Open access peer-reviewed chapter

Extracranial Herpetic Paresis

By Vesna Martic

Submitted: July 19th 2019Reviewed: November 14th 2019Published: April 1st 2020

DOI: 10.5772/intechopen.90493

Downloaded: 37

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].

Figure 1.

Origin of segmental zoster paresis: the spread of varicella zoster infection from the posterior horn of spinal cord to the anterior horn and the motor nerve root.

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.

LiteratureDemographics, medical historySubjective complaintsNeurological impairmentsEMG findingsOther testsTreatmentCourse/Outcome
[14]58-year old. femaleRash in left shoulder, lateral part of left arm and hand; pain in neck and interscapular regionWeakness of left shoulder, wasting of left M. deltoideusExercisesAfter 2 months, power and mass of M. deltoideus increase
[15]#1: 82-year old femalePain and rash in right shoulder and upper armWeakness in proximal arm mild elbow flexion contractureDenervation in deltoid, biceps, supraspinatus; polyphasia and decreased number of MUAPMoist hot packs, ultrasound, exerciseIncomplete recovery
#2: 72-year old female, breast carcinoma with mastectomyWeakness in biceps, deltoid, wrist flexors/ extensorsDenervation in almost all arm muscles; ulnar and median conduction velocities reducedNot reportedB12 injections, heat, paraffin baths, exercisesIncomplete recovery
#3: 65-year old female with knee amputation for vascular disease, chronic leukemiaPain and rash in right armWeakness in deltoid, biceps, triceps, wrist extensors, thenar musclesMarked denervationNot reportedHeat, electrical stimulation, strengthening exercisesFunctional recovery within 6 weeks
#4: 62-year old female, rheumatoid arthritisPain and rash in right armWeakness in deltoid, infraspinatus, supraspinatus, bicepsDenervation; polyphasic MUAPNot reportedHeat, electrical stimulationslingSome recovery of strength and less pain
#5: 71-year old femalePain and rash in right shoulder and forearmWeakness in deltoid, biceps, wrist flexors/extensorsDenervation in C7–8/T1 musclesNot reportedCodeine, whirlpool, paraffin bathsNearly complete recovery after unknown time period
[16]#1: 70-year old femalePain in left shoulder with radiation to arm, hyperesthesia in shoulder and neck; rash in C4–5 dermatomesWeakness in arm; reflexes absent in biceps, supinatorDenervation in deltoid and biceps musclesUnremarkable chest X-ray and laboratory work-upPhysical therapyFull recovery after 3 weeks
#2. 60-year old man, lymphatic leukemiaRash and hypersensitivity in right C5 dermatome after weeks of coughing; enlarged liverNot reportedChest X-ray: elevated right hemidiaphragm, paralysis confirmed on fluoroscopyComplete recovery after 1 month
[17]25-year old femaleRash over shoulderWeakness in deltoidNot reportedX-ray: shoulder subluxationNot reportedFull recovery after unspecified number of months
[18]59-year old man, diabetes, mild hypertensionPain and rash in left C7 dermatome; hyperalgesia in inner half of left handWeakness in whole arm; marked atrophy in almost all arm muscles, reflexes diminishedFibrillations, reduced interference pattern in atrophied muscles; conduction velocities normalX-ray: spine normal; CSF: normalNot reportedUnknown
[13]#1. 81-year old manPain in left arm and left lateral chest wall; rash in C5–7 dermatomesComplete flaccid weakness of left armNot reportedNot reportedPhysical therapyComplete recovery after 3 months
#2. 75-year old female, diabetesPain and rash in right shoulder and over back of the headMarked weakness in deltoid, biceps, infraspinatus; reflexes absent in bicepsDenervation in infraspinatus, deltoid, biceps; conduction velocities normalNot reportedNot reportedMinimal recovery after 2 months
[5]64-year old femalePain and rash in left inner arm and fingers after 4 months of an episode of shinglesSevere weakness in wrist and hand intrinsic musclesLesion of anterior interosseus nerveSurgical exploration‑no compressionNot reportedNo recovery after 8 months
[19]73-year old man, left biceps rupture 10 yrs. earlier with full recoveryPain in left shoulder; rash over biceps, brachioradialisModerate atrophy of deltoid; weakness in biceps and infraspinatus; reflexes diminished in biceps, brachioradialis muscleAcute motor axonal lesion in C5–6 dermatomesNeck MRI: no spinal cord, root compressionAcyclovir, opioid analgesics, amitriptyline, physical therapy, home exercisesSlight deltoid weakness after 2 years
[7]72-year old man with oral prednisolone due to myasthenia gravisRash in left C3–5 dermatomes10 days before the rash, weakness of the left arm limited to muscles controlled by the C5 myelomaT2-weighted MRI
[20]73-year old manRight shoulder pain and herpes zoster eruptions over the C5 dermatomeTwo days later, he found it impossible to lift up the right arm
[21]A 48-year old femaleRash and vesicles over her left C5–7 dermatomesWeakness in the left deltoids and biceps muscles, and a diminished left biceps reflex; dyspnea with paradoxical abdominal wall movementChest CT normal, MRI-hyperintensity in the left anterolateral aspect of the spinal cord at C5 level1000 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 femaleBurning pain and rash in the left shoulder and its weaknessHyperalgesia skin in left C4–T2 dermatomes, left shoulder abduction weaknessEMG-denervation in left biceps, deltoid, brachioradialis and C5–6 paraspinal muscles; sensory and motor nerve-conduction, and somatosensory evoked potentials are normalMRI of cervical spine-degenerative changes with foraminal stenosis at C3–T1750 mg/d famciclovir per os, cervical epidural blocks with 20 mg triamcinolone twice during 4 weeks, 450 mg pregabalin and 10 mg nortriptyline, physiotherapyAfter 2 months, pain and weakness were significantly retracted
[23]88-year old man with corticosteroids in therapy because of polymyalgia rheumaticaRash in the right C3–5 dermatomeParesis in the right shoulder 2 days after the rashOral valaciclovir, acyclovir iv after paresis and methylprednisoloneParesis has gradually improved
[24]59-year old-female with carcinoma mammae dextri and lung metastasis, chemotherapy and radiotherapyPain and rash in whole right arm and scapulaPatient was able to adduct shoulder, flexor and extensor wrist and passively extend elbowEMNG-low amplitude action potentials in left superior radial and median, SNAPs and MUAPs in left deltoid musclePositive direct fluorescent antibody test for VZV, neck MRI-C4-C5-C6 levels protruded diskAcyclovir 10 mg/8 hours i.v. for 7 daysMotor activities recovered after 5 months with physiotherapy but postherpetic neuritis had a poor decrement

