Carotid Health in Patients with Cerebral Venous Thrombosis.

Background: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke and shares common risk factors with arterial strokes such as hyperhomocysteinemia, tobacco, alcohol, drugs, and hypercoagulable state. These risk factors can alter both arterial and venous health leading to the occurrence of atherosclerosis in CVT patients. Aims: To evaluate carotid hemodynamics in CVT patients. Settings and Design: Prospective hospital-based case-control study. Methods: This study included 50 consecutive CVT patients and 50 healthy controls. The demographic data, vascular risk factors, clinical data, biochemical, and radiological parameters were recorded. Carotid sonography was performed in CVT patients within the first 24 h of admission. Statistical Analysis: MedCalc 17. Results: The age of the patients was 35.04 ± 9.48 years and the controls 38.88 ± 10.41 years with male preponderance in both groups. Risk factors for atherosclerosis among patients included hyperhomocysteinemia (40 patients), diabetes mellitus (4 patients), hypertension (9 patients), alcohol (17 patients), and tobacco (21 patients). Eight patients had abnormal carotid sonography. Six had nonflow-limiting plaques, one had carotid occlusion, two had increased intimal-medial thickness, and one had increased peak systolic velocity. Among the controls, three subjects had nonflow-limiting plaques. There was no difference in carotid hemodynamic parameters between controls and patients; and those with normal and elevated homocysteine. Conclusion: This is the first study to our knowledge looking at carotid health in venous strokes. The relative risk for carotid atherosclerosis in CVT patients is higher and requires long-term follow-up for the initiation of preventive measures.

Prospective Hospital-Based Clinical and Electrophysiological Evaluation of Acute Organophosphate Poisoning.

INTRODUCTION: Acute organophosphate (OP) poisoning is one of the most common poisoning causing significant morbidity and mortality in developing countries. Acute cholinergic manifestations predominate with many patients requiring intensive care management and ventilator support. Nerve conduction studies including repetitive nerve stimulation can evaluate the altered neuromuscular transmission and peripheral nerve function by OPs. OBJECTIVE: To evaluate the electrophysiological abnormalities in patients with acute OP poisoning and correlate with clinical status. MATERIALS AND METHODS: Patients with acute OP poisoning admitted from August 2016 to August 2017 were prospectively studied. Nerve conduction studies including phrenic nerve conduction were performed within 24 h of admission. Repetitive nerve stimulation was performed at 3 and 30 Hz. Nerve conduction findings were compared with data from age-matched healthy controls. RESULTS: Thirty patients were included (18 men and 12 women) in the study. Their age ranged from 16 to 47 years (30 ± 9.2). The first assessment revealed a mild reduction of compound muscle action potential (CMAP) amplitude and reduced F-wave persistence. Eleven patients had repetitive CMAPs suggesting cholinergic excess. Seven among the 11 patients requiring mechanical ventilation had decrement-increment response with 30 Hz stimulation and reduced diaphragmatic CMAP amplitude (P = 0.02). CONCLUSION: The presence of repetitive CMAPs, decrement-increment response to tetanic stimulation and reduced diaphragmatic CMAP amplitude in OP poisoning patients correlate with neuromuscular paralysis and need for mechanical ventilation.

Therapeutic Plasma Exchange - An Emerging Treatment Modality in Patients with Neurologic and Non-Neurologic Diseases.

INTRODUCTION: Therapeutic Plasma Exchange (TPE) is a procedure in which the patient's blood is passed through an apheresis machine, where the filtered plasma is removed and discarded with reinfusion of red blood cells along with replacement fluid such as plasma or albumin in to the patient. We present our experience with TPE in treatment of various neurologic and non-neurologic diseases. AIM: To evaluate TPE as primary therapy or as a first-line adjunct to other initial therapies as mentioned by American Society for Apheresis (ASFA). MATERIALS AND METHODS: A retrospective analysis of TPE procedures was done for a period of nine years, from January 2007 to May 2016 in a tertiary care teaching hospital. A total of 584 TPE procedures were performed in 161 patients between 2 to 75 years of age. Clinical and laboratory investigations like ECG, chest X-ray, cardiorespiratory status and serology were carried out before the TPE procedure. RESULTS: A total of 161 patients were enrolled in the present study. Guillain-Barre Syndrome (GBS) (67.7%, n=109) was the main indication for TPE, followed by Myasthenia Gravis (MG) (13.04%, n=21). Overall incidence of adverse reactions was 9.93%, inadequate vascular access was a common complication encountered in paediatric age group. CONCLUSION: Our results show that TPE is not only safe and effective treatment alternative to Intravenous Immunoglobulin (IVIG), it also strongly holds evidence in the improvement of neurological disorders compared to non-neurological disorders. There is need of further detail evaluation on large number of cases for proper evidence based practice.

Hospital-based Retrospective Study of Cryptococcal Meningitis in a Large Cohort from India.

BACKGROUND: Cