not reported | Not reported | Full recovery after 4 months | |
[14] | 58-year old female | Pain over left side of neck, rash over left shoulder, lateral left arm, hand and wrist | Weakness of left shoulder and left deltoid muscle | Splint | After 2 months mass and power of the deltoid had increased | ||
[36] | #1. 53-year old man | Disseminated vesicular rash and general malaise | Developed leg weakness in 2 days unable to stand; facial diplegia; reflexes depressed | Normal in arms and legs | Diagnosis of GBS was made and no specific treatment was given | One year later was totally asymptomatic | |
#2. 69-year old man with asthma | Painful rash on right buttock | Bilateral facial and truncal weakness; weakness in legs all reflexes absent, loss of light touch and position sense | Mild cyanosis, reduced lung capacity | Received course of plasmapheresis | 5 months later only residual sign of mild reduction of hip power | ||
[21] | 48-year old woman | Pain in left arm, rash over the C5 to C7 dermatomes | Weakness in the left deltoids and biceps muscle and a diminished left biceps reflex; dyspnea | 1000 mg Valacyclovir orally three times daily for 7 days | After 2 years- without further complications; pain resolved in three months | ||
[1] | 80-year old female, dementia | Pain in left part of neck | Left facial palsy; difficulty walking with left lower limb | Gait was normal after 3 months, but left facial palsy remained complete | |||
[37] | #1. 61-year old man | Painful rash on dorsum of right foot, sensory loss over the lateral right leg | Weakness of all parts of right leg, reduced right ankle reflex | Fibrillation and reduced MUAP in muscles innervated by the distal sciatic nerve | PCR of cutaneous crusted of right foot lesions was positive for VZV | No neurologic follow-up | |
#2. 69-year old man | Burning pain in right upper limb | Weakness in muscles innervated by right median nerve (right hand) | Fibrillation potentials and reduced MUAP | MRI of arm enlargement T2 signal within the median nerve with gadolinium contrast | Gabapentin | Incomplete improvement- weakness with residual pain | |
#3 83-year old woman | Rash over right upper limb | Weakness of right hand grip | Fibrillation and reduced MUAP in distribution of C7-T1 roots; conduction block in the median nerve | After 11 months moderate residual weakness in median innervated muscles | |||
#4 55-year old man with migraine, restless legs syndrome | Rash on knee and ankle, pain in left buttock, anterolateral thigh and knee | Left knee stretch reflex absent | Fibrillation and reduced MUAP in left iliopsoas and rectus femoris muscles | MRI enlargement and T2 signal in left femoral nerve | No clinical follow-up | ||
[38] | 60-year old man | Pain in right leg, rash in anteromedial part of right thigh | Weakness, atrophy and fasciculation of right quadriceps; right knee reflex absent | Reaction of degeneration in the right quadriceps femoris | Thiamine hydrochloride 10 mg. three times a day orally, heat and electrical stimulation | After 3 months incomplete recovery with atrophy of the thigh and fasciculations | |
[39] | #1. 71-year old man | Pain on right side of chest weakness in both leg and right hand | Deep reflexes brisk | Physiotherapy | 3 months later almost full recovery | ||
#2. 58-year old man | Rash left side of neck and right upper arm | Weakness in both legs and right arm; reflexes absent in all limbs | Fibrillation potentials affecting muscles of right limb | Velocities affecting in right median and popliteal nerves | Physiotherapy, tetracycline | After 11 months little residual deficit | |
[10] | #1. 47-year old man | Rash in right shoulder and anterolateral arm | Weakness in right C5–6 myotomes | Denervation in C5–6 distribution | Recovery in 3 months | ||
#2. 70-year old female with DM | Rash in right lateral arm and forearm | After 20 days weakness in right C5–7 myotomes | Right brachial plexopathy with denervation | Hyperintensity in spinal dorsal horns at C4–5 | No recovery after 2.0 years | ||
#3. 63-year old male with DM | Rash of the right foot and a right L5-S1 plexopathy | Weakness in right L5-S1 myotomes | No recovery after 1.8 years | ||||
#4. 80–90-year old with DM | Rash in neck first | After 22 days right C8 myotome weakness | Denervation in right plexus brachialis | No recovery after 1.9 years | |||
#5. 87-year old male with DM | Rash in right lateral arm and forearm | After 14 days right C6–8 myotome weakness | Denervation in right C6–8 distribution | Increased signal in the C6–8 nerve roots | No recovery 1.0 year | ||
#6. 60-70-year (nn gender) | Rash in right buttocks and lateral calf | Weakness in right L5 myotome | Right L5 radiculoplexopathy with denervation | Partial recovery after 1.0 year | |||
#7. 61-year old male | Rash in left thumb, index finger and forearm | After 15 days weakness in left C6–8 myotomes | Left C7 radiculopathy with denervation | Increased signal in median and radial nerve on MRI | No recovery after 0.5 year | ||
#8. 80-year old female | Rash in right shoulder, anterolateral arm and thumb | Weakness in right C5 myotomes | Denervation in right C5 distribution | Increased signal in the C5 nerve roots on MRI | Partial recovery after 0.5 year |
There are few comorbidities in this population of patients: 2 patients with carcinoma mammae, 7 patients suffering from lymphoma, 4 were taking corticosteroids due to autoimmune diseases (2 patients with rheumatoid arthritis, 1 with polymyalgia rheumatica, and 1 with myasthenia gravis), and 4 of them were diabetic.
