Porphyria-induced Postpartum Reversible Posterior Encephalopathy Syndrome.

Acute intermittent porphyria (AIP) is a rare condition that needs to be kept in mind where its early recognition, conservative management, and removal of the precipitating factor are the key factors in its management. This "little imitator" presented with varied symptoms is often misdiagnosed. The diagnosis requires a strong index of suspicion as choosing an antiepileptic medication in the management of seizure requires a judicial choice to avoid precipitation of the underlying illness. How to cite this article: Sharma SR, Sharma N, Synmon B, Hynniewtaya Y. Porphyria-induced Postpartum Reversible Posterior Encephalopathy Syndrome. Indian J Crit Care Med 2022;26(6):728-730.

Clinical spectrum of myasthenia gravis: An institute experience over 8 years from North East India.

Background: Myasthenia gravis (MG) is a neuromuscular junction disorder usually associated with a thymic lesion. Aims and Objective: To study the clinical, serological, and thymic pathology in patient of MG from this corner of the country. Material and Method: A retrospective study involving all myasthenia patients presenting to neurology and cardio-thoracic department from the year 2013 to 2020. The clinical findings, Osserman grade of severity, antibodies profile, computed scanning thorax findings and histopathology of the thymic lesion were noted and collected as data. Results: Thirty patients of MG were included with mean age of onset being 39.10 ± 15.77 years which included 22 females and eight males. Four patients had only ocular findings while 26 patients had generalized myasthenia with three patients of respiratory failure. Ach receptor antibodies were positive in 27 patients and negative in two patients. Anti-MUSK was positive in one out of five patients. Abnormal findings in CT thorax seen in 20 patients which included enlarged thymic gland in 11 patients, thymic hyperplasia in two patients, thymoma in four patients, and anterior mediastinal mass in three patients. Thymectomy was done in eighteen patients with thymoma as the most common histopathological findings seen in eight patients, follicular hyperplasia in five patients; other was thymic hyperplasia, thymic cyst, normal thymus gland, and features of sarcoidosis in one patient. Conclusion: MG is a treatable autoimmune disorder with a variety of clinical, radiological, and histopathological findings.

Etiological and Radiological Spectrum of Longitudinal Myelitis: A Hospital-Based Study in North East India.

Introduction An inflammatory lesion of the spinal cord where three or more than three vertebral segments of the cord is involved is called longitudinal extensive myelitis (LETM). It has several varied causes out of which neuromyelitis optica (NMO) and its spectrum disorder have received a distinct entity. Various radiological and clinical features help us to suspect an etiology which then further guides us into the treatment protocol and prognosis of the patients. Materials and Methods A retrospective study performed in a referral center in North East India in 15 months. Thirty-two patients of LETM were enrolled based on clinical and radiological available data. An attempt was made to classify the various etiologies and correlate with their radiological findings. Results The most common etiology noted was NMO seen in 7 patients (21.8%) followed by tuberculosis (TB) (18.7%) and post-infection myelitis (18.7%). Other etiology seen was acute disseminated encephalomyelitis (6.24%), spinal cord infarct (3.12%), radiation myelitis (6.24%), Japanese encephalitis sequalae (3.12%), systemic lupus erythematosus (3.12%), and remained undiagnosed in six patients (18.7%). Radiologically, cervico-dorsal spine was most common location in NMO (71%) whereas dorsolumbar in TB (50%). The lesion was predominantly central in both NMO (100%) and TB (80%) as compared with the other causes of LETM. It was noted that more than 50% of the transverse area of the cord was involved in both NMO (71%) and TB (50%), but < 50% involvement were more common in the post-infectious and others causes of LETM. Conclusion LETM has a various differential diagnosis, infection need to be kept in mind while ruling out NMO. Radiological features can suggest or help differentiate the various etiologies of LETM but NMO and infection like TB almost has the same features except for a different cord site predilection.

Clinical and radiological spectrum of intracranial tuberculosis: A hospital based study in Northeast India.

