MOTOR COMPLICATIONS OF HERPES ZOSTER

Transcription

1 Age and Ageing (1979), 8, 75 MOTOR COMPLICATIONS OF HERPES ZOSTER M. S. PATHY Department of Geriatric Medicine, University Hospital of Wales, Heath Park, Cardiff CF4 4XW Summary Motor involvement is uncommon in herpes zoster. Of 14 subjects observed over an arbitrary period of five years, nine had flaccid limb paralyses, involving the upper limb in eight patients. Recovery was not always complete and occurred over a period of nine weeks to four years. INTRODUCTION The causative agent of herpes zoster is a neurotropic virus with a marked predilection for involvement of the posterior root ganglia, but may affect the central nervous system in general (Kendall 1957). The uncommon occurrence of various motor paralyses is well documented. Sir William Broadbent (1866) first described paralysis of the muscles of the shoulder and upper arm following herpes zoster. Taterka & O'Sullivan (1943) undertook a comprehensive review of the literature and collected 42 cases of herpes zoster associated with motor involvement, other than of the cranial nerves, to which they added two of their own cases. Thomas & Howard (1972) reported 80 cases of limb paresis in a study of the world literature. Halpern & Covenner (1949) first described diaphragmatic paralysis complicating zoster and in 1970 Shivalingappa reported a seventh case of diaphragmatic paralysis. The age structure of a population must be a significant determinant of the prevalence of overt zoster. Hope-Simpson's (1965) study of a family practice of 3500 people in England detected 3.4 patients with zoster per 1000 persons per annum over a 16-year period. McGregor (1957) found an annual rate of 4.8 per 1000 in a general practice in Scotland. In a review of 666 patients with herpes zoster in Sweden (Hellgren & Herle 1966), no case with motor involvement was recorded. Materials and Methods This report emphasizes the uncommon but important complication of flaccid paralysis of a limb, particularly the upper limb, often with prolonged disability. During an arbitrary period of the last five years, 14 subjects with motor paralysis were under our care. Three had facial palsy of the Ramsey-Hunt syndrome. One female had a right 6th nerve paralysis following herpes zoster of the 1st division of the 5 th nerve which persisted unchanged over the period of three years, and another female patient had solitary ptosis of the right eyelid which persisted unchanged for two years following herpes zoster of the 1st division of the 5th nerve. The remaining nine subjects had flaccid limb paralyses and these are described in detail.

