52 Chroni, Howard, Panayiotopoul0s, Spencer Postgrad Med J: first published as 10.1136/pgmj.71.831.52 on 1 January 1995. Downloaded from Multiple sclerosis presenting as late functional deterioration after poliomyelitis
E Chroni, RS Howard, CP Panayiotopoulos, GT Spencer
Summary Case reports We describe five patients with previous poliomyelitis who developed multiple Patient 1 sclerosis (two laboratory supported A 25-year-old woman developed poliomyelitis definite and three clinically definite). The during pregnancy. She was extensively initial symptoms offunctional deteriora- paralysed during the acute illness but did not tion developed a mean of 30 years follow- receive ventilatory support. Following ing poliomyelitis. Initial functional rehabilitation she was left wheelchair-bound, deterioration was due to progressive limb with severe upper limb weakness and flaccid weakness or impaired mobility and in paraplegia. At the age of 40 she developed three cases this led to an initial diagnosis progressive increasing weakness of the right of post-polio muscular atrophy. The hand and arm manifest as a loss ofability to feed clinical diagnosis became apparent with herself, comb her hair or brush her teeth. Over the subsequent development of charac- the next eight years she had episodes of dip- teristic clinical features, including optic lopia, upper limb spasticity, weakness and nerve, brainstem, cerebellum, and spinal hyperreflexia. She was referred because of cord involvement. The occurrence of post-polio deterioration at which time she had multiple sclerosis in these patients bilateral internuclear ophthalmoplegia, vertical emphasises that late functional deterior- nystagmus, optic disc pallor, left relative ation may be apparently unrelated to afferent pupillary defect, bilateral upper limb previous poliomyelitis. Furthermore, spasticity and weakness in addition to the characteristic clinical features may be pre-existing wasting, spasticity and proprio- masked by the severe pre-existing ceptive loss in the right leg. She subsequently neuromuscular and orthopaedic impair- developed progressive bulbar failure before her ment leading to diagnostic delay and death from aspiration pneumonia in 1986. The confusion with the ill-defined clinical diagnosis of MS was clinical and based on the syndrome of progressive post-polio mus- occurrence of clear episodes of relapsing and http://pmj.bmj.com/ cular atrophy. remitting neurological symptoms and signs. Patient 2 Keywords: multiple sclerosis, poliomyelitis A 42-year-old woman had developed acute paralytic poliomyelitis when aged three months. She was extensively paralysed during
the acute illness and received artificial ventila- on September 29, 2021 by guest. Protected copyright. tion with an Iron Lung for six months. She was Introduction left with severe upper limb weakness, flaccid paraplegia and was wheelchair-bound. She had Many patients with previous poliomyelitis a transient episode of painful visual loss from develop post-polio syndromes (PPS) in which the left eye when aged 25 years. Nine years later a of she developed progressive weakness of both Lane-Fox Unit and late functional deterioration follows period Departments of relative stability.' New weakness or atrophy arms, frequency and urge incontinence. In Neurophysiology and may occur in the absence of any underlying addition to the preceding severe flaccid Neurology, Guy's and cause in progressive post-polio muscular weakness there were myoclonic jerks of the St. Thomas' Hospital atrophy (PPMA) which is a form of PPS. right arm and reduced spinothalamic sensory Trust, St Thomas' However, because of their existing disability, appreciation below the clavicle on the left. Campus, London, UK Visual- and brainstem-evoked potentials were E Chroni post-polio patients are also particularly suscep- RS Howard tible to the effects of intercurrent orthopaedic, normal but there was delayed central motor CP Panayiotopoulos neurological, respiratory or general medical conduction bilaterally. She developed episodic GT Spencer factors.2 Diagnostic difficulty can arise because increasing weakness and paraesthesia of both the characteristic features of secondary causes arms with clear functional deterioration, Correspondence to of deterioration may be masked by the pre- urinary incontinence and chronic constipation. Dr RS Howard, Department ofNeurology, Guy's and St existing impairments leading to an erroneous In addition to the severe upper limb weakness Thomas' Trust, St Thomas' diagnosis ofPPMA. We report five cases from a and flaccid paraplegia there was a low thoracic Campus, London SEl 7EH, personal series of 589 patients (GTS) in whom level to vibration sense and joint position sense UK post-polio deterioration was due to multiple appreciation. A diagnosis of clinically definite Accepted 27 July 1994 sclerosis (MS). MS was made. Magnetic resonance imaging MS following poliomyelitis 53 Postgrad Med J: first published as 10.1136/pgmj.71.831.52 on 1 January 1995. Downloaded from
