ABSTRACT
OBJECTIVES: To present a case of anti NMDA receptor encephalitis in an adolescent male; to report a case of anti-NMDA receptor encephalitis who showed a rapid response to methylprednisone. INTRODUCTION: Anti NMDA receptor encephalitis is an inflammatory encephalopathic autoimmune disease frequently affecting young women with teratomas of the ovary. It is also observed in men, children, and females without tumors. THE CASE: A case of a 14 year adolescent right handed male adolescent presented with fever, hypertension , psychiatric symptom ,aphasia and orofacial dyskinesia. On admission he was highly febrile with dysautonomia, rigidity, catatonia and prominent orofacial dyskinesia. Upper motor signs include hyperreflexia and bilateral clonus. He latter developed Ileus, bladder and bowel incontinence, seizures, chorea, dystonia and dIe orofacial dyskinesia worsened. EEG showed continuous background slowing, Cranial MRI was normal and CSF analysis showed pleocytosis with lymphatic predominance with a normal protein and sugar. Work up for Herpes and Japanese B encephalitis was negative. Work up for tumor was negative. After 5 days of methylprednisone a rapid improvement was noted with resolution catatonia, rigidity hypereflexia and clonus. Results of the anti NMDA receptor antibodies showed positive for anti NMDA receptor encephalitis. DISCUSSION: Anti NMDA receptor encephalitis presents with fever, headache,or malaise, followed mood and behavioral changes, psychiatric symptoms, decline of level of consciousness, hypoventilation and hyperkinesias. Immunotherapy is the current treatment. In patients with underlying tumor, immunotherapy, enhanced the effectiveness and speed of recovery. In patients without a tumour an additional treatment with second-line immunotherapy is needed but was not noted in this case. The patient showed a rapid recovery after methylprednisone and after 4 months post immunotherapy there was no recurrence of symptoms. CONCLUSION: Anti NMDA receptor encephalitis is an inflammatory encephalopathic autoimmune disease and can be seen in children without tumors. Behavioral and hyperkinesias are prominent symptoms. Treatment with methylprednisone showed a good response.
Subject(s)
Humans , Male , Adolescent , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Catatonia , Chorea , Dyskinesias , Dystonia , Electroencephalography , Encephalitis, Japanese , Hashimoto Disease , Hyperkinesis , Leukocytosis , Primary Dysautonomias , Receptors, N-Methyl-D-AspartateABSTRACT
CHAP syndorme consists of choreoathetosis, orofacial dyskinesia, affective change, hypotonia, and pseudobulbar signs. CHAP syndrome was firstly des- cribed with onset 1 to 7 days after induced profound hypothermia and complete circulatory arrest for con- genital heart lesion repair. Since then, only a few cases have been reported until now. Case We report a patient who demonstrated all of the elements of the CHAP syndrome in severe cachexic state. Brain SPECT(99mTC-ECD) shows decreased perfusion in left fronto-parieto-temporal lobe. Comments The pathogenesis of CHAP syndrome is obscure. However our case led us to suggest that more essential component pre- disposing to the CHAP syndrome is hypoxia rather than hypothermia. Furthermore, it seems to be related with circulatory insufficiency in extrapyramidal nervous systems.