Acute Disseminated Encephalomyelitis presenting as ... - medIND

DELHI PSYCHIATRY JOURNAL Vol. 13 No.1

APRIL 2010

Case Report

Acute Disseminated Encephalomyelitis presenting as Dissociative Disorder Jyoti Prakash, H.R.A. Prabhu, Kalpana Srivastava, P.S. Bhat, R. Shashikumar Department of Psychiatry, AFMC, Pune

Introduction Diagnostic dilemmas have always been matters of challenge as well as interest in the field of medical science. Varied presentation of disorders are becoming more of a norm than exception and the psychiatric presentation of the medical disorder had always been vexing. Acute disseminated encephalomyelitis (ADEM), an immune mediated disease of the brain, usually occurs following a viral infection but may appear following vaccination, bacterial or parasitic infection, or even appear spontaneously1-3. It is an uncommon inflammatory demyelinating disease of the central nervous system and often presents with various combinations of motor, sensory, visual, gait, and memory disturbances. The incidence rate is about 8 per 1,000,000 people per year4. Though it is common in children and adolescents it can occur across all ages5-7. The mortality rate may be as high as 5% with average time of recovery anywhere from one to six months8. Some case reports of ADEM presented as psychiatric disorders primarily as confusion 9 psychosis10-11 and at times Dissociative disorder12. One of such rare presentation is brought here for the clinical interest. The case has its salience in nature of presentation and the way high index of suspicion led to early diagnosis and remediation. Case Report A 30 year old Oriya speaking lady was brought by her husband to the emergency room with history of sudden onset mutism and stupor. History revealed that she was second wife of her husband and married since last five years following the death of her husband’s first wife owing to chronic heart illness. There has been no issue from the first wife and 150

individual had consented for the marriage in the background of the marriageable age of husband, his government job and no pending liability from the first marriage. However despite 4 years of marriage and adequate conjugal relations the couple was not able to bear a child and she would often get depressed and preoccupied due to the same. Her husband came to Punjab on transfer six months back. This place was quite distant from her native place and because of absence of anybody of her vernacular in the vicinity and being poorly conversant in English she found herself more lonely. She would be often alone at home with nothing much to do, after the domestic chores and following departure of her husband to work which involved long duty hours. This further increased her yearning for a child and she would often get sad due to her inability to have the same despite treatment at infertility clinic and inconclusive evidence of any disability to that effect, of either of the couple, in any of the tests. She however was able to improve her mood by sharing her feelings with husband, domestic chores and listening to folk music of her native place. After few months of arrival to new place they got acquainted to a family from their neighboring town and with their regular interaction the intimacy between the two families grew further. On one occasion the other family had to go out of town owing to some official commitments and left their son at her’s place as he could not have been taken along because of the semester examination next week. The lady was pretty happy in the company of a child at her house and she took great care of him, as if it was her own child. She talked to him in vernacular language and answered his simple queries. When the parents of the child came

Delhi Psychiatry Journal 2010; 13:(1) © Delhi Psychiatric Society

APRIL 2010


to take their child in the evening she insisted that, he should be left at their house for the night. Child was also happy being at a new place where he had a pleasant aunt who took great care and showered affection on him. The couple had the good company of the child for the night, however had to part with him the next morning. Husband also left for the work leaving his wife alone to herself. On arrival back from the work in the evening husband found the lady lying motionless in the bed. She did not respond to his questions, was mute and did not move her body despite persuasion or command. She was take to a physician who suspected Dissociative disorder and after initial evaluation called for a psychiatric consult. Initial evaluation brought out history as above. There was no past or family history of neurological or psychiatric illness. General and systemic examination except for evaluation of Central Nervous System were within normal limits. Assessment of higher mental functions revealed a mute and stuporose individual who responded to painful stimuli by moans only. She stared blankly ahead and had occasionally strained expression on he face. Pupils were normal and equal. No cranial nerve deficit or signs of meningeal irritation were seen. No spontaneous or induced movement of any limb were seen. Tone was equally decreased and reflexes were equal in all limbs. Plantar reflex was down going on left side and equivocal on right side. Though the history and evaluation pointed towards the possibility of a psychiatric disorder, soft neurological signs pointed towards an organic etiology. She was admitted in intensive care unit of the hospital. Repeat evaluation after half an hour revealed positive babinski reflex on the right side. Further history from the husband for any febrile illness in the recent past brought out that she had suffered from malaise and coryza of 3 days duration a week back which was self limiting and had appeared insignificant. Detailed hematological and biochemical parameters were within normal limits. CT scan brain did not reveal anything abnormal. Repeated medical and neurological evaluation by the physician did not suggest any specific neurological disability and the soft neurological sign was taken as of inconclusive significance . Ward observation revealed continued stuporose status without any significant deterioration. She was placed on intravenous fluids and nasogastric feeds.