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Figure Multiple sclerosis presenting as late funmctional deterioration after poliomyelitis
(MRI) showed multiple high-signal periventri- and an extensor plantar response. Visual- cular lesions on T2-weighted images. evoked potentials showed delayed latency bilaterally and central motor stimulation Patient 3 showed delay in the central motor pathways on A 48-year-old woman was referred for further the left. MRI of the cervical cord showed investigation of post-polio functional significant narrowing at the C6-7 interspace deterioration. She had poliomyelitis at the age but T2-weighted images showed multiple high of 8 years and was left with severe flaccid signal lesions in a predominantly periventri- weakness of the right leg which was subse- cular distribution. quently amputated. She remained wheelchair- http://pmj.bmj.com/ bound. Between the ages of 39 and 40 she had Patient 5 three episodes ofweakness ofthe right arm and A 43-year-old woman had developed polio leg. Examination showed a right facial weak- when aged five years and was left with a flaccid ness, asymmetrical bilateral wasting without paraparesis although she was able to walk short fasciculations, variable flaccid weakness pre- distances with leg calipers. At the age of 40 dominantly in a pyramidal distribution on the years she developed transient episodes of
right with pathologically brisk reflexes and a weakness and numbness affecting the left leg. on September 29, 2021 by guest. Protected copyright. global flaccid weakness of the left leg. Visual- The following year she had an episode of and auditory-evoked potentials were normal, transverse myelitis with complete sensory loss somatosensory-evoked potentials showed delay below the waist and urinary incontinence and following left arm stimulation and an absent frequency. There was a T7 sensory level to all response on the right. Cerebrospinal fluid modalities. There was deterioration in the (CSF) showed positive oligoclonal bands and paraparesis and both plantar responses were MRI showed multiple small focal areas of high extensor. Investigation showed positive oligo- signal on T2-weighted images in a predomi- clonal bands in the CSF, MRI brain scan nantly periventricular distribution. showed multiple areas of high signal in a periventricular distribution on the T2- Patient 4 weighted images. She has subsequently A 44-year-old man developed polio when aged developed an episode of left optic neuritis. 18 months, leaving him with flaccid leg weakness. He was referred at the age of42 years having developed intermittent diplopia, left Discussion foot drop and increasing incoordination of the left leg. On examination there was horizontal The Lane Fox unit is a national referral centre nystagmus, mild distal weakness of the left for patients with previous poliomyelitis and hand, left-sided ataxia and dysdiadochokinesis, this population of 589 patients forms a selected absent abdominal reflexes, severe flaccid right group because of the severe nature of their leg weakness and moderate pyramidal weak- underlying disability and late functional ness of the left leg with left-sided hyperreflexia deterioration.2 In the present series of five 54 Chroni, Howard, Panayiotopoulos, Spencer
Post-polio syndromes Learning points/summary box
* post-polio muscular atrophy * late functional deterioration may be unrelated Postgrad Med J: first published as 10.1136/pgmj.71.831.52 on 1 January 1995. Downloaded from * syringomyelia to previous poliomyelitis * radiculopathy/myelopathy due to cervical * characteristic clinical features may be masked spondylosis by pre-existing neurological signs