2 76 M. S. Patky Case 1 Mrs M.C., aged 71, developed a vesicular rash along the ulnar border of the right forearm and right hand which eventually healed. Five days after the onset of the eruption she noticed marked weakness of the right forearm and hand. She was first seen as an out-patient two months after the onset of the rash. Pink scars of recent herpes zoster extended along the ulnar and anterior border of the upper limb from just above the elbow to just proximal to the palm of the right hand. Power and flexion were moderately diminished and flexion of the wrist was considerably impaired with no flexor power in the 4th and 5 th fingers. Over the next five years she developed modest wasting of the ulnar group of muscles, but there was a gradual return of power in the previously weak and paralysed muscles. Fine movements did not return. Due to determination and perseverance the patient learned to write surprisingly well with the left hand. Case 2 Mrs A.M.H., aged 76 years, developed a vesicular rash over the right shoulder and arm while on holiday. Ten days later she rapidly developed progressive weakness of the right upper limb which was diagnosed by a family doctor as a stroke. She was initially seen in the out-patient clinic four weeks after the onset of the rash. Examination revealed an alert, active woman with pink scars over the shoulder and outer aspect of the arm to just above the elbow, diagnostic of herpes zoster. She had complete paralysis of the right deltoid and flexors of the elbow, with weakness of the flexors of the fingers. The right bicep's jerk was absent. She complained of marked pain in the affected shoulder and arm. Power returned in the fingers within five weeks and in the flexors of the arm within six months. Marked wasting developed in the right deltoid and muscle power remained very weak for a year. She was seen at regular intervals for about four years. Improvement in muscle bulk in the affected deltoid occurred progressively, but abduction at the right shoulder remained significantly weaker than on the opposite side despite intensive physiotherapeutic measures. Post herpetic neuralgia continued to be severe during the first three years, but significantly diminished in the fourth year. Case 3 Mrs G.S., aged 83 years, presented with severe pain and a rash over the right shoulder and the following day developed severe weakness at the right shoulder. Examination revealed a well preserved and intelligent old lady with early vesicles of herpes zoster over the right shoulder and arm to just above the elbow. Though voluntary abduction was impossible, some contraction was palpable in the posterior part of the deltoid. Weakness in the right biceps was moderately severe and the bicep's tendon reflex was absent and supinator reflex diminished in comparison with the opposite side. She experienced severe post-herpetic neuralgia and when seen eight months later pain remained a major symptom. Wasting in the right deltoid had improved considerably, but weakness of the right deltoid and biceps remained greater than on the opposite side. Case 4 Mrs M.E.B., aged 85, developed pain in the left shoulder followed three days later by weakness in the shoulder and upper arm. The following day she noticed a rash over the affected area. Examination revealed an alert, active lady with an early vesicular rash of herpes zoster and moderately severe weakness of the left deltoid, supraspinatus and left biceps. The left tricep's tendon reflex was diminished, but the bicep's reflex was normal. Following intensive physiotherapy, muscle power improved sufficiently within one month so that there was no significant functional disturbance, but slight impairment in power was detected in the right deltoid for six months. She had a moderate post herpetic neuralgia despite early application of topical idoxuridine. She died seven months after first being seen. Case 5 Mr C.C.H., aged 73, developed a severe pain in the right shoulder which he ascribed to hoeing his garden in a wind. Two days later he developed a rash over the shoulder, which he diagnosed

3 Motor Complications of Herpes Zoster 77 as a reaction to the liniment that he applied to his shoulder to ease the pain. The rash progressed to involve the upper third of the arm and six days later he found dressing difficult due to weakness of the right shoulder. When seen in the out-patient department nine days later he had typical pink scars of herpes zoster over the deltoid area and the upper third of the arm. There was no movement in the right deltoid and moderate fasciculation was evident. His tendon jerks were normal. Over the following ten weeks he developed considerable wasting of the right deltoid. He persevered with vigorous exercises and seven months later muscle bulk in the right deltoid was comparable with that on the left side and power was normal. Case 6 Miss M.H., aged 74, noticed a rash over the front of the left thigh. Five days later she developed weakness of the left lower limb. Examination on the fifth day revealed a segmental distribution of scabs of herpes zoster over the upper half of the anterior aspect of the left thigh. Moderately severe weakness was present in the left hip flexors and hip adduction, and knee extension was slighdy weaker than on the right, but advanced osteoarthrosis of the left knee made accurate assessment of the true power of extension at the knee uncertain. Within nine weeks the power in the left lower limb was equal to that in the right and the patient was fully mobile with the walking cane that she had used for three years due to her oateoarthrosis of the left knee. Case 7 Mr J. H., aged 73, developed a vesicular rash of herpes zoster over the left shoulder and upper third of the arm. Seven days later he noticed inability to lift the arm. He was seen as an out-patient 13 weeks after the onset of the rash. He was a thin active man who complained bitterly that the weakness of his shoulder and arm was making him dependent on his wife for many personal activities. The fading pink scars of herpes zoster were evident over the anterior and posterior aspect of the left shoulder and outer aspect of the upper third of the arm. Movements at the left shoulder were very weak and he was just able to flex the arm against gentle resistance. He was treated with hydrotherapy and resisted exercises, and within eight weeks he had made considerable recovery and 15 weeks later power appeared equal to that in the opposite upper limb. No muscle wasting was detectable at any time. Case 8 Mrs M.G.J., aged 81, developed a rash of herpes zoster over the right hemithorax posteriorly extending from approximately Dl to D7. She was admitted to hospital via the emergency bed service one week later due to severe abdominal pain and vomiting, later proved to be due to a hiatus hernia with oesophageal reflux. She had a mixed eruption of flattened vesicles and scabs. She complained of marked pain at the site of the rash. Ten days later she complained of difficulty in combing her hair and raising her right arm above the head. She was found to have marked weakness of the right serratus anterior with slight winging of the scapula. There was no correlation between the site of the eruption and the nerve supply to the only muscle which appeared clinically affected. With intensive physiotherapy muscle weakness improved until no difference could be detected in the previously affected limb and the left side by the eighth month. Case 9 Mrs A.H., aged 75, was first seen in the out-patient clinic three weeks after an episode of herpes zoster involving the outer aspect of the right arm. Three days after the onset of the rash the patient rapidly developed severe weakness at the right shoulder, noted as severe weakness of flexion and abduction of the arm with moderate weakness of flexion at the elbow. She was treated with intensive physiotherapy and when last seenfivemonths later, power in the right upper limb had returned to normal, but she complained of severe pain in the region of herpetic scarring.