Clinically, segmental zoster paresis is usually unilateral paresis of arm, predominantly on the right side (42/26, 33 cases do not specify the affected side).
Proximal muscles were affected in most cases (in 52 patients), while the entire arm [40] or only distal muscles [41] were significantly less affected.
Besides weakness, in 9 patients, muscle atrophy was detected.
Phrenic nerve affection followed by dyspnea was observed in 6 patients.
Electromyoneurography was performed in 58 patients, and denervation potential has been described in most cases (51 patients), often associated with reduced pattern and polyphasia of motor unit potentials in most cases (17 patients). Reduced motor or sensor velocity was seen in 4 patients. In some cases, neurophysiological finding was only descriptive: “lesion of nerve,” “acute motor axonal lesion,” and “severe median and ulnar nerve neuropathy.”
Another test was performed in only small number of patients: neck MRI (unremarkable in 2 patients, foraminal stenosis in 1, and protruded disk in 1) and MRI of the affected arm in 1 patient with enlargement of T2 signal of the median nerve.
X-ray of the shoulder was performed in 2 patients (1 patient showed subluxation) and 2 X-rays of the neck (1 unremarkable and 1 with spondyloarthrosis) and 2 X-rays of the chest with 1 showing elevated hemidiaphragm and paralysis confirmed by following fluoroscopy were performed.
Standard laboratory in 2 patients was unremarkable; positive sera antibody test for HZ was found in 3 patients.
Hyperproteinorachia was found in 2 patients and normal CSF was found in 1 patient among patients with lumbar puncture.
In 1 patient, surgical exploration was done and it was without compression.
In almost all cases with a mentioned type of treatment were treated with physical therapy (24 reported patients). Some of them had other therapies: Valacyclovir 3 g/7 days (3 patients), Acyclovir 750 mg/7 days (2 patients iv and 1 per os); and Methylprednisolone 500 mg iv for 3 days (1 patient), and some of them were taking steroids [11], cervical epidural bloc [11], analgesics [42], opioids [43], Amitriptyline [43], Pregabalin [11], and Gabapentin [11].
Most reported patients recovered significantly: complete or near complete recovering is recorded in 8 patients that are reported during following periods: 3 weeks, 1 month, 2 months, 3 months, 6 months, and 2 years. Incomplete recovery was reported in 3 patients, minimal recovery in 1 patient after 2 months, and no recovery after 8 months in 1 patient. One patient recovered from weakness after 5 months but with persisted neuralgia.
3.1.1 Lower limb involvement
There are 43 presented patients with isolated segmental zoster paresis of one leg in the total of 26 previously published papers. Nine of these papers present only zoster paresis of the lower limbs, while the rest of them describe affection of arms and torso, also. According to available information, mean age of this group of patients was 64.19 ± 15.28, and the patient group was dominated by males (20/12).
From medical history, these patients had: diabetes mellitus (4 patients), lymphatic leukemia [11], ulcerative colitis [11], myelofibrosis [11], renal failure [11], hypertension arterialis [43], dementia [11], restless legs syndrome, and chronic low back pain [11].
Almost all patients had weakness in one leg; but 3 of them developed weakness in both legs.