patients with multiple sclerosis (two These included corticobulbar swallowing laboratory-supported definite and three clini- difficulties, spastic paraparesis, hyperreflexia cally definite3) initial functional deterioration and extensor plantar responses. The cause of was due to progressive limb weakness or im- these signs was attributed to a polioencephalitis paired mobility and in three cases this led to an accompanying the original poliomyelitis or as initial diagnosis of PPMA. The clinical diag- part of the progressive post-polio syndrome. nosis became apparent with the subsequent Martyn et all'"' argue that subclinical infection development of characteristic clinical features with poliomyelitis might be severe enough to including optic nerve, brainstem, cerebellum deplete motor neurones and that motor neur- and spinal cord involvement. One patient had one disease might then develop as a conse- relapsing and remitting multiple sclerosis and quence of further motor neurone loss due to the remaining four secondary progressive MS.4 ageing or a second insult. However, analysis of In one patient a diagnosis of clinically definite morbidity and mortality rates for motor MS was made and no further investigations neurone disease in Scotland shows no evidence were undertaken because of her severe that motor neurone disease is caused by early disability. In the remaining four patients, asymptomatic polio infection or that polio oligoclonal bands were present in two of the vaccination offers protection against this condi- three patients in whom lumbar puncture was tion. 2 Furthermore in a literature review performed, evoked potentials were abnormal in Armon et all3 suggested the paucity of reports four and MRI scan showed multiple periven- of classical amyotrophic lateral sclerosis tricular white matter lesions in four patients. developing in survivors of paralytic polio- A number of neurological disorders may myelitis might indicate that antecedent cause late deterioration in patients with poliomyelitis has a protective role against the previous poliomyelitis (see box).6'7 Other development of amyotrophic lateral sclerosis. neurological disorders described in patients The occurrence of MS in these patients with previous poliomyelitis include cerebro- emphasises that late functional deterioration vascular disease, epilepsy, cerebral and spinal apparently may be unrelated to previous tumours; however, there is no suggestion of an poliomyelitis. Furthermore characteristic increased prevalence of these disorders.2 clinical features may be masked by the severe Salazar-Grueso8 described neurological dis- pre-existing neuromuscular and orthopaedic orders in 6/21 patients, including one patient impairment leading to diagnostic delay and with clinically definite MS. The presence of confusion with the ill-defined clinical syn- http://pmj.bmj.com/ upper motor neurone signs has also been noted drome of progressive post-polio muscular in 13/150 patients seen in a post-polio clinic.9 atrophy.
1 Dalakas MC, Elder G, Hallett M, et al. A long-term 8 Salazar-Grueso EF, Grimaldi LME, Roos RP, Variakojis R, follow-up study of patients with post-poliomyelitis Jubelt B, Cashman NR. Isoelectric focusing studies ofserum neuromuscular syndromes. N Engl J Med 1986; 314: and cerebrospinal fluid in patients with antecedent
959-63. poliomyelitis. Ann Neurol 1989; 26: 709-13. on September 29, 2021 by guest. Protected copyright. 2 Howard RS, Wiles CM, Spencer GT. The late sequelae of 9 Salazar-Grueso EF, Cashman NR, Maselli R, Roos RP. poliomyelitis. Q J Med 1988; 251: 219-32. Upper motor neurone (UMN) findings in patients with 3 McDonald WI, Silberberg DH. The diagnosis of multiple antecedent poliomyelitis. Neurology 1987; 37: 215. sclerosis. In: WI McDonald, DA Silberberg, eds. Multiple 10 Martyn CN, Barker DJP, Osmond C. Motoneurone disease sclerosis. London: Butterworth, 1986; pp 1-11. and postpoliomyelitis in England and Wales. Lancet 1988; i: 4 Thompson AJ, Kermode AG, MacManus DG, et al. Pat- 1319-22. terns of disease activity in multiple sclerosis: clinical and 1 1 Martyn CN. Poliovirus and motor neurone disease.J Neurol magnetic resonance imaging study. BMJ 1990; 300: 631-4. 1990; 237: 336-8. 5 Pezeshkpour GH, Dalakas MC. Long term changes in the 12 Swingler RJ, Fraser H, Warlow CP. Motor neurone disease spinal cords of patients with old poliomyelitis: signs of and polio in Scotland. J Neurol Neurosurg Psychiatry 1992; continuous disease activity. Arch Neurol 1988; 45: 505-8. 55: 1116-20. 6 Feve A, Wallays C, Nicolle MH, Guillard A. Syringomyelia 13 Armon C, Daube JR, Windebank AJ, Kurland LT. How as a late complication of paralytic poliomyelitis. Neurology frequently does classic amyotrophic lateral sclerosis develop 1992; 42: 1421-2. in survivors of poliomyelitis? Neurology 1990; 40: 172-4. 7 Carroll SD. Syringomyelia as a possible complication of poliomyelitis. Neurology 1967; 17: 213-5.