Analysis of Neuromyelitis Optica Spectrum Disorder in an Indian Cohort using International Consensus Diagnostic Criteria.

Context: Updates on clinical, investigatory, and therapeutic aspects of neuromyelitis optica (NMO) spectrum disorders are rapidly evolving. Recently published international consensus diagnostic criteria (ICDC) allowed clinicians to rapidly diagnose the expanding spectrum of NMO spectrum disorders more accurately. Aims: The aim of the study was to retrospectively analyze 36 consecutive cases of comprehensively evaluated NMO spectrum disorders using the ICDC. Patients and Methods: We retrospectively collected 36 cases of NMO spectrum disorders who attended our unit between August 2012 andOctober 2016 and fulfilled the ICDC. All patients underwent magnetic resonance imaging (MRI) of the brain and whole spine with contrast, anti-aquaporin 4 antibody, and detailed blood investigations to rule out systemic vasculitis and other alternate diagnoses. Results: Female-to-male ratio was 6.2:1; 50% of the cases were in the 20-40-year age group. Six patients (16.67%) had combined optic neuritis and myelitis.Nine patients (25%) had pure longitudinally extending transverse myelitis LETM with positive anti aquaporin 4 antibody AQ4Ab. Fourteen patients (38.9%) had myelitis and optic neuritis separately. Nine patients (25%) had area postrema syndrome. Two patients (5.6%) had acute brainstem syndrome and one (2.8%) had hypothalamic syndrome. LETM was commonly found in the cervical level (69.4%).Four patients (11.1%) had no spinal cord involvement. Anti-aquaporin 4 antibody was positive only in 23 cases (63.9%). Conclusions: Initial presentation of NMO spectrum disorder is often due to brain lesions. The ICDC criteria have enhanced clinician's ability to diagnose NMO spectrum disorder in the early stages. In our study, ICDC criteria helped us to diagnose 33% additional cases that would have been missed if the old 2006 revised criteria was applied.

Mercury Toxicity Following Unauthorized Siddha Medicine Intake - A Mimicker of Acquired Neuromyotonia - Report of 32 Cases.

CONTEXT: Mercury is used extensively in the preparation of Siddha medicines, after purification. In this study, we present 32 patients of mercury toxicity following unauthorized Siddha medicine intake who mimicked neuromyotonia clinically. We analyzed the clinical features of these patients, the role of autoimmunity in etiopathology, and compared it with acquired neuromyotonia. SUBJECTS AND METHODS: This is a retrospective study to analyze inpatients in a tertiary care center, admitted with mercury toxicity following Siddha medicine intake from August 2012 to October 2016. We analyzed the clinical features, laboratory data including mercury, arsenic and lead levels in blood, and serum voltage-gated potassium channels (VGKC)-CASPR2 Ab in selected patients. RESULTS: Thirty-two patients who had high blood mercury levels following Siddha medicine intake were included in the study. All patients (100%) had severe intractable neuropathic pain predominantly involving lower limbs. Twenty-six (81.25%) patients had fasciculations and myokymia. Fifteen patients (46.86%) had autonomic dysfunction (postural hypotension and resting tachycardia). Nine (28.12%) patients had encephalopathic features such as dullness, apathy, drowsiness, or delirium. Anti-VGKC Ab was positive in 12 patients with myokymia. All the patients in the study consumed Siddha medicines obtained from unauthorized dealers. CONCLUSIONS: Mercury toxicity following Siddha medicine intake closely mimics acquired neuromyotonia; severe intolerable neuropathic pain is the hallmark feature; Positive VGKC-CASPR2 antibody in some patients must be due to triggered autoimmunity secondary to mercury toxicity due to Siddha medicine intake. The government should establish licensing system to prevent distribution of unauthorized Siddha medicines.

"Hyperfrontality" as seen on FDG PET in unmedicated schizophrenia patients with positive symptoms.

INTRODUCTION: Decreased frontal activity has been reported widely in unmedicated schizophrenic patients with predominantly negative symptoms. Not many studies have assessed the frontal lobe status in unmedicated patients with positive symptoms. PATIENTS AND METHODS: Fifty-one patients with schizophrenia (all unmedicated, 38 never medicated) and 12 healthy age-matched controls were evaluated with FDG PET CT. The patients met ICD-10 and DSM-IV criteria for schizophrenia, and all reported psychotic, "positive" symptoms when tested. RESULTS: Schizophrenic patients with positive symptoms had a hypermetabolic frontal metabolic pattern on quantification by region to occipital ratio comparison. Associated statistically significant differences were also found when comparing ratios of occipital to thalamic, striatal and temporal cortex in schizophrenic patients. CONCLUSION: The finding of a hyperfrontality in unmedicated and never medicated psychotic schizophrenic patients is observed when there is a predominance of positive symptoms. There could be a possible disruption of cortico-striato-thalamic feedback loops causing hyperfrontality as seen in experimentally induced models of psychosis .