In most cases, proximal muscles were affected (in 13 patients); then in much lesser number, distal muscles (6 patients) were affected; and only 4 of them had developed entire limb weakness.
Muscle atrophy was seen in 3 patients in this group (in
With regard to other complications, 2 patients developed incontinency, 1 urinary retention, 1 ileus, and 2 abdominal wall weakness.
Denervation has been the most common report (in 18 patients) among patients with electromyoneurography performed; reduced interference pattern was found in 12 patients and polyphasia was present in 4 patients.
Laboratory is sporadically performed in these patients: ELISA for HZV in sera done in 2 patients and were positive in IgG and IgM fraction in both of them, and in the third patient, performed VZV complement fixation was positive, also.
A lumbar puncture was done in a small number of patients [44, 45]: cerebrospinal fluid (CSF) was normal in two cases, but in the other three patients, hyperproteinorachia was discovered.
The patient with flaccid paraparesis had elevated lymphocytes and hyperproteinorachia in cerebrospinal fluid, while PCR for VZV was positive in this case.
MRI of lumbar spine performed in 4 patients did not explain the nature of deficit in these patients, and 2 patients were generally described by degenerative changes, 1 was normal, and in 1 enlargement and T2 signal in the left femoral nerve was found.
The most frequent treatments were physical therapy (4 patients), Acyclovir (2 patients), Methylprednisolone (2 patients), vitamins B1 and B12 [11], paravertebral sympathetic block [11], analgesics, and Gabapentin, lately [11].
There is no information about outcome of the disease for some patients. However, among patients who were followed for a period of time, a majority of them (20 patients) fully or almost fully recovered in the period between 2 and 9 months; 11 patients recovered incompletely; 2 patients did not recover, and 2 patients had with lethal outcome.
3.1.2 Diaphragmatic paralysis
Diaphragmatic paralysis due to phrenic nerve involvement with VZV is described in 24 previously published papers with a total of 26 patients with a mean age of 67.13 ± 11.50 years.
Among comorbidities in this population of patients are rheumatoid arthritis [11], leukemia [11], breast cancer [11], pyelonephritis and nephrectomy, hysterectomy [11], DM [11], hypertension [43], peptic ulcer [11], pancreatitis [11], and bypass [11].
Hemidiaphragmatic paresis is unilateral usually, predominantly on the left side [46], while in one patient, hemidiaphragmatic affection on both sides were recorded.
Although EMNG of phrenic nerve is one of the reliable signs of affection in this nerve, it is rarely performed (1 patient). More often, fluoroscopy is performed when weakness of the diaphragm is suspected (7 patients). Although not a reliable sign of weakness of the diaphragm, X-ray is often done in the case of a suspected weakness of the diaphragm paresis (13 patients).
Phrenic nerve affection is usually followed by dyspnea, but there is a case with hemidiaphragmatic paresis on X-ray, but without visible dyspnea.
To the contrary, there are cases with normal hemidiaphragm position on non-CT chest, when dyspnea is present clinically.
Half of the patients [47] did not recover after significant a follow-up period (1 year and more). Partial recovery was seen in significantly smaller number of patients [48], while complete recovery was present in the fewest number of patients [42].
In most cases, the type of treatment of these patients was not mentioned: Acyclovir in 2 patients, Valacyclovir in 1 patient, physical therapy in 1 patient, and topical hydrocortisone in 1 patient.
The incidence of segmental zoster abdominal paresis mimicking an abdominal hernia is relatively rare. After review from 2013 [49] with 36 patients and segmental zoster paresis of abdominal muscle (14), 8 papers with 11 patients with this problem have been published.
Their mean age (66.5 years) is not much different from the average age of the patients in the review paper from 2013 (67.5 years).
As in the review paper, the predominant level of abdominal involvement of herpes zoster was Th11.
It is the most important to exclude organic disease of the abdomen in the case of abdominal herniation when beneficial are ultrasound or CT abdomen showing normal results.
This is particularly relevant when the abdominal herniation is complicated with, for example, ileus, which is described in 1 patient.
The presence of denervation potentials in EMG of paraspinal (3 patients), or abdominal muscles (2 patients), indicates the involvement of the abdominal musculature, which separates EMG as well as a particularly useful diagnostic method in this case.
Prognosis of these patients is generally good, and there is full recovery in almost all of them in just few months [42, 43, 50].
Acyclovir is rarely used as a therapy in these patients (2 patients).
