Outcome of Guillain-Barré Syndrome (GBS) During Peripartum Period: A Hospital-Based Observational Study.

Objective: 1. To study the clinical profile, progression and outcome of GBS during peripartum period in a tertiary care hospital. 2. To identify the determinants of outcome in GBS during peripartum period. Background: Guillain-Barré Syndrome (GBS) is an acute, autoimmune disorder of the peripheral nervous system triggered by a bacterial or viral infection or other antecedent events. Modern day critical care has dramatically improved the outcome of GBS. GBS during pregnancy is rare with an annual incidence of 2.8/100,000 population. Pregnancy itself is a life-threatening condition and full of complications. GBS during pregnancy makes its more complicated in terms of both maternal and fetal care during peripartum period. Methods: This was a prospective study conducted by the Department of Neurology, Gauhati Medical College including 36 patients of GBS presenting during peripartum period in between December 2019 and November 2021. Their clinical and electrophysiological features were analyzed. Hughes grading, EGRIS, mEGOS, MRC sum score were used. The fetal outcome was observed and patients were followed up for GBS outcome at 3 months and 6 months. Results: The mean age of patients was 25.97 years. AIDP was the most common subtype found in 21 (58.33%) patients followed by AMAN in 7 (19.4%), AMSAN in 3 (8.33%). In 3 cases NCS was equivocal and in 3 cases inexcitable. Respiratory distress was found in 13 (36.1%) cases, out of which 4 (11.1%) required mechanical ventilation and 1 (2.8%) died. The pre-term birth rate and stillbirth rate were 35.7% (n = 10) and 8.33% (n = 3), respectively with 66.7% (n = 24) spontaneous vaginal delivery(SVD). At 3 month 26 (72.2%) had complete recovery. 5 (13.9%) and 4 (11.1%) had partial and poor recovery, respectively. 78.9% of primigravidae had complete recovery in comparison to 64.7% in multigravidae indicating better outcome in primigravidae in this study. Conclusion: The outcome of GBS during peripartum period is favorable. Primigravidae are more commonly affected but have better outcome than the multigravidae. The risk of developing GBS in pregnancy decreases significantly after delivery and is minimal after 2 weeks. GBS is not an indication for LSCS. Stillbirth rate and preterm birth rate is higher in pregnancy associated with GBS without fearsome neonatal complications.

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.

Myopathies of endocrine disorders: A prospective clinical and biochemical study.

INTRODUCTION: MAJOR CATEGORIES OF ENDOCRINE MYOPATHY INCLUDE THOSE ASSOCIATED WITH: Adrenal dysfunction (as in Cushing's disease or steroid myopathy); thyroid dysfunction (as in myxedema coma or thyrotoxic myopathy); vitamin D deficiency; parathyroid dysfunction; and pituitary dysfunction. Steroid myopathy is the most common endocrine myopathy. OBJECTIVE: To study the etiology, varied presentations, and outcome after therapy of patients with endocrine myopathies. MATERIALS AND METHODS: MYOPATHY WAS EVALUATED BY THE STANDARD CLINICAL PROCEDURES: Detailed clinical history, manual muscle strength testing, and creatine phosphokinase (CPK). Endocrine disorders were diagnosed as per clinical features and biochemical parameters. The treatment was given to patients as per underlying endocrine disease. Myopathy was assessed before and after treatment. RESULTS: Out of the 37 patients who were diagnosed with endocrine myopathies, thyroid dysfunction was the most common cause (17 cases), followed by vitamin D deficiency in nine, adrenal dysfunction in six, parathyroid dysfunction in three, and pituitary dysfunction in two. Some patients had atypical presentation (repeated falls in one, tongue fasciculations in one, neck weakness in five, one with ptosis and facial weakness, asymmetrical onset in one, and calf hypertrophy in one. The serum creatine kinase (CK) concentration did not correlate with muscle weakness. Following the treatment regimen which was specific for a given myopathy, 26 patients recovered fully. CONCLUSION: We found varied clinical presentations of endocrine myopathies. All the patients with neuromuscular complaints should be investigated for endocrine causes because significant number of them recovers fully with specific treatment.

Varied presentations of moyamoya disease in a tertiary care hospital of north-east India.

