<p style="text-align: justify;">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.p>
Human ; 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-aspartate

<p style="text-align: justify;">OBJECTIVES: To present a case of childhood primary dystonia with good response to zolpidem.
INTRODUCTION: Dystonia is a syndrome of sustained muscle contractions causing twisting and repetitive movements or abnormal postures. It is not uncommon in children. Although there are several options to treat dystonia,its medical treatment is notoriously difficult and often unsuccessful. Zolpidem, an imidazopyridine agonist with a high affinity to benzodiazepine subtype receptor BZ1, is reported to improve basal ganglia disease including Parkinson'sdisease and various types of dystonia.
THE CASE: 11 year old female with torticollis starting the 7years old. At q10years old symptoms progressed with her back hyperexteded. Her cranial MRI was normal. She was started on Levodopa and carbidopa however no improvement of symptoms was noted. She was started on zolpidem and there was a marked improvement in speech, writing, swallowing, walking and posture observed.
DISCUSSION: Primary dystonia is defined as syndromes in which dystonia is the sole phenotypic manifestation. There is no cognitive decline and cranial MRI is normal. Management of childhood dystonia differs in certain respects from that of adult dystonia. Dopamine responsive dystonia in children is rare, but a trial of L-dopa should be performed on all patients rith childhood-onset dystonia. Dystonia is associated with hypoactivity of the globus pallidus interna, and widespread brain alterations in GABAA/benzodiazepine receptors. Zolpidem binding sites are abundant in the basal ganglia output structure the globus pallidus interna and substantia nigra pars reticulata. It is thought that by binding to these sites, zolpidem help restore Ie ganglionic output influence to the thalamus and motor cortex.
CONCLUSION: There are several options to treat dystonia,its medical treatment is notoriously difficult and often unsuccessful. Zolpidem an imidazopyridine agonist can be given in childhood dystonia unresponsive to dopamine.p>
Human ; Female ; Child ; Basal Ganglia ; Benzodiazepines ; Carbidopa ; Deglutition ; Dopamine ; Dystonic Disorders ; Globus Pallidus ; Levodopa ; Motor Cortex ; Parkinson Disease ; Pars Reticulata ; Dystonia