4 78 M. S. Pathy DISCUSSION Since Garland (1943) suggested that herpes zoster is the consequence of activation of the latent varicella virus, numerous virological studies (Wheeler 1953, Wheeler & Coons 1954, Wheeler & Whitton 1958, Wheeler, Whitton & Bell 1958) have amply confirmed this monistic concept. There is no definitive evidence as yet to indicate whether the latency period of the varicella-zoster virus is due to a few virions being continuously elaborated or due to the persistence of viral genome, but without viral replication. In either event the development of clinical zoster indicates a phase of rapid varicella-zoster replication in the presence of diminished host immunity. Hope-Simpson (1965) proposed that most sensory ganglia contain latent varicellazoster virus following clinical varicella and that the immunity threshold could eventually diminish sufficiently to permit selective varicella-zoster virus replication in a sensory ganglion. Subsequent migration down a sensory nerve and then zonal release around sensory nerve endings in the skin would produce the characteristic segment picture of herpes zoster. This thesis fails to explain the not uncommon widely distributed lesions, euphemistically referred to as herpes zoster with aberrant vesicles, which are so highly suggestive of a viraemic phase. C.S.F. pleocytosis and the isolation of varicella-zoster virus from C.S.F. (Evans & Melnick 1949, Gold & Robbins 1958) in acute herpes zoster, indicate a more widespread C.N.S. infective process than is commonly conceded. These findings make some of the less common non-sensory neurological manifestations of zoster more comprehensible. The varicella-zoster virus has not been isolated from dorsal root ganglia at autopsies on varicella immune subjects (Weller, 1976). The varicella-zoster virus has, however, been demonstrated in affected nervous tissues by ultrastructural (Eseri & Tomlinson 1972, Ghatak & Zimmerman 1973) and specific immunofluorescent (Eseri & Tomlinson 1972) procedures. Hardy (1876) first described an ascending myelopathy subsequent to herpes zoster. McCormick et al. (1969) demonstrated type A intranuclear bodies in the cord and brain of two patients infected with varicella-zoster virus, but dying of a reticulosis. Hogan & Krigman (1973) reported a case of myelitis due to herpes zoster, but without evidence of malignancy, shown at autopsy to have type A inclusions and particles in the cord and later the varicella-zoster virus was isolated from the cord. Rose et al. (1964) discussed the clinical and post-mortem findings of a patient with evidence of encephalomyelitis. The patient developed herpes zoster over the lower abdomen followed by lower limb paresis and signs of encephalitis. At autopsy widespread patchy inflammatory changes were noted in the cord and brain. Brain (1969) indicated that muscle involvement of segmental distribution was a rare manifestation of herpes zoster and was probably due to extension of the infection from the posterior to the anterior horns. Indeed Lhermitte & Nicholas (1924) showed histological evidence of demyelinization of the posterior horns and intense inflammatory changes in the anterior horns in the cord of a patient who died seven weeks after an acute episode of herpes zoster. Kendall (1957) suggested that the less typical features of herpes zoster were best explained on the basis of zoster being a disease of the C.N.S. in addition to the posterior root. Head & Campbell (1900), Wohlwill (1924), Lhermitte & Vermes (1930) and Denny Brown et al. (1944) have also advanced evidence for zoster being a disease of the motor portion as well as the more commonly afflicted sensory