INTRODUCTION: Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India. OBJECTIVES: To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India. MATERIAL AND METHODS: Relevant investigations were done to rule out other causes of moyamoya syndrome. RESULTS: We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs. CONCLUSION: Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.

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.

Deletion pattern in the dystrophin gene in Duchenne muscular dystrophy patients in northeast India.

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder that affects 1 in 3,500 males and is caused by mutations in the dystrophin gene. In this paper, we have reported DNA analysis of DMD patients by multiplex polymerase chain reaction (PCR) from various states of northeast India. Of the 69 clinically suspected patients of DMD, deletion was detected by multiplex PCR in 49 (71%) patients. Majority of the deletions (42/49, 85.7%) were located at distal hot spot region that encompasses exons 44-55 and 14.3% of the deletions were located at the proximal hot spot region (exons 2-19). In this study population, the deletion rate was 71% and was more frequent in the distal end exon.

Clinical and biochemical spectrum of hypokalemic paralysis in North: East India.

BACKGROUND: Acute hypokalemic paralysis, characterized by acute flaccid paralysis is primarily a calcium channelopathy, but secondary causes like renal tubular acidosis (RTA), thyrotoxic periodic paralysis (TPP), primary hyperaldosteronism, Gitelman's syndrome are also frequent. OBJECTIVE: To study the etiology, varied presentations, and outcome after therapy of patients with hypokalemic paralysis. MATERIALS AND METHODS: All patients who presented with acute flaccid paralysis with hypokalemia from October 2009 to September 2011 were included in the study. A detailed physical examination and laboratory tests including serum electrolytes, serum creatine phosphokinase (CPK), urine analysis, arterial blood gas analysis, thyroid hormones estimation, and electrocardiogram were carried out. Patients were further investigated for any secondary causes and treated with potassium supplementation. RESULT: The study included 56 patients aged 15-92 years (mean 36.76 ± 13.72), including 15 female patients. Twenty-four patients had hypokalemic paralysis due to secondary cause, which included 4 with distal RTA, 4 with Gitelman syndrome, 3 with TPP, 2 each with hypothyroidism, gastroenteritis, and Liddle's syndrome, 1 primary hyperaldosteronism, 3 with alcoholism, and 1 with dengue fever. Two female patients were antinuclear antibody-positive. Eleven patient had atypical presentation (neck muscle weakness in 4, bladder involvement in 3, 1 each with finger drop and foot drop, tetany in 1, and calf hypertrophy in 1), and 2 patient had respiratory paralysis. Five patients had positive family history of similar illness. All patients improved dramatically with potassium supplementation. CONCLUSION: A high percentage (42.9%) of secondary cause for hypokalemic paralysis warrants that the underlying cause must be adequately addressed to prevent the persistence or recurrence of paralysis.

Etiological profile of epilepsia partialis continua among adults in a tertiary care hospital.

BACKGROUND: Epilepsia partialis continua (EPC), is a subtype of status epilepticus, have a varied spectrum of etiology and the out-come depends on the etiology. AIMS AND OBJECTIVES: The present study is aimed to analyze the clinical characteristics and outcome. MATERIALS AND METHODS: This is a prospective analysis of 17 patients admitted to our center between August 2010 and April 2012. EPC was defined as regular or irregular clonic muscular twitches affecting a limited part of the body, occurring for a minimum of 1 h, and recurring at intervals of no more than 10 s. The data collected included etiology, radiological findings, electroencephalogram (EEG) abnormalities, associated comorbid conditions, and outcome. RESULTS: The mean age at presentation was 44.26 ± 13.77 years and the mean duration was 2.7 ± 1.5 days. There were ten patients with diabetic non-ketotic hyperosmolar state and one patient each of oligodendroglioma, varicella zoster vasculitis, central nervous demyelination, ischemic stroke, post traumatic seizure, arteriovenous malformation, and in one patient no cause could be established. Imaging showed abnormality only in five patients and EEG was abnormal in four patients. The EPC was controlled by one antiepileptic drug (AED) in eight patients, with two AEDs in seven patients and two patients required three AEDs. CONCLUSION: EPC is a rare type of focal motor status epilepticus. Treatment of the underlying cause in addition to controlling EPC is essential to achieve the good outcomes.