4. Discussion
Increasing rate of herpes zoster infection with increasing age particularly after age 50 years can be explained by natural decline in cell-mediated immunity to VZV with age [51]: the mean age of patients with the most frequent segmental paresis was for arms 68.56 ± 11.97 years and for legs 64.19 ± 15.28 years.
Healthy people can get HZ [31], although immunocompromised individuals are known to be at increased risk of reactivation and VZV infection [52].
The most immunocompromised patients with zoster paresis have a coexisting malignancy, diabetes mellitus, and chronic steroid therapy [53]: 7 patients suffering from lymphoma and 2 with carcinoma mammae in group with arm segmental paresis and 1 patient with segmental paresis of leg had lymphatic leukemia; as per 4 patient in each group and 1 in group with diaphragmatic paresis suffered from DM and as per 4 patient in both groups of patients with limb segmental paresis on chronic steroid therapy because of autoimmune disorders.
In clinical presentation of HZ infection, pain usually precedes the onset of the rash, and most patients have skin lesions that develop within 7 days of onset of pain [32].
Motor palsy is usually segmental, with abrupt onset reaching its maximum within a few hours [31], corresponding to the dermatomes with cutaneous lesions [18]. Interval between skin eruptions and onset of muscle weakness is generally about 2 weeks in cases with developing segmental paresis [54], but there are some reports of weakness and rash developing simultaneously [55]. With reference to the literature, maximal rash-to-weakness interval in patients with segmental limb paresis was 19 days [37] although there are different experiences. Variations in rash-to-weakness interval is best illustrated in a study of 51 patients: <3 days in 6 patients, 3–6 days in 12, 7–10 days in 14, 11–14 days in 11, 15–20 days in 5, 21–28 days in 2, and 29–35 days in 1 [33]. A delay of 4.5 months has been documented in a patient with diaphragmatic paralysis [56], when the average minimum duration of weakness was 193 days [57]. The fact that the phrenicus nerve is a motor nerve and is the longest in the body is the explanation for such a long period of time required for the development of paralysis of phrenic nerve.
Limb involvement by segmental zoster paresis is seen from 0.5 to 0.8% of all patients with cutaneous zoster [10, 11].
Upper extremity involvement is the most common region of extracranial zoster paresis involvement. There are a total of 101 patients with segmental paresis of upper limbs in recently published papers: 55 women and 45 men (Tables 1 and 3).
Literature | Demographics, medical history | Subjective complaints | Neurological impairments | EMG findings | Other tests | Treatment | Course/outcome |
---|---|---|---|---|---|---|---|
[58] | 40-year old women; in contact with a child with chicken pox | Rash on left buttock, burning pain in lower back, hip, and left leg | Moderate weakness in left ankle plantar flexors | Normal | Blood and CSF normal, chest and spine X-ray normal | Intrathecal Methylprednisolone, analgesics, bed rest, sodium iodide and oxytetracycline i.v.; hydrocortisone ointment for rash | Complete motor recovery after 9 months |
[59] | 20-year old man, chicken pox at age 7 | Numbness in left foot; pain and rash over left gluteal region | Weakness in left ankle dorsiflexors | Not reported | Not reported | B1 and B12 injections | Full recovery after 2 months |
[60] | 31-year old man, diabetes, end stage renal failure with maternal renal allograft, autonomic neuropathy, blindness | Rash and pain in right lower back, urinary retention, weakness in right leg, diminished sensation in lumbar and sacral segments | The right ankle plantar flexors (3/5 on MRC scale) and diminished ankle jerk | Not reported | ELISA test on HZV in sera was positive for IgM and IgG, which confirmed the presence of HZ infection | Not reported | Unknown recovery of leg function, some bladder recovery |
[61] | 57-year old man | Rash over left T8–9 dermatomes, hyperalgesia in both legs, unable to walk, fasciculations in both legs | Flaccid paralysis of both legs, reflexes absent | Not reported | CPK: mild transient elevation; VZV complement fixation positive | Not reported | Full recovery after 3 months |