Table 1.

List of studies that reviewed herpes zoster infection and motor paresis of only upper limbs.

LiteratureDemographics, medical historySubjective complaintsNeurological impairmentsEMG findingsOther testsTreatmentCourse/outcome
[25]#1: 89-year old man, transient facial weakness 1 year earlier, conjunctivitis 6 months earlierPain and rash in right thigh and kneeWeakness and atrophy in right thigh, decreased sensation; reflexes diminished in knees and anklesDenervation in M. quadriceps, tibialis anterior, peroneus longus and gastrocnemiusX-ray: degenerative changes in LS spinePhysical therapy, galvanic electrical stimulationIncomplete recovery after 3 months
#2. 44-year old femalePain and rash over right distal arm in C7–8 dermatomeWeakness of proximal and distal muscles of right armFibrillations in right upper arm in C8 myotomeNot reportedPhysical therapyIncomplete recovery
#3. 74-year old womanPain and rash in T12-L1 dermatomesBulging of right abdomen and decreased muscle toneNot reportedNot reportedCorsetNot reported
#4. 58-year old woman, diabetesPain in left leg and rash over S1–2 dermatomesWeakness in left knee extensors, ankle dorsiflexors and plantar flexors; reflexes absent in left legNot reportedNot reportedNot reportedAble to walk after 5 weeks
[26]#1. 71-year old femaleRash along right ulnar border of forearm and handWeakness in forearm and hand musclesNot reportedNot reportedPhysical therapyReturn of strength but impaired fine movements
#2. 76-year old femaleRash over right shoulder and armWeakness in complete right arm, reflex absent in bicepsNot reportedNot reportedPhysical therapyFull recovery except in deltoid muscle, neuralgia over 3 years
#3. 83-year old femalePain and rash over right shoulderWeakness in deltoid, biceps; reflexes absent in bicepsNot reportedNot reportedHydrotherapy, exercisesWeakness in deltoid and biceps; neuralgia over 8 months
#4. 85-year old womanPain and rash in left shoulderWeakness in deltoid, supraspinatus biceps; reflex diminished in tricepsNot reportedNot reportedPhysical therapyIncomplete recovery, residual neuralgia died after 7 months
#5. 73-year old manPain and rash in right shoulder and in upper armWeakness in shoulder with deltoid fasciculationsNot reportedNot reportedIntensive physical therapyFull recovery after 7 months
#6. 74-year old womanRash over left thighWeakness of hip flexors, adductors, knee extensorsNot reportedNot reportedNot reportedFull recovery after 4 months
#7. 73-year old manRash over left shoulder and upper third of armWeakness in left armNot reportedNot reportedHydrotherapy, physical therapyFull recovery
#8. 81-year old femalePain and rash over C1–7 dermatomesWeakness of right serratus anterior, scapula wingingNot reportedNot reportedIntensive physiotherapyFull recovery in arm, residual pain after 5 months
#9. 75-year old femaleRash of outer part of right armWeakness in shoulder flexors and abductors, and in elbow flexorsNot reportedNot reportedIntensive physiotherapyFull recovery after 5 months; some pain persisted
[27]#1. 73-year old femaleRash in right C4–7 dermatomes; segmental severe paresis in C6, 7, 8DenervationCSF normal; myelogram normalSupportive treatmentFull recovery after 7 months
#2. 78-year old female, diabetesRash in right C5–7 dermatomesModerate weakness in C5–7 myotomesDenervationCSF normal; myelogram normalSupportive treatmentFull recovery after 9 months
#3. 67-year old manRash in left C5–6 dermatomesModerate weakness in C5–6 myotomesDenervationNot reportedSupportive treatmentFull recovery after 3 months
[28]#1. 84-year old manPain and rash in right L2–3 dermatomes; impaired sensation in L2–3 dermatomesWeakness in quadriceps and hip adductors, and flexors; reflexes absent in kneeFibrillations in hip flexors, vastus medialisNot reportedNot reportedNo recovery, died 6 years later
# 2. 66-year old manRash and pain in left shoulder and arm in C5 dermatomeWeakness in deltoid and spinatus musclesFibrillation, polyphasia and reduced interference patterns MUAPNot reportedNot reportedComplete recovery after 4 months
#3. 85-year old femaleRash, pain and impaired sensation in left in C4–5 dermatomesWeakness in deltoid, spinatus, biceps; reflexes absentFibrillations and reduced interference pattern in deltoidNot reportedNot reportedMinimal weakness after 4 years, residual neuralgia
#4. 83-year old. femalePain in outer part of right arm and digitis III-V, rash on posterior forearm and digitisWeakness in biceps, in triceps, fingers, hand intrinsics; absent reflex in triceps, depressed in biceps, supinatorFibrillations polyphasia and reduced interference patterns, motor median velocity reduced; absent median sensory neurogramNot reportedNot reportedIncomplete recovery of hand after 1 year, full recovery in triceps wrist extensors
