Post-Encephalitic Parkinsonism: Current Experience
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.44.8.670 on 1 August 1981. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry, 1981, 44, 670-676 Post-encephalitic Parkinsonism: current experience DAVID RAIL, CARL SCHOLTZ, AND MICHAEL SWASH From the Departments of Neurology and Neuropathology, The London Hospital and The London Hospital Medical College, London S U MMARY The diagnosis of post-encephalitic Parkinsonism is rarely entertained today. In this paper we discuss eight recent patients, six of whom presented in the last 20 years, that conform to the diagnosis of encephalitis lethargica. The clinical features by which the diagnosis may be made are defined so that a contemporary definition of this disorder may be attained. Encephalitis lethargica occurred in epidemic pro- Doherty'5 found that 13% of a series of 83 Parkin- portion in Europe and other countries between sonian patients in Australia gave a history con- 1915 and 1930.1 2 It was an acute illness, usually sistent with previous encephalitis lethargica, and accompanied by fever, characterised by somno- a further 10% had experienced other encephali- lence with ophthalmoplegia and sometimes with tides. Only one of 50 non-Parkinsonian patients other focal neurological disturbances such as gave such a history. However, this apparent associ- Protected by copyright. hemiplegia or aphasia. In a second form of the ation has not been the experience of others.'6'-8 disease hyperkinesis, chorea and myoclonus, and Before the epidemic of encephalitis lethargica, inversion of the sleep rhythm occurred and in a Parkinsonism was very rare before the age of 40 third type an acute Parkinsonian syndrome, some- years.2 Willige,'9 found reports of only 20 cases times with a catatonia-like stupor, was observed.' beginning before the age of 30 in the 60 years Many other symptoms were recorded during the before 1910. Nonetheless, it seems important to epidemic and, despite contemporary reviewsl'5 it recognise that a form of encephalitis still exists is difficult to 'be certain, in the absence of a in which Parkinsonism occurs as a residual deficit, specific diagnostic test, that these apparently di- since only when the features of this modern post- verse neurological syndromes were variations of encephalitic extrapyramidal disease have been de- the same entity. Wilson in particular expressed fined can it be recognised, and investigated. this view: "There is reason to believe the en- cephalitis is not identical at all times and in all Patients localities, and that it may cover states of dis- similar aetiology.""l Case 1 (PT 335618) In 1961 this 20-year-old woman became acutely ill In the years since encephalitis lethargica ceased http://jnnp.bmj.com/ to be endemic it has generally been held that post- with headaches, fever, drowsiness, and vomiting. During this illness, which lasted several weeks, her encephalitic Parkinsonism is an uncommon dis- left arm and leg became weak and numb. Investi- order. However, even before the epidemic, sporadic gations including a right carotid angiogram, an air cases similar to encephalitis lethargica occurredl-3 encephalogram and CSF examination were normal. and, in recent years, there have been several She improved at first !but during the next three years isolated reports of cases thought to be examples a pronounced dystonic bradykinesia developed with of newly-acquired post-encephalitic Parkinson- prominent cogwheel rigidity. There was a slight rest- ism. "3 These reports lend some support to the ing Parkinsonian tremor, more marked in the left- on October 2, 2021 by guest. suggestion that idiopathic Parkinsonism itself sided limbs. The tendon reflexes were normal and both might be caused by encephalitis, perhaps acquired plantar responses were flexor. There was no sensory impairment. By 1967 her face and her right arm and years earlier.'4 Indeed, Eadie, Sutherland and leg were severely affected. At this stage the EEG showed irregular theta and delta waves, and sharp Address for reprint requests: Dr M Swash, The London Hospital, elements bilaterally. Wilson's disease was excluded, London El 1BB, UK. and she had never been treated with neuroleptic drugs. Accepted 22 May 1981 She failed to respond to treatment with anticholinergic 670 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.44.8.670 on 1 August 1981. Downloaded from Post-encephalitic Parkinsonism: current experience 671 drugs and levodopa and by 1970 she was wheelchair- upward gaze and of conjugate gaze to the left: down- bound and unable to look after herself without help. ward gaze was normal. There was a marked dysarthria, with cogwheel tone and a slight Parkinsonian resting tremor in the arms. All limbs were weak and both Case 2 (VM 733224) plantar responses were extensor. The EEG was In 1976 this 29-year-old man was admitted to another diffusely abnormal with a large amount of irregular