Central nervous system tuberculosis (TB) is the most severe extra pulmonary TB having a high mortality and morbidity. OBJECTIVE: To study the various clinical, biochemical, and radiological spectrum of intracranial TB. MATERIALS AND METHOD: Ninety-three patients were enrolled in this prospective study after ethical clearance and consent from August 2013 to May 2015. The entire clinical course with complications and predictors of mortality were assessed. RESULTS: 36 females (38.7%) and 57 males (61.3%) were included whose mean age of presentation was 32.3±17.05 years. Alcohol was the most common risk factor seen in 19.4%. Headache (90.3%) was the most common symptom. Co-infection with human immunodeficiency virus, cryptococcal, and toxoplasmosis were seen in 11, 3, and 2 patients, respectively. Cerebrospinal fluid analysis showed acid-fast bacilli in 1 patient; polymerase chain reaction for TB and BACTEC was positive in one and three patients, respectively. Neuroimaging showed basal exudates (21.7%), tuberculoma (28.6%), brain edema (27%), hydrocephalus (32.9%), infarct (21%), and abscess (2.9%). Complications were noted such as brain edema (24.7%), vasculitis (26.9%), hydrocephalus (17.2%), hyponatremia (11.8%), drug-induced hepatitis (4.3%), and drug rash in 5 patients (5.4%). A total of 25 patients (26.9%) died and 38 patients (40.9%) developed neurological sequelae like hemiparesis, paraparesis, visual loss, and hearing loss. Logistic regression showed that a Glasgow scale of <10, British Medical Research Council stage 3, and vasculitis were associated with poor outcome. CONCLUSION: Lack of sensitive diagnostic method and criteria makes central nervous system TB a challenge where early diagnosis and prompt management is required.

Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India.

BACKGROUND: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21st century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic. OBJECTIVE: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India. MATERIALS AND METHODS: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Patients of noncompressive myelopathies who underwent magnetic resonance imaging (MRI) of the spine were segregated into two categories: acute-to-subacute myelopathy (ASM) and chronic myelopathy (CM). In addition to routine blood tests, chest X-ray, urinalysis, and visual evoked potentials, investigations included MRI of the brain, cerebrospinal fluid analysis, and immunological, infectious, and metabolic profile based on the pattern of involvement. RESULTS: The study had 151 patients (96 ASM and 55 CM) with a median age of 35 years and male: female ratio 1.4:1. The causes of ASM were neuromyelitis optica spectrum disorder (23), multiple sclerosis (MS) (8), systemic lupus erythematosus (1), Hashimoto's disease (1), postinfectious acute disseminated encephalomyelitis (6), postinfectious myelitis (8), infections (9), spinal cord infarct (5), and electrocution (1). The causes of CM were MS (1), probable or possible sarcoidosis (7), mixed connective tissue disease (1), Hashimoto's disease (2), infections (9), Vitamin B12 deficiency (4), folate deficiency (2), hepatic myelopathy (2), radiation (11), and paraneoplastic (1). No etiology could be found in 48 (31.8%) patients (34 ASM and 14 CM). In 21/96 (21.9%) patients of ASM, acute transverse myelitis was idiopathic based on current diagnostic criteria. CONCLUSION: Underlying etiology (demyelinating, autoimmune, infectious, vascular, metabolic disorder, or physical agent) was found in 68% patients of noncompressive myelopathy.

Relapsing Anti-NMDAR Encephalitis after a gap of eight years in a girl from North-East India.

It has been just 7 years since the discovery of anti-NMDAR encephalitis as distinct immune-mediated encephalitis and we have such cases being reported from our country. Herein, we describe a case of a 13-year-old girl who had relapsing encephalitis consisting of multiple types of difficult-to-control seizures, abnormal behavior, language disintegration, memory loss and abnormal movements eight years after the first clinical attack. In 2005, when she was 5 yearsold, anti-NMDAR encephalitis was not yet discovered and she was provisionally diagnosed as a case of viral encephalitis. During her second attack in 2013, antibodies against NMDAR were demonstrated by immunofluoresence in serum (1:10). This is the first report from our country of a case of relapsing anti-NMDAR encephalitis of such a long duration, successfully treated by immunotherapy.