#5. 64-year old manPain in right shoulder, rash in C5 distributionWeakness in deltoideus, supraspinatus; impaired sensation in C5 dermatome; reflexes absent in whole armFibrillations in deltoid, moderately reduced interference patterns in deltoid and supraspinatusNot reportedNot reportedFull motor recovery after 2 years
C#6. 52-year old femalePain and rash in left arm (C5–6 distribution); numbness in left thumbWeakness in left deltoid, spinatus, biceps; reflexes absent in biceps, supinatorReduced interference pattern in left deltoideus with polyphasic unitsNot reportedNot reportedIncomplete recovery after 7 months
#7. 77-year old manPain in right groin, rash in anterior and medial thighWeakness in hip flexors, adductors, knee extensors; reflex absent at kneeFibrillations in thigh muscles, reduced patterns, polyphasic unitsNot reportedNot reportedFull recovery after 4 months
#8. 73-year old manPain around knee, rash in medial aspect of left thighWasting and moderate weakness in quadriceps; reflex diminished at the kneeFibrillations slightly reduced interference pattern, polyphasic unitsNot reportedNot reportedFull recovery after 4 months
#9.69-year old manPain in right axilla, rash in medial arm and medial aspects of two fingersWeakness in hand intrinstics, sensory impairment in C8, T1 and T2 dermatomes; absent reflexesFibrillations polyphasia and reduced interference pattern, reduced motor velocity in ulnar nerve, absent sensory neurogramsNot reportedNot reportedMarked wasting and weakness in thenar, hypothenar, and intrinsic muscles after 1 year
[29]#1. 77-year old manPain and rash in left shoulderWeakness in shoulder, biceps, triceps, and wrist extensors musclesDenervation in deltoid and biceps, reduced MUAP recruitmentRoutine lab normal; CSF increased protein, normal cell count; VZV antibodies IgG positive IgM negative; cervical spine MRI normalValacyclovir 3 g for 7 days, Acyclovir iv 750 mg for 7 days, Methylprednisolone iv 500 mg for 3 days, Prednisol 60 mg and taperingFull recovery after 1 year
#2. 57-year old man with diabetesPain and rash over right wrist and groin unable to walkWeakness in right hip, thigh muscles, ankle dorsiflexors, patellar reflex diminishedDenervation in iliopsoas, quadriceps, tibial, F waves reduced in frequencyBlood tests normal; CSF increased protein and cell count; VZV antibodies IgG and IgM positive; lumbar spine MRI normalAcyclovir 750 mg for 7 days, Acyclovir iv 750 mg for 7 days, Methylprednisolone iv 1000 mg for 3 days, Prednisolone 60 mgFull recovery after 3 months
#3. 65-year old femalePain and rash in left shoulder and arm C5–6 dermatomesWeakness in shoulder, reflexes diminishedReduced interference with denervation in deltoid and C5 paraspinals musclesCervical spine MRI normalValacyclovir 3 gMarked recovery after 3 months
[11]#1.84-year old man with myelodysplastic syndromeR C5,6Weakness in C5,6 and atrophiaDenervation, polyphasia, normal conduction velocityModerate recovery after 6 months
#2. 85-year old femalePain and rash in left C5Weakness and atrophia in left C5,6 distributionDenervation, polyphasia, normal conduction velocityPoor recovery after 1 year and 7 months
#3. 79-year old femalePain and rash in right C 5,6Weakness in 5, 6 distributionDenervation, polyphasiaModerate recovery after 2 months
#4. 67-year old femalePain and rash in right C5,7 dermatomeWeakness in C5–8Denervation, polyphasiaGood outcome after 6 y + 8 m
#5. 82/FPain and rash in dermatome L1-S2 bilateralWeakness in projection L1-S1 bilateralModerate recovery after 3y + 8 m
#6. 80-year old manPain and rash in left C6,7Weakness in left C5-Th1Good recovery after 1y + 8 m
#7. 76-year old femalePain and rash in right C6,7 dermatomeWeakness in C6-Th1 distributionGood recovery after 7y
#8. 83-year old malePain and rash in right C8-Th1 dermatomeWeakness in C7, 8 distributionGood recovery after 5y + 9 m
#9. 7 year old femalePain and rash in right L4-S1 dermatomeWeakness in right L1,S1 distributionGood recovery after 5y + 4 m
#10. 72-year old femalePain and rash in right C5–7 dermatomeWeakness in right C5-Th1 distributionModerate recovery after 2 years and 10 month
#11. 43-year old male bone marrow transplantationRash and pain in right C8-Th1 dermatomeWeakness in C5-Th1 distributionUncertain 6y + 8 m
[30]#1. 69-year old female with diabetesPain and rash in right arm, rash over faceWeakness and impaired sensation in shoulder, reflexes diminished in right armNot reportedNot reportedSteroid, procaine, physical therapyAlmost full recovery
#2. 67-year old manPain and rash in low back and right leg in distribution of the sciatic nerveWeakness in right hip flexors; knee jerk absentNot reportedNot reportedParavertebral sympathetic blockUnknown
[31]#1. 63-year old manPain and rash in right shoulder, arm, and handWeakness in shoulder, elbow flexors, extensors, in hand muscles; reflexes absentDenervation in hand, motor amplitudes decreased, velocity normal; absent sensory neurogramsComplement fixing for antibodies VZV in sera was elevatedPhysical therapySignificant recovery except hand intrinsics