5 Motor Complications of Herpes Zoster 79 component of the nervous system. It remains debatable whether motor paresis results from involvement of the anterior horn cells or the anterior nerve roots. The general picture has points in common with acute anterior poliomyelitis in which there is muscle paralysis due to a rapid destruction of a variable number of anterior horn cells, and partly due to functional impairment in the surviving cells with slow recovery (Russell 1956). Muscle fibres innervated by these surviving cells can be induced to hypertrophy by active resisted exercises (Russell & Fischer-Williams 1954). Kendall (1957), Thomas & Howard (1972) and Weiss et al. (1975) have variously pointed out that the segmental distribution of the eruption and the anatomical site of the muscle paresis are not always coincidental. This finding militates against the simplistic concept of direct segmental transmission of the disease from posterior to anterior horns. Flaccid paralysis complicating herpes zoster is most commonly found in the upper limbs. In the review by Taterka and O'Sullivan (1943) involvement of the upper extremity was noted in 45.7% of patients and in the trunk muscles in 48.9% of subjects. Only six cases of lower limb paresis were reported up to that time. Thomas & Howard (1972) undertook a retrospective study of 1210 patients with recorded evidence of herpes zoster seen at the Mayo Clinic over a ten-year period. Sixty-one had segmented zoster paresis, but upper limb muscles were involved in 16 patients, lower limb muscles in 15 and abdominal muscles in two patients. Unless routine electromyography and nerve conduction studies are undertaken, minor degrees of trunk muscle weakness are likely to be overlooked. Of 117 cases of herpes zoster admitted to the Tel-Aviv Municipal Government Centre over a period of ten years, Weiss et al. (1975) recorded six patients with limb paresis involving the upper or lower limb with equal frequency. Of the 11 cases reported by Kendall, six had cranial nerve paresis and five had upper limb paresis. Parkinson (1948) reported one subject with motor paresis in the distribution of C5. Our subjects with motor paresis recorded over a period of five years were seen among 140 patients with herpes zoster 58 in-patients and 82 out-patients. Brain (1969) suggested that paralysis might sometimes be permanent, but that recovery took place over a period of several months. Taterka & O'Sullivan (1943) reported that 16% of subjects recovered with no residual abnormalities over a period of 6 to 12 months, but that in 81.7% of patients some residual muscle wasting and weakness persisted for over a year or more. In a questionnaire follow-up (time span two months to 11 years), of patients seen at the Mayo Clinic, Thomas & Howard (1972) noted that 55% of respondents reported recovery and 25% significant improvement. Good recovery was obtained more often in the proximal than in the distal muscles. Of our 9 patients, five recovered fully within six months, but the other four showed some muscle weakness during a period of up to two to four years. The relationship between recovery and an active resisted muscle exercise programme could not be validly evaluated in the small number of patients under our care. ACKNOWLEDGMENT I am grateful to my colleague Dr D. A. O. Sutton for permission to quote Case No. 9. REFERENCES BRAIN, W. R. (1969) Diseases of the Nervous System 7 th edn. London: Oxford University Press. BROADBENT, W. H. (1866) Case of herpetic eruption in the course of branches of the brachial plexus followed by partial paralysis in corresponding motor nerves. Br. Med. J. 2, 460.

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