With a working diagnosis of Sydenham’s chorea, sodium valproate and, subsequently, tetrabenazine were tried without clear benefit

With a working diagnosis of Sydenham’s chorea, sodium valproate and, subsequently, tetrabenazine were tried without clear benefit. receptor (NMDA\R) encephalitis is increasingly recognized worldwide as one of the most common forms of encephalitis surpassing any specific viral encephalitis.1, 2 The disorder still remains highly under\recognized in developing countries owing to a decreased awareness among the medical fraternity as well as a lack of easily available and reliable antibody testing. The well\known clinical narrative in this encephalitis is that of a subacute onset multistage progressive encephalopathy with complex movement disorders, psychiatric manifestations, seizures, language disturbances, and autonomic dysfunctions.1 Lesser known are the incomplete or forme frustes of the disorder, which HQ-415 have recently drawn interest. Here, we describe 2 boys with HQ-415 anti\NMDA\R encephalitis presenting predominantly as a syndrome of ataxia and dyskinetic movement disorder without significant neuropsychiatric manifestations. Case 1 The first patient was a 3.5\yr\old boy who had 3 episodes of afebrile, brief focal motor seizures over 2?days without intervening encephalopathy. Scalp EEG and cranial MRI were normal. Levitiracetam (LEV) was commenced, and 1?week later he developed gait difficulty. There were minor behavioral changes with irritability and sleep disturbances, though language and cognition remained normal. Neurological examination revealed normal mental status examination with ataxia, loss of postural reflexes, and significant chorea affecting primarily the lower limbs (see Video 1). Considering these as drug adverse effects, LEV was discontinued, without benefit. Other normal investigations included a cerebrospinal fluid (CSF) routine examination, CSF HQ-415 lactate, and CSF total immunoglobulin (immunoglobulin G; IgG). Complete blood count and routine biochemistry were also normal. Serum and CSF anti\NMDA\R antibody was then detected. Ultrasonography (USG) of the abdomen and scrotum were normal. The patient was initiated on intravenous immunoglobulin (IVIG) at a dose of 2?g/kg over 2?days, followed by a monthly single infusion of 400?mg/kg for 6?mo. Concomitant steroid therapy was offered, but refused by the parents. A gradual improvement in symptoms was noted with complete recovery by the fourth dose. Repeat serum NMDA\R antibody titer done HQ-415 1?mo after completion of immune therapy was negative. The child remains asymptomatic at 1.5\yr follow\up. Case 2 This 9\yr\old boy presented initially with a new\onset anxiety of heights. This was then followed by a gait disturbance Rabbit Polyclonal to CNTN4 with frequent falls and a change in voice in the form of slow scanning speech. He also had worsening of handwriting and continuous arrhythmic quasi\purposeful movements of both hands thought to be chorea by the treating physician at the time. Normal investigations at the time were routine blood counts, biochemistries, anti\streptolysin\O titers, serum ceruloplasmin, and anti\nuclear antibody. Cranial MRI and a two\dimensional echocardiogram were also normal. With a working diagnosis of Sydenham’s chorea, sodium valproate and, subsequently, tetrabenazine were tried without clear benefit. Oral prednisolone (1.5?mg/kg/day) resulted in a prolonged remission lasting 3?mo. Steroid withdrawal led to a relapse of symptoms when he was referred to our institution. Cerebellar signs were prominent with gait ataxia, slow HQ-415 scanning speech, dysmetria, and dysdiadochokinesia. Though associated chorea was also observed, this was markedly reduced compared to the chorea recorded earlier on video (see Video 2). Language and cognitive skills were intact with normal behavior and no seizures. An EEG was normal. CSF analysis revealed 22 cells (lymphocytes, 82%; neutrophils, 18%) with normal proteins, glucose, and lactate. Oligoclonal bands were present whereas CSF total IgG was normal. Anti\GAD antibody was normal. However, anti\NMDA\R antibodies were detected in both serum and CSF. Screening for abdominal and scrotal neoplasms using ultrasound was negative. Pulse methylprednisone followed by prolonged oral steroids tapered over 9?mo resulted in a complete remission of symptoms within 1?mo of starting treatment. His anti\NMDA\R antibody.