The next day she was found to have autonomic disturbance in frequent fall of Blood pressure without any specific reason and nasogastric aspirate showed coffee ground vomitus. Investigation revealed normal CPK titres. EEG was unremarkable and lumbar puncture revealed normal CSF on analysis including the viral markers. Plantar was still up going on the right side. Maintaining a high index of suspicion of organicity a MRI of the brain was asked for, which revealed bilateral, left more than right, multiple areas of increased signal intensity in the periventricular deep white matter, suggestive of demyelination. Lesions demonstrated no enhancement with gadolinium. Based on these finding a revised diagnosis of Acute Disseminated Encephalomylitis was made. She was started on steroid injections and immunoglobulins apart from broad spectrum antibiotics and other supportive measures. She made slow and gradual recovery over next one week and on stabilization was transferred to neurological centre for onward management. Discussion The patient had Acute Disseminated Encephalomyelitis following an infection of upper respiratory tract1-2. Initial history and evaluation strongly pointed towards the possible diagnosis of Dissociative disorder as no neurological or medical disorder was detected in the emergency room, and it was thought that there was significant association between the onset of symptom and a stressor (overwhelming desire to have a child and sudden deprivation due to return of friend’s child) and inability to resolve the conflict. Psychiatric history in this patient could have lead to misdiagnosis and delayed management of a more serious neurological illness. High index of suspicion and regular assessment of the patient prevented such delay and the diagnosis was confirmed by MRI. Thus it is imperative that even if dissociative disorder is suspected because of the clinical presentation and presence of a preceding stressor in the environment, a thorough physical examination including neurological examination is mandatory to rule out possible organic etiology. References 1. Dale RC. Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis 2003; 14 (2): 90–5.


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2. Garg RK. Acute disseminated encephalomyelitis. Postgrad Med J 2003; 79 (927): 11– 17. 3. Jones CT. Childhood autoimmune neurologic diseases of the central nervous system. Neurol Clin 2003; 21 (4) : 745–64. 4. Leake JA, Albani S, Kao AS, et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr. Infect Dis J 2004; 23 (8) : 756–64. 5. Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001; 56 (10) : 1308–12. 6. Anlar B, Basaran C, Kose G, et al. Acute disseminated encephalomyelitis in children: outcome and prognosis. Neuropediatrics 2003; 34 (4) : 194–9. 7. Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B. Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology 2001; 56 (10) : 1313– 8.

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8. Menge T, Kieseier BC, Nessler S, Hemmer B, Hartung HP, Stüve O. Acute disseminated encephalomyelitis: an acute hit against the brain. Curr Opin Neurol 2007; 20 (3) : 247–54. 9. Tei H, Soma Y, Iwata M. Korsakoff’s syndrome as the first clinical manifestation of acute disseminated encephalomyelitis. Behav Neurol 1995; 8 : 181–185. 10. Moscovich DG, Singh MB, Eva FJ, et al. Acute disseminated encephalomyelitis presenting as an acute psychotic state. J Nerv Ment Dis 1995; 183:116. 11. Nasr JT, Andriola MR, Coyle PK. ADEM: literature review and case report of acute psychosis presentation. Pediatr Neurol 2000; 22 : 8–18. 12. Abay E, Balci K,Ates I. Acute Disseminated Encephalomyelitis Presenting as Conversion Disorder J Neuropsychiatry Clin Neurosci 2005; 17 : 259-260.