[62] | 70-year old woman, with hypertension | Pain in buttocks and legs; rash over left knee, thigh, buttocks; hyperesthesia in left leg; urinary incontinence | Moderate weakness in knee flexors/ extensors, mild in ankle flexors; reflexes absent in left knee, ankle | Diffuse denervation in leg and paraspinal muscles in L3–5 myotomes | X-ray: mild narrowing of L5-S1 disc space; spine MRI: right L4–5 facet joint disease | acetaminophen, oxytocin, bed rest, bupivacaine via L4–5 epidural catheter; parenteral meperidine | Near complete recovery |
[63] | 78-year old man, idiopathic myelofibrosis receiving cytoreductive therapy | Sudden weakness in both legs; rash in right lower leg involving knee and thigh | Bilateral leg paralysis | Not reported | CSF: increased lymphocytes, monocytes, protein, glucose; PCR on VZV highly positive; X- ray: ileus | Wide spectrum antibiotic, granulocyte colony stimulating factor and erythrocyte transfusions; high-dose acyclovir i.v. | After initial improvement, progressive worsening complicated by pneumonia and death |
[64] | 60-year old man with lymphatic leukemia | Burning pain along the inner aspect of the right lower leg (6/10) and rash, weakness of the right foot | Weakness of the right ankle plantar flexors (3/5 on MRC scale) and diminished ankle jerk | Fibrillations and positive sharp waves in the right gastrocnemius and paravertebral muscles (S1 root); polyphasic MUAP during activation of right foot, nerve conduction was unchanged | MRI of lumbosacral area: degenerative changes; ELISA test on VZV in sera positive for IgM and IgG | Physical therapy and oral gabapentin 900 mg/day | Motor weakness completely resolved about 6 months after the onset of neurologic symptoms but the pain was sporadic but mild |
[65] | 74-year old with diabetes mellitus, hypertension, and ischemic heart disease | 5-day history of paraesthesia starting in the right foot and ascending up the right lower limb | Vesicular rash in the L2/3 region with MRC grading 3/5 in the right hip flexors | MRI: unremarkable | Acyclovir i.v. | Motor paresis that recovered fully with resolution of the rash | |
[40] | 37-year old-female with history of paresis in both legs secondary to spinal cord atrophy and Vogt-Koyanagi-Harada disease and with chronic corticosteroid and azathioprine treatment of ulcerative colitis | Worsening of her baseline residual muscle strength in the right lower limb shortly after herpes zoster eruption | EMG: denervation in L3-L4 and moderate axonal polyneuropathy affecting both lower limbs |
Segmental zoster paresis of the legs present in less than half the number (43 patients with leg involvement) dominated by males (20/12) (Tables 2 and 3).
Phrenic nerve affection was described in 26 patients (Table 4).
Literature | Demographics, medical history | Subjective complaints | Neurological impairments | EMG findings | Other tests | Treatment | Course/Outcome |
---|---|---|---|---|---|---|---|
[66] | M, 53-year old | HZ in C3,4 dermatome | dyspnoea | ||||
[67] | 80-year old female, nephrectomy because pyelonephritis | Pain and rash over left shoulder and anterior part of chest | weakness of left sholder and proximal muscles, atrophy of supraspinatus and infraspinatus muscles; dyspnoea on left hemidiaphragm | denervation of infraspinatus and supraspinatus | Rtg- paretic left hemidiaphragm | ||
[44, 45] | 56-year old male with peptic ulcer | rash and pain in later aspect of right arm and 3 middle fingers | general weakness of right arm and hand, atrophy of right part of neck, paralysis right hemidiaphragm | X-ray and fluoroscopy-complete paralysis of rigth hemidiaphragm | |||
[68] | 77, F rheumatoid artritis, DM | Rash and pain in C3-5 dermatome | paralysis right hemidiaphragm | ||||
[46] | 72-year old female, RA, hypertension | Rash and pain in right C3,4 dermatome | dyspnoea | ||||
[69] | 66-year old female | HZ of left C3,4,5 | after 12 months dyspnoea | X-ray –elevated left hemidiaphragm; Radioscopy-paralysis of left hemidiaphragm | |||
[50] | 56, male bronchitis | HZ in 1st cervix dermatome | none | X-ray –elevated left hemidiaphragm | |||
62-year old female astma | HZ in 1st cervix dermatome | dysponea | |||||
67-year old female | HZ in 1st cervix | dysponea | |||||
57-year old female | HZ in 1st cervix | dysponea | |||||
[70] | 74-year old female | HZ in C3,4 | dysponea | ||||
[71] | 80-year old female, hypertension,histerectomy | HZ in C3-6 | Dyspnea, upper limb muscle weakness | ||||