#2. 80-year old womanPain from left knee to foot, rash in L5-S2 dermatomes, urinary frequency and incontinenceWeakness in knee flexors, ankle flexors and extensors; reflexes in ankles absentNot reportedCSF: hyperproteinorachiaNot reportedIncomplete 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 1Pain-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 diminishedDenervation in 12 patientsIncreased protein and cell count in CSF of one patientNot reportedFull 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 manPain and rash in C5 myotomeNot reportedNot reportedNot reportedNot reportedMarked recovery
#2. 56-year old manPain and rash in L3 myotomeDiminished knee reflexesNot reportedNot reportedNot reportedMarked recovery
#3. 59-year old manPain and rash in C5 myotomeAbsent reflexesNot reportedNot reportedNot reportedFull recovery
#4. 70-year old manPain and rash in C5 myotomeAbsent reflexesNot reportedNot reportedNot reportedMarked recovery
#5. 71-year old manPain and rash in C5–6 myotomeAbsent reflexesNot reportedNot reportedNot reportedFull recovery
#6. 67-year old femalePain and rash in C7 myotomeNot reportedNot reportedNot reportedNot reportedFull recovery
#7. 91-year old womanPain and rash in T1 myotomeNot reportedNot reportedNot reportedNot reportedNo recovery
#8. 65-year old femalePain and rash in C5 myotomeAbsent reflexesNot reportedNot reportedNot reportedFull recovery
#9. 72-year old manPain and rash in C5 myotomeAbsent reflexesNot reportedNot reportedNot reportedUnknown
#10. 65-year old manPain and rash in L2–3 myotomeAbsent knee reflexesNot reportedNot reportedNot reportedMarked recovery
#11. 56-year old manPain and rash in L5 myotomeNot reportedNot reportedNot reportedNot reportedNo recovery
#12. 76-year old manPain and rash in C5 myotomeAbsent SJ and BJNot reportedNot reportedNot reportedModest recovery
#13. 62-year old womanPain and rash in L3 myotomeAbsent knee reflexNot reportedNot reportedNot reportedModest recovery
#14. 70-year old womanPain and rash in L3 myotomeAbsent knee reflexNot reportedNot reportedNot reportedFull 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 1Rash-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 1Weakness 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 patientsEMG 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 casesCSF proteins and cell count increased in 2 patientsNot reportedLimb recovery full in 55%, marked in 25%; residual pain in 8 patients (4 of them are with leg distribution)
[34]#1. 45-year old womanPain in right thigh, rash along the sciatic nerveDecreased sensation in L2-S1-S2 dermatomes, reflexes diminished; weakness in right ankle dorsi/plantar flexors7 years later- high amplitude and polyphasia on MUAPLumbar/pelvic X-ray and CSF normalNot reportedFull recovery at 3 months after the first episode; moderate after each of next relapses over 7 years
#2. 80-year old manPain in all right arm, rash over anterolateral part of armWeakness in all right arm, reflexes diminished, shoulder atrophy and subluxation of humeral headFasciculations, rare action potentials in deltoid, supraspinatus and biceps musclesNeck and spine X-ray: spondyloarthritic changesNot reportedIncomplete recovery in shoulder muscles after 1.5 years
[35]#1. 77-year old woman with hypertension and cardiomegalyPain and rash in right thigh, decreased sensation in L1–3 dermatomesWeakness and atrophy in quadriceps; knee reflex absentNot reportedNot reportedAnalgesics, physical therapyFull recovery after 3–4 months
#2. 65-year old manPain in left chest radiating in left arm, rash in left C5–6 dermatomesWeakness in proximal and distal muscles of left arm; reflexes absent in armNot reportedNot reportedSymptomatic treatmentFull recovery
#3. 74-year old female with osteoarthritisPain and rash in right shoulder (C5–7)Weakness in proximal and distal muscles of right armDenervation in upper and middle trunks of the brachial plexusChest X-ray normalNot reportedFull recovery after 6 months
#4. 67-year old femalePain and rash in distal right arm, sensory loss in C6–8/T1 segmentsWeakness and atrophy in hand muscles, reflexes diminishedSevere median and ulnar neuropathyCSF increased protein, no cellsAnalgesics, physical therapyFull recovery after 8 months
#5. 64-year old manRash over lower lateral chest on left side, T9–10 dermatomeWeakness in left rectus abdominis and oblique musclesDenervation in external oblique musclesNot reportedMild analgesia
#6. 80-year old man with DM, myocardial infarction, CVIRash and burning pain over right lower abdomenBulging of lateral and anterior abdominal wallFibrillations and positive waves in right abdominal muscles with later reinnervationNot reportedNot reportedFull recovery after 4 months