Tenofovir induced Fanconi syndrome: A rare cause of hypokalemic paralysis.

We report a 55-year-old female who presented to the emergency department with acute onset quadriparesis. She was diagnosed to have acquired immunodeficiency syndrome 7 years ago and was on tenofovir based anti-retroviral therapy for past 10 months. As the patient also had hypophosphatemia, glucosuria and proteinuria Fanconi syndrome (FS) was suspected. She improved dramatically over next 12 h to regain normal power and also her renal functions improved over next few days. Tenofovir induced FS presenting as hypokalemic paralysis is very rare complication and is the first case reported from India.

Hyperostosis frontalis interna mimicking Mount Fuji sign.

We report an interesting case of 'Hyperostosis frontalis interna' in a 73-yr-old female whose MRI pictures mimics the CT appearance of 'Mount Fuji sign' in tension pneumocephalus a neurological emergency.

Outcomes of endovascular coiling of anterior communicating artery aneurysms in the early post-rupture period: a prospective analysis.

BACKGROUND: There have been significant advances in the technical aspects of endovascular therapy of cerebral aneurysms. Anterior communicating artery (Acom A) aneurysms were traditionally treated by surgical clipping. Endovascular coiling has the distinct advantage of being minimally invasive and can be performed anytime during the course of subarachnoid hemorrhage (SAH). AIMS: To evaluate the results of endovascular coiling of Acom A aneurysms in the early post-rupture period. MATERIAL AND METHODS: Between June 1999 and December 2009, 103 Acom A aneurysms were treated with endovascular coiling. All the patients underwent digital subtraction angiography (DSA) and a diagnostic 3D rotational angiogram (3D-RA), followed by coiling using dedicated intracranial coils. RESULTS: Of the 103 patients coiled, 52% presented in Fischer grade 3/4 SAH and 13.5% in Hunt and Hess grade 4/5. Technical success was 98%. Complete obliteration of the aneurysm was achieved in 97 (94%) patients. Only one patient died of direct procedure-related complication due to coil prolapse. None of the patients had rebleeds. Six-month check angiogram performed in 34 patients showed significant recanalization in one patient. CONCLUSION: Ruptured Acom A aneurysms are implicated in majority of cases of SAH. Our results support the latest guideline "that endovascular coil occlusion of the aneurysm is appropriate for patients with a ruptured cerebral artery aneurysm that is deemed treatable either by endovascular coiling or by surgical clipping."

Non-Hodgkin's lymphoma of the brainstem with atrial septal mass.

Simultaneous occurrence of cardiac and central nervous system tumors have been rarely reported. A 23 years male presented to us with right cerebello-pontine (CP) angle symptoms and signs. Cranial imaging showed a mass lesion in the right pons infiltrating into the right and middle cerebellar peduncles. There was also a cardiac-atrial septal mass. The brainstem lesion was found to be a non-Hodgkin's lymphoma where as the cardiac lesion was not accessible. Central nervous system lymphomas are reported rarely and the prognosis is poor. The chances of the cardiac lesion in this patient also being a lymphoma are high, as it was a infiltrative cardiac mass, infiltrating the atrial septum.

Hemicraniectomy for large middle cerebral artery territory infarction: outcome in 19 patients.

BACKGROUND: Large space-occupying middle cerebral artery infarction accounts for 10-15% of all supratentorial infarctions and carries a mortality of 50% to 80%. Hemicraniectomy may be useful when optimal medical management has failed. METHODS: Between June 1997 and June 2000, 19 patients who fulfilled the clinical and imaging criteria for large middle cerebral artery infarction underwent hemicraniectomy because of impending herniation despite best medical therapy. The National Institute of Health Stroke Scale (NIHSS) assessed neurological status on admission and at one week after surgery. At 3 month follow up, The Barthel Index (BI) and Rankin Scale (RS) were used to assess the functional outcome among survivors. RESULTS: There were 15 males and 4 females with a mean age of 46.5 years (range 27-76 years). Ten patients (53%) had dominant hemisphere stroke. The mean interval between stroke onset and surgery was 60.3 hours (range 20-103 hours). The mean NIHSS score before surgery was 20.5 (range 17-26) and 10.5 (range 6-22) after surgery. One patient (5.2%) died due to post-operative meningitis. At follow up, mean BI was 56.4 (range 25-90) and RS revealed severe handicap in 4 patients (21%). Patients under 50 years of age had a significantly better outcome with mean BI of 60.7 as compared to only 41.3 (p=<0.048) in older patients. Speech function, especially comprehension improved in all patients with dominant hemisphere infarction. CONCLUSION: These findings add to previous studies suggesting hemicraniectomy may be a useful procedure in patients with large middle cerebral artery territory infarction. The functional outcome is good in younger patients. A randomised controlled trial is required to substantiate these findings.