[72] | 74-year old male, pulmonary emphysema | HZ in C2-5 | deltoid muscle weakness, dyspnoea | ||||
[16] | #2. 60-year old man with lymphatic leukemia | Rash and hypersensitivity in right C5 dermatome | coughing; enlarged liver; | Not reported | Chest X-ray: elevated right hemidiaphragm, paralysis confirmed on fluoroscopia | Complete Recovery after 1 month | |
[56] | 74-year old male | HZ in 1st cervix | cough, dyspnea | not alleviated after 4 month | |||
[73] | 79-year old male, hypertension, carotid endarterectomy, bypass | HZ in cervix region | dyspnea, orthopnea | After 12 months not alleviated | |||
[56] | 74-year old male | HZ in cervix | cough, dyspnea | After 4 month not alleviated | |||
[42] | A 73-year old woman | herpes zoster of left shoulder and proximal arm | weakness of left shoulder and proximal arm muscles 3 weeks after a diagnosis of herpes zoster | involvement of the C5-6 myotomes and the upper trunk of the brachial plexus | Chest X-ray and electromyographic studies documented paralysis of the left. | One year after muscle strength returned to normal, but radiographic and electrophysiologic findings of diaphragm paralysis were unchanged. | |
[74] | 74-year old man | HZ on left shoulder and neck | Left hemidiaphragm paralysis | Axonal changes in left nervus phrenicus | CT and X-ray- left hemidiaphragmatic relaxation | Acyclovir | not allevia after 18 months |
[75] | HZ in left side of neck | Dyspnea after 3 months | X-ray- left hemidiaphragm relaxation; Pulmo-rary functions-restriction | ||||
[76] | 54-year old male | thoracic herpes zoster, 1st chest, neck, bilateral shoulders | bilateral diaphragmatic paralysis associated with brachial neuritis, orthopnea, deltoid and biceps brachii muscle weakness | Fluoroscopy | not allevia after 19 months | ||
[42] | 73-year old woman | HZ and pain of left shoulder | weakness of left shoulder and proximal arm muscles 3 weeks after HZ was diagnosed and paralysis of left hemidiaphragm | EMG – denervation, reduced recruitment od MUP Phrenic nerve conduction study-denervation without MUP | Chest X-ray- paralysis of the left diaphragm. | Weakness of arm return to normal after one year but X-ray of paralysis hemidiaphragm was unchanged | |
[21] | A 48-year old female | rash and vesicles over left C5-7 dermatomes | weakness in the left deltoids and biceps muscles, diminished left biceps reflex. dyspnoea with paradoxical abdominal wall movement | CT of chest normal, MRI-hyper-intensity in spinal cord at C5 level | 1000 mg valacyclovir orally three times daily for seven days. | Pain resolved three months later, follow-up for 2 years without further complications. | |
[77] | 85-year old female breast cancer at age 84, pancreatitis, choleatitis | Rash and pain in her left neck, chest, and arm in C4,5 dermatome | A chest X-ray elevated left diaphragm | Famciclovir for 7 days at a dose of 750 mg per day. | After 14 months dyspnea and no alleviation | ||
[48] | 43-year old man | Rash on right neck and apper right hemithorax C3-5 | hiccups | On X-ray paralysis of the right hemidiaphragm, HIV + | intravenous acyclovir and admitted to the hospital |
Among patients with limb paresis, proximal muscle involvement usually predominates (C5, 6, 7 or L2, 3, 4). The most commonly affected muscle is deltoid in upper limbs [78].
Besides weakness in upper limbs affected by segmental zoster paresis, in 9 patients, muscle atrophy was detected and in 3 patients in group had leg affection (in
It is important to recognize severe muscle weakness and atrophy in herpes zoster paresis of limb because it can be so severe to cause marked dislocation of the joint. Because of that, except pain medication, treatment for segmental paresis includes exercise that may prevent muscle atrophy and contractures [17].
Risk of postherpetic neuralgia and pain after 3 months of HZ infection increased and occurs in 8–70% of patients with HZ. People above 50 years are 15 times more likely to develop this complication [79].
In studies involving a larger number of patients with segmental zoster paresis, postherpetic neuralgia persisted in 2 and 6.6% [32, 33].
Among the patients monitored over several months and multiyear period, 13 patients with segmental zoster paresis of arm and 6 patients with paresis of leg have reported pain as the postherpetic neuralgia type (Tables 1–3).