[14]58-year old femalePain over left side of neck, rash over left shoulder, lateral left arm, hand and wristWeakness of left shoulder and left deltoid muscleSplintAfter 2 months mass and power of the deltoid had increased
[36]#1. 53-year old manDisseminated vesicular rash and general malaiseDeveloped leg weakness in 2 days unable to stand; facial diplegia; reflexes depressedNormal in arms and legsDiagnosis of GBS was made and no specific treatment was givenOne year later was totally asymptomatic
#2. 69-year old man with asthmaPainful rash on right buttockBilateral facial and truncal weakness; weakness in legs all reflexes absent, loss of light touch and position senseMild cyanosis, reduced lung capacityReceived course of plasmapheresis5 months later only residual sign of mild reduction of hip power
[21]48-year old womanPain in left arm, rash over the C5 to C7 dermatomesWeakness in the left deltoids and biceps muscle and a diminished left biceps reflex; dyspnea1000 mg Valacyclovir orally three times daily for 7 daysAfter 2 years- without further complications; pain resolved in three months
[1]80-year old female, dementiaPain in left part of neckLeft facial palsy; difficulty walking with left lower limbGait was normal after 3 months, but left facial palsy remained complete
[37]#1. 61-year old manPainful rash on dorsum of right foot, sensory loss over the lateral right legWeakness of all parts of right leg, reduced right ankle reflexFibrillation and reduced MUAP in muscles innervated by the distal sciatic nervePCR of cutaneous crusted of right foot lesions was positive for VZVNo neurologic follow-up
#2. 69-year old manBurning pain in right upper limbWeakness in muscles innervated by right median nerve (right hand)Fibrillation potentials and reduced MUAPMRI of arm enlargement T2 signal within the median nerve with gadolinium contrastGabapentinIncomplete improvement- weakness with residual pain
#3 83-year old womanRash over right upper limbWeakness of right hand gripFibrillation and reduced MUAP in distribution of C7-T1 roots; conduction block in the median nerveAfter 11 months moderate residual weakness in median innervated muscles
#4 55-year old man with migraine, restless legs syndromeRash on knee and ankle, pain in left buttock, anterolateral thigh and kneeLeft knee stretch reflex absentFibrillation and reduced MUAP in left iliopsoas and rectus femoris musclesMRI enlargement and T2 signal in left femoral nerveNo clinical follow-up
[38]60-year old manPain in right leg, rash in anteromedial part of right thighWeakness, atrophy and fasciculation of right quadriceps; right knee reflex absentReaction of degeneration in the right quadriceps femorisThiamine hydrochloride 10 mg. three times a day orally, heat and electrical stimulationAfter 3 months incomplete recovery with atrophy of the thigh and fasciculations
[39]#1. 71-year old manPain on right side of chest weakness in both leg and right handDeep reflexes briskPhysiotherapy3 months later almost full recovery
#2. 58-year old manRash left side of neck and right upper armWeakness in both legs and right arm; reflexes absent in all limbsFibrillation potentials affecting muscles of right limbVelocities affecting in right median and popliteal nervesPhysiotherapy, tetracyclineAfter 11 months little residual deficit
[10]#1. 47-year old manRash in right shoulder and anterolateral armWeakness in right C5–6 myotomesDenervation in C5–6 distributionRecovery in 3 months
#2. 70-year old female with DMRash in right lateral arm and forearmAfter 20 days weakness in right C5–7 myotomesRight brachial plexopathy with denervationHyperintensity in spinal dorsal horns at C4–5No recovery after 2.0 years
#3. 63-year old male with DMRash of the right foot and a right L5-S1 plexopathyWeakness in right L5-S1 myotomesNo recovery after 1.8 years
#4. 80–90-year old with DMRash in neck firstAfter 22 days right C8 myotome weaknessDenervation in right plexus brachialisNo recovery after 1.9 years
#5. 87-year old male with DMRash in right lateral arm and forearmAfter 14 days right C6–8 myotome weaknessDenervation in right C6–8 distributionIncreased signal in the C6–8 nerve rootsNo recovery 1.0 year
#6. 60-70-year (nn gender)Rash in right buttocks and lateral calfWeakness in right L5 myotomeRight L5 radiculoplexopathy with denervationPartial recovery after 1.0 year
#7. 61-year old maleRash in left thumb, index finger and forearmAfter 15 days weakness in left C6–8 myotomesLeft C7 radiculopathy with denervationIncreased signal in median and radial nerve on MRINo recovery after 0.5 year
#8. 80-year old femaleRash in right shoulder, anterolateral arm and thumbWeakness in right C5 myotomesDenervation in right C5 distributionIncreased signal in the C5 nerve roots on MRIPartial recovery after 0.5 year