Clinical and radiological spectrum of Japanese encephalitis.

INTRODUCTION: Japanese encephalitis (JE) is mosquito-borne flaviviral encephalitis that remains to be a major health problem in India--it still continues to cause havoc in many parts of the country. We undertook the study to analyze the clinical and radiological spectrum of JE in adults and children. METHOD: This prospective study consists of 148 patients with JE. The diagnosis of JE was based on clinical, epidemiological, radiological features and demonstration of JE virus specific IgM in CSF and serum by JE virus immunoglobulin M capture enzyme-linked immunosorbent assay (MAC ELISA). All patients underwent a detailed neurological examination, CSF study & neuroimaging of brain (either CT or MRI or both). All patients were followed-up at regular interval. RESULT: Seizures were present in adults (52.88%) and in children (43.18%). Dystonia was more common in children 19 (43.18%) compared to adults 19 (18.2%), and Parkinsonian features were observed in both groups 47 (45.19%) of the adults and 20 (45.45%) of the children. JE-specific IgM antibody was detected in both CSF and serum in 81.7%. In neuroimaging, apart from classical involvement of thalami, basal ganglia & midbrain, prominent involvement of hippocampus and other areas of the cortex was also found in 27 (45.6%) patients. Presence of thalamic lesion in CT/MRI showed significant relationship to the development of dystonia. However, no correlation was found between the neuroimaging features and poor clinical outcome. Twenty three patients (15.5%) died during acute phase of illness. On multivariate logistic regression analysis age, prolonged fever, Glasgow coma scale, recurrent seizures and reflex changes were found to be the predictors of outcome at the time of discharge. CONCLUSION: A trend of severe and frequent involvement in younger patients with dystonia and other movement disorders was observed. It should be emphasized that presence of atypical cranial CT/MRI features in JE was not unknown and they need to be differentiated from herpes simplex encephalitis in appropriate clinical setting.

A case of Spinocerebellar Ataxia from ethnic tribe of Assam.

Here we present the case of a 17-year-old girl belonging to an ethnic tribe (Bodo tribe) of Assam, presenting with bilateral cerebellar signs and with history suggestive of an autosomal dominant pattern of inheritance, who was found to have spinocerebellar ataxia 7 on genetic testing. This case throws light on the probability of more such cases in the multi-ethnic society of the North-Eastern Indian states, which are not studied or reported till date.

Clinical spectrum of neurosyphilis in North East India.

BACKGROUND: Symptomatic neurosyphilis (NS) can have varied syndromic presentations: Meningitis, meningovascular and parenchymatous involvement. AIMS: To evaluate the different types of clinical syndrome of NS in a tertiary care hospital. MATERIAL AND METHODS: This was a study of clinical profile of 16 patients with NS, seen in between August 2008 and December 2010. RESULTS: There were 13 male and 3 female patients in the age group of 23-48 years. The clinical syndromes included: Neuropsychiatric syndromes (10), myelopathy (5), and posterior circulation stroke (1). Neuropsychiatric symptoms were dementia, behavioral abnormalities, chronic psychosis, and myelopathy syndromes included acute transverse myelitis (ATM), chronic myelopathy, and syphilitic amyotrophy. Thirteen patients had positive venereal disease research laboratory test (VDRL) and/or high Treponema pallidum hemagglutination titer in blood. Cerebrospinal fluid was positive for VDRL in 14 patients, raised protein was seen in 13 patients and lymphocytic pleocytosis was seen in 10 patients. Human immunodeficiency virus serology was negative in all the patients. Fourteen patients fulfilled the criteria of definitive NS and two of presumptive NS. All the patients except one received injection Procaine penicillin for 14 days. The patient with myelitis received a course of steroid, and one patient with associated hypothyroidism received thyroid supplement in addition to penicillin. On follow-up, dementia of short duration and ischemic stroke improved significantly and clinical status remained the same for ATM; others with mild symptoms improved with residual deficit. CONCLUSION: Syphilis can affect any part of the neuraxis. A high index of clinical suspicion is required to diagnose NS and institute the treatment early, particularly in patients with promiscuous sexual behavior.