The clinical diagnosis makes pain followed by rash and by weakness at the end. Once the rash appears, diagnosis of HZ can be made and laboratory confirmation is not always required.
Some patients may have zoster sine herpete, and it is zoster infection without vesicular eruptions. In making this diagnosis benefit is from varicella zoster virus antibodies in sera or cerebrospinal fluid [13]. In rare cases with herpes sine herpete, cases with prolonged period between rash and muscular weakness and cases with dissociation between motor segment and level of dermatomal involvement recommended laboratory confirmation of VZV infection because herpes zoster paresis may be difficult to recognize in these cases.
Possible explanation for zoster sine herpete and for herpes zoster paresis without associated dermatome eruption could be viral spread to anterior roots without corresponding axonal transport through the sensory nerve [6, 12].
Electrophysiologic study of segmental zoster paresis found reinnervation of muscles, absence of fasciculations in involved muscles, and slow motor nerve conduction velocity suggesting motor axon injury rather than anterior horn cells [80].
In electromyoneurography of 58 patients with arm paresis, denervation potential has been described in most cases (51 patients), often associated with reduced pattern and polyphasia of motor unit potentials in most cases (17 patients) and reduced motor or sensor velocity in 4 patients.
Denervation has been the most common report (in 18 patients) among patients who had electromyoneurography of leg paresis; reduced interference pattern in 12 patients and polyphasia in 4 patients were present.
Although EMNG of phrenic nerve is one of the reliable signs of affection in this nerve, it is rarely performed (1 patient).
The presence of denervation potentials in electromyography of paraspinal (3 patients), or in abdominal muscles (2 patients), indicates involvement of the abdominal musculature, which separates EMG as well as a particularly useful diagnostic method in case of abdominal wall zoster paresis (Table 5). Because of its self-limited nature and good prognosis, recognition of this complication is important to prevent unnecessary diagnostic studies and procedures, and because of that, abdominal hernia needs no surgery. Thus, electrodiagnostic studies can be effectively used to confirm the diagnosis.
Abominal wall | Demographics, medical history | Subjective complaints | Neurological impairments | EMG findings | Other tests | Treatment | Course/outcome |
---|---|---|---|---|---|---|---|
[81] | 73-year old man with L3 vertebral compression fracture and RA (Prednisolone) | Rash and pain with blisters on his right flank | T12 and L1 segmental paresis caused abdominal wall pseudohernia, scoliosis, and standing and gait disturbance | Denervation in right T12 myotomal muscles, and MUAP markedly decreased | Orthosis Exercise | After 4 months of rehabilitation, marked improvement | |
[82] | 72-year old man | Herpes zoster infection in T11-T12 left dermatomes | Segmental abdominal wall protrusion | Denervation in left external oblique muscle and left paraspinal muscles at T11-T12 level | SSEPs- no response in the left side at T12 dermatome | After 3 months abdominal wall protrusion had completely resolved | |
[83] | Abdominal wall postherpetic pseudohernia | MRI- increased signal intensity in abdominal wall muscles. Ultrasound- normal | Full recovery | ||||
[43] | 62-year old male | Cutaneous vesicular eruption on the left side of the abdominal wall | Abdominal distention and paralytic ileus because of a visceral neuropathy | X-rays and CT showed distended small bowel | Acyclovir iv, oral Valacyclovir, Gabapentin 8 d after admission | Full recovery abdominal distention gradually resolved over the next 7 days and pain subsided | |
[47] | 35 articles that described 36 individuals; mean age was 67.5 years. The ratio of men to women was 4:1. | The most affected dermatome was with rash is T11. The left and right sides were approximately equally affected | In 88.9% of the patients, herpetic rash preceded abdominal weakness. The mean latent period from rash to onset of abdominal muscle weakness was 3.5 weeks. | Electrodiagnostic studies confirmed the diagnosis in 95% of tested patients. | Conservative measures | Complete recovery with conservative measures occurred in 79.3% patients, with a mean time of 4.9 months | |
[84] | 58-year old man | Rash in area 9th to 11th | Protrusion in the right abdominal wall with no pain | Ultrasonography excluded the abdominal wall defect | Oral acyclovir, mecobalamin, and vitamin B1 | Disappeared after 2 months | |
[85] | 4 patients | Involvement of posterior rami of spinal nerves in abdominal wall pseudohernia | In 3 patients, EMG of paraspinal muscles showed denervation potentials | MRI-hyperintensity of these muscles on short T1 inversion recovery imaging | |||
[86] | 57-year-old man | Rash and dull squeezing pain | Bulge on his left flank 2 weeks after cutaneous changes |
The treatment for segmental zoster paresis includes physical therapy for weakened muscles and protection contractures with graduated exercise. This program may prevent muscle atrophy. Muscle weakness and atrophy can be so severe to cause marked dislocation of the involved joint [17].