hospital after a bout of "influenza," an illness which theta waveforms and occasional delta activity. The consisted of headache, fever, malaise and cough of a C2SF contained 4 white cells/mm3. CT scan of the few days duration. Two weeks later, while still in head was normal. Virological studies, including titres hospital he complained of a general feeling of "restless- of antibodies to influenza A and B, parainfluenza, ness within myself" which was relieved by walking. mumps and V virus and to mycoplasma pneumoniae Mental concentration was impaired. He was treated were normal in both blood and CSF. After a two- with diazepam but became progressively slowed and week period of observation, during which there was no catatonic. He was transferred to a psychiatric hospital improvement, he was treated with Sinemet 110, 1 tablet where it was thought that he was depersonalised and twice daily increasing to Sinement 275, 1 tablet four electroconvulsive therapy was started. Neuroleptic times daily after 2 weeks. Within 3 weeks of starting drugs were not used. However, there was no improve- he could sit out of bed and talk coherently, although ment after two treatments and he was transferred to his speech was still slow and slurred. When re- The London Hospital. On admission marked Parkin- examined at that time his pupils were midsize and sonian features were observed. He was strikingly did not respond to light, although they responded to akinetic with a fixed expression and there was cog- accommodation. Pursuit gaze movements were slowed wheel rigidity and bradykinesia of all limbs. There and interrupted by jerky saccades. Voluntary upward was a resting distal Parkinsonian tremor in both arms, gaze was impaired but doll's-head eye movements were which disappeared during movement. Tremor was full. His facies was immobile and there was marked less evident in the legs. The tendon reflexes were akinesia of the limbs, with cogwheel rigidity and brisk, but the plantar responses were flexor. There resting tremor in both arms. The right plantar response Protected by copyright. was no other neurological signs. Despite the akinetic flexor and the left equivocal. expression and posture there was pronounced aka- was thisia, so that he could not sit in one place for more than a few minutes. Investigations, including a CSF Case 4 (AM 509075) examination and an isotope brain scan were normal. This 44-year-old man was admitted to The London He showed no response to levodopa but improved Hospital in 1969 two weeks after the onset of an with bromocryptine 60 mg daily and by June 1976 illness consisting of persistent inappropriate laughing, his bradykinesia was much less marked. Blood and followed by fever, drowsiness and difficulty dressing. CSF titres for antibodies against influenza A and B, These symptoms continued for two days, when he had parainfluenza, adenovirus, measles, mumps and mumps a convulsion and was admitted to another hospital. V, H simplex, varicella zoster, coxiella burneti, At that time he was noted to be "drowsy with slow psittacosis-LGV and mycoplasma pneumoniae were movements." The CSF was normal. After several all within the normal range (1: 32). days his mood changed to one of aggression and it was noted that he had difficulty looking up and down. There was no history of administration of neuroleptic Case 3 (PS 49639) drugs. On admission he was impassive but could be This 50-year-old man was admitted to hospital in aroused to inappropriate jocosity. There was bilateral 1973 two weeks after the onset of his illness. Soon ptosis. On gaze filation he tended to look tonically after arriving in Majorca on holiday he had become downwards, with exaggeration of resting divergence. http://jnnp.bmj.com/ unrousable and was admitted to hospital. At that Lateral gaze was full but there was paralysis of vol- stage he was in coma with constricted pupils, eyes untary reflex upward gaze. Optokinetic nystagmus was deviated to the right, and corticospinal signs in all absent in all directions. He was akinetic in all limbs four limbs. Treatment with dexamethasone 12 mg/ and the right arm showed slight plastic rigidity. There day was begun after a lumbar puncture had shown was no tremor. Both plantar responses were flexor. CSF containing 0,80 mg protein/l and 25 white cells/ There was released palmar grasping. The EEG showed mm3, predominantly mononuclears. The blood bilateral diffuse high voltage delta activity with some serology, ESR and echoencephalogram were normal. theta components. The CSF and an air encephalo- He improved slightly during the next three days, gram were normal. His mental state improved slowly on October 2, 2021 by guest. becoming stuporose with larger and reactive pupils. during several weeks. When reassessed in 1976 vol- He was then transferred to the Atkinson Morley's untary gaze upwards and downwards were absent Hospital. At this time his level of consciousness although lateral gaze was normal.