Table 2.

Lists of studies that reviewed herpes zoster infection and motor paresis of arms and legs.

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 M. quadriceps).

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).

LiteratureDemographics, medical historySubjective complaintsNeurological impairmentsEMG findingsOther testsTreatmentCourse/outcome
[58]40-year old women; in contact with a child with chicken poxRash on left buttock, burning pain in lower back, hip, and left legModerate weakness in left ankle plantar flexorsNormalBlood and CSF normal, chest and spine X-ray normalIntrathecal Methylprednisolone, analgesics, bed rest, sodium iodide and oxytetracycline i.v.; hydrocortisone ointment for rashComplete motor recovery after 9 months
[59]20-year old man, chicken pox at age 7Numbness in left foot; pain and rash over left gluteal regionWeakness in left ankle dorsiflexorsNot reportedNot reportedB1 and B12 injectionsFull recovery after 2 months
[60]31-year old man, diabetes, end stage renal failure with maternal renal allograft, autonomic neuropathy, blindnessRash and pain in right lower back, urinary retention, weakness in right leg, diminished sensation in lumbar and sacral segmentsThe right ankle plantar flexors (3/5 on MRC scale) and diminished ankle jerkNot reportedELISA test on HZV in sera was positive for IgM and IgG, which confirmed the presence of HZ infectionNot reportedUnknown recovery of leg function, some bladder recovery
[61]57-year old manRash over left T8–9 dermatomes, hyperalgesia in both legs, unable to walk, fasciculations in both legsFlaccid paralysis of both legs, reflexes absentNot reportedCPK: mild transient elevation; VZV complement fixation positiveNot reportedFull recovery after 3 months
[62]70-year old woman, with hypertensionPain in buttocks and legs; rash over left knee, thigh, buttocks; hyperesthesia in left leg; urinary incontinenceModerate weakness in knee flexors/ extensors, mild in ankle flexors; reflexes absent in left knee, ankleDiffuse denervation in leg and paraspinal muscles in L3–5 myotomesX-ray: mild narrowing of L5-S1 disc space; spine MRI: right L4–5 facet joint diseaseacetaminophen, oxytocin, bed rest, bupivacaine via L4–5 epidural catheter; parenteral meperidineNear complete recovery
[63]78-year old man, idiopathic myelofibrosis receiving cytoreductive therapySudden weakness in both legs; rash in right lower leg involving knee and thighBilateral leg paralysisNot reportedCSF: increased lymphocytes, monocytes, protein, glucose; PCR on VZV highly positive; X- ray: ileusWide 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 leukemiaBurning pain along the inner aspect of the right lower leg (6/10) and rash, weakness of the right footWeakness of the right ankle plantar flexors (3/5 on MRC scale) and diminished ankle jerkFibrillations and positive sharp waves in the right gastrocnemius and paravertebral muscles (S1 root); polyphasic MUAP during activation of right foot, nerve conduction was unchangedMRI of lumbosacral area: degenerative changes; ELISA test on VZV in sera positive for IgM and IgGPhysical therapy and oral gabapentin 900 mg/dayMotor 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 disease5-day history of paraesthesia starting in the right foot and ascending up the right lower limbVesicular rash in the L2/3 region with MRC grading 3/5 in the right hip flexorsMRI: unremarkableAcyclovir 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 colitisWorsening of her baseline residual muscle strength in the right lower limb shortly after herpes zoster eruptionEMG: denervation in L3-L4 and moderate axonal polyneuropathy affecting both lower limbs

Table 3.

Lists of studies that reviewed herpes zoster infection and motor paresis of only 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).