In segmental paresis of arm, in most cases, a way of treating these patients is not mentioned. In others, physical therapy was the most common way of treating these patients (24 reported patients) and patients with leg affection, also (4 patients).
Beside physical therapy, for these patients, it is often necessary to provide pain medication, and therefore they are usually given analgesics, opioids, Amitriptyline, Pregabalin, and Gabapentin.
According to some recommendations, initiating treatment with antiviral agents as soon as the rash appears is the key to improve the outcome of herpes zoster. A 3-week course of oral corticosteroids (prednisone 60 mg/day for first week, 30 mg/day for second week, and 15 mg/day for third week) administered with the antiviral medication also has some effect on severity and duration of pain and may decrease the incidence of postherpetic neuralgia [87]. Local, epidural, and sympathetic blocks, if administered within the first 2 weeks of disease, have been reported to decrease pain and the incidence of postherpetic neuralgia [88, 89].
Initiating treatment with antiviral agents as soon as the rash appears is the key to improve the outcome of herpes zoster. As the effect of this therapy on the development of segmental paresis is not known, despite such recommendations, it appears that very small number of patients from the literature with segmental zoster paresis was treated with antiviral medication and corticosteroids. Among the antiviral drugs, Valacyclovir and Acyclovir (in total 13 patients) and corticosteroids in total 5 patients were usually used.
Previous experience in groups with a higher number of patients with segmental zoster paresis shows that the outcome of lower motor neuron involvement is relatively good [55]. Motor paralysis is recovered completely or nearly completely in 50–70% of cases usually within 12 months, spontaneously [11]. The time of recovery varies from 1 to 2 years [90]. Only 15% have significant deficit [33].
Among presented patients with known clinical outcomes, complete or near complete recovery is recorded in 9 patients with arm involvement and in 20 patients with leg affection.
Incomplete recovery was reported in 3 patients with arm affection and in 11 patients with leg affection (Tables 1–3). Two patients from each group, with segmental paresis of arms and legs, had no clinical recovery. As possible explanation of absence of a complete recovery after motor segmental paralysis caused by herpes zoster is glial scar polyradiculitis evident on MRI [91].
Prognosis in patients with diaphragmatic paralysis is not good because of prolonged reinnervation of diaphragm due to relatively long course of phrenic nerve affection, and the lack of spontaneous recovery is not surprising [74]. It is common for zoster phrenic nerve affection and associated diaphragmatic paralysis to be permanent, but occasionally, recovery has been reported after 7 and 12 months [92].
There are interesting experiences related to Piramat. It may reverse phrenic nerve paralysis in patients with diabetes, but there are no data about its use in patients with zoster-induced diaphragmatic paralysis [90].
It is important to distinguish segmental zoster paresis of abdominal wall from real abdominal wall hernia because abdominal wall hernia is mainly treated by surgery, while segmental zoster abdominal paresis needs no surgery. Ultrasonography or computed tomography (CT) is necessary to do in this case. Electromyographic testing revealed denervation in the affected dermatome and pseudohernia caused by abdominal wall paresis and is of great benefit in defining the problem and recommended in these situations.
5. Conclusion
Segmental zoster paresis is a rare complication of VZV infection. Increasing rate of herpes zoster infection and its segmental paresis is confirmed by the mean age of presented patients‑it is above 65 years.
Recognizing segmental zoster paresis is important in the differential diagnosis of muscle weakness of other origin—it is of particular importance to perform electromyoneurography of paretic muscle.
Physical therapy is the most common therapy for these patients, although a lot of patients did not conducted any specific way of treatment.
Prognosis for these patients is generally good, and there is full recovery in most cases, except VZV infection of phrenic nerve and diaphragmatic paresis, where there is no significant recovery of muscle weakness in significant number of patients.
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