ObjectiveNA.BackgroundAutoimmune Encephalitis is an inflammatory condition of the brain due to antibodies against onconeuronal proteins. Paraneoplastic Parkinsonism is very rare. We report the first case of AMPA receptor encephalitis presenting with symptoms of parkinsonism, an atypical presentation of a rare entity.Design/MethodsNA.ResultsA 71-year-old female with multiple comorbidities presented in a state of stupor with complaints of insidious onset slowly progressive recent memory impairment, progressive slowness in performing daily activities, inability to communicate, and acute onset urinary incontinence. CNS examination initially showed a GCS of E2V1M4 with Grade 2 rigidity in all extremities. MRI Brain showed subtle T2 FLAIR hyperintensities in the peri-ventricular and subcortical white matter. Serum and CSF studies showed AMPA 1 antibody positivity. The FDG PET showed an avid speculated soft tissue density lesion in the upper inner quadrant of the right breast with active right axillary lymph nodes (Histopathology- Infiltrating duct carcinoma Grade 2 NST T2N2aMx). The patient was managed using IVIg and steroids following which her sensorium improved to a GCS of E4M6V5. UPDRS at this point was 29. She subsequently underwent therapy for her tumor following which there was a significant decrease in parkinsonian symptoms and an improvement in memory without the use of any antiparkinsonian medications (UPDRS score-6). During the four years of follow-up, she has remained independent and can perform all her activities of daily living. Hence this autoimmune encephalitis case can be classified as a definite paraneoplastic neurological syndrome (PNS Care score-9).ConclusionsWe propose that Parkinsonism, in our case, is probably a paraneoplastic neurological syndrome associated with antibodies against the AMPA receptor, as the symptoms and signs recovered with cancer treatment.
<p class="abstract"><strong>Background:</strong> Cochlear implantation (CI) has revolutionized the treatment of sensorineural deafness. The aim of the study was to compare auditory based performance in cochlear implants who underwent round window insertion and cochleostomy.</p><p class="abstract"><strong>Methods:</strong> Ambispective cohort study was done among the children who underwent perilingual cochlear implant in a tertiary care hospital for period of 1 year. Children who satisfied the inclusion criteria were selected and randomly subdivided into 2 groups: group A- round window insertion and group B-cochleostomy. Pre- and post-operative pure tone average (PTA) and residual hearing preserved were evaluated among the cochleostomy and round window insertion groups separately. Post-operatively, children were evaluated, from three months to 1 year from the activation of cochlear implant, with the use of scores such as Category of Auditory Performance (CAP), Meaningful Auditory Integration Scale (MAIS), Speech Intelligibility rating (SIR) and Meaningful use of speech scale (MUSS score) to measure speech production skills by auditory verbal therapist. </p><p class="abstract"><strong>Results:</strong> A total of 80 patients were included in the study. Complete hearing preservation (within 10 dB) was significantly high in round window insertion group compared to cochleostomy technique (p<0.001). None the subjects had complete loss of residual hearing. On evaluating the post CI performance, MAIS score in the round window insertion group (9.34, 18.21, 27.79) were significantly better compared to cochleostomy group during the 3 months, 6 months and 12 months follow-up.</p><p class="abstract"><strong>Conclusions:</strong> Round window insertion technique is significantly more successful in complete hearing preservation at low frequencies compared to cochleostomy technique. Among the auditory scores, only MAIS in the round window insertion group was found to be significantly better compared to cochleostomy group.</p>
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