LiteratureDemographics, medical historySubjective complaintsNeurological impairmentsEMG findingsOther testsTreatmentCourse/Outcome
[66]M, 53-year oldHZ in C3,4 dermatomedyspnoea
[67]80-year old female, nephrectomy because pyelonephritisPain and rash over left shoulder and anterior part of chestweakness of left sholder and proximal muscles, atrophy of supraspinatus and infraspinatus muscles; dyspnoea on left hemidiaphragmdenervation of infraspinatus and supraspinatusRtg- paretic left hemidiaphragm
[44, 45]56-year old male with peptic ulcerrash and pain in later aspect of right arm and 3 middle fingersgeneral weakness of right arm and hand, atrophy of right part of neck, paralysis right hemidiaphragmX-ray and fluoroscopy-complete paralysis of rigth hemidiaphragm
[68]77, F
rheumatoid artritis, DM
Rash and pain in C3-5 dermatomeparalysis right hemidiaphragm
[46]72-year old female, RA, hypertensionRash and pain in right C3,4 dermatomedyspnoea
[69]66-year old femaleHZ of left C3,4,5after 12 months dyspnoeaX-ray –elevated left hemidiaphragm;
Radioscopy-paralysis of left hemidiaphragm
[50]56, male bronchitisHZ in 1st cervix dermatomenoneX-ray –elevated left hemidiaphragm
62-year old female astmaHZ in 1st cervix dermatomedysponea
67-year old femaleHZ in 1st cervixdysponea
57-year old femaleHZ in 1st cervixdysponea
[70]74-year old femaleHZ in C3,4dysponea
[71]80-year old female, hypertension,histerectomyHZ in C3-6Dyspnea, upper limb muscle
weakness
[72]74-year old male,
pulmonary emphysema
HZ in C2-5deltoid muscle
weakness, dyspnoea
[16]#2. 60-year old man with lymphatic leukemiaRash and hypersensitivity in right C5 dermatomecoughing; enlarged liver;Not reportedChest X-ray: elevated right hemidiaphragm, paralysis confirmed on fluoroscopiaComplete
Recovery
after 1
month
[56]74-year old maleHZ in 1st cervixcough,
dyspnea
not
alleviated
after 4 month
[73]79-year old male, hypertension, carotid endarterectomy, bypassHZ in cervix regiondyspnea,
orthopnea
After 12 months not alleviated
[56]74-year old maleHZ in cervixcough,
dyspnea
After 4 month not alleviated
[42]A 73-year old womanherpes zoster of left shoulder and proximal armweakness of left shoulder and proximal arm muscles 3 weeks after a diagnosis of herpes zosterinvolvement of the C5-6 myotomes and the upper trunk of the brachial plexusChest 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 manHZ on left shoulder and neckLeft hemidiaphragm paralysisAxonal changes in left nervus phrenicusCT and
X-ray- left hemidiaphragmatic relaxation
Acyclovirnot allevia
after 18 months
[75]HZ in left side of neckDyspnea after 3 monthsX-ray- left hemidiaphragm relaxation;
Pulmo-rary functions-restriction
[76]54-year old malethoracic herpes zoster,
1st chest, neck, bilateral shoulders
bilateral diaphragmatic paralysis associated with brachial neuritis,
orthopnea,
deltoid and biceps
brachii muscle
weakness
Fluoroscopynot allevia
after 19 months
[42]73-year old womanHZ and pain of left shoulderweakness of left shoulder and proximal arm muscles 3 weeks after HZ was diagnosed and paralysis of left hemidiaphragmEMG – 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 femalerash and vesicles over left C5-7 dermatomesweakness in the left deltoids and biceps muscles, diminished left biceps reflex. dyspnoea with paradoxical abdominal wall movementCT of chest normal, MRI-hyper-intensity in spinal cord at C5 level1000 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 dermatomeA chest X-ray elevated left diaphragmFamciclovir for 7 days at a dose of
750 mg per day.
After 14 months dyspnea and no alleviation
[48]43-year old manRash on right neck and apper right hemithorax C3-5hiccupsOn X-ray paralysis of the right hemidiaphragm, HIV +intravenous acyclovir and admitted to the hospital

Table 4.

Lists of studies that reviewed herpes zoster infection and motor paresis of diaphragmatic paralysis.

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 M. quadriceps).

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 13).

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 wallDemographics, medical historySubjective complaintsNeurological impairmentsEMG findingsOther testsTreatmentCourse/outcome
[81]73-year old man with L3 vertebral compression fracture and RA (Prednisolone)Rash and pain with blisters on his right flankT12 and L1 segmental paresis caused abdominal wall pseudohernia, scoliosis, and standing and gait disturbanceDenervation in right T12 myotomal muscles, and MUAP markedly decreasedOrthosis ExerciseAfter 4 months of rehabilitation, marked improvement
[82]72-year old manHerpes zoster infection in T11-T12 left dermatomesSegmental abdominal wall protrusionDenervation in left external oblique muscle and left paraspinal muscles at T11-T12 levelSSEPs- no response in the left side at T12 dermatomeAfter 3 months abdominal wall protrusion had completely resolved
[83]Abdominal wall postherpetic pseudoherniaMRI- increased signal intensity in abdominal wall muscles. Ultrasound- normalFull recovery
[43]62-year old maleCutaneous vesicular eruption on the left side of the abdominal wallAbdominal distention and paralytic ileus because of a visceral neuropathyX-rays and CT showed distended small bowelAcyclovir iv, oral Valacyclovir, Gabapentin 8 d after admissionFull 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 affectedIn 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 measuresComplete recovery with conservative measures occurred in 79.3% patients, with a mean time of 4.9 months
[84]58-year old manRash in area 9th to 11thProtrusion in the right abdominal wall with no painUltrasonography excluded the abdominal wall defectOral acyclovir, mecobalamin, and vitamin B1Disappeared after 2 months
[85]4 patientsInvolvement of posterior rami of spinal nerves in abdominal wall pseudoherniaIn 3 patients, EMG of paraspinal muscles showed denervation potentialsMRI-hyperintensity of these muscles on short T1 inversion recovery imaging
[86]57-year-old manRash and dull squeezing painBulge on his left flank 2 weeks after cutaneous changes

Table 5.

Lists of studies that reviewed herpes zoster infection and motor paresis of abdominal wall.

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 13). 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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Vesna Martic (April 1st 2020). Extracranial Herpetic Paresis, Human Herpesvirus Infection - Biological Features, Transmission, Symptoms, Diagnosis and Treatment, Ronaldo Luis Thomasini, IntechOpen, DOI: 10.5772/intechopen.90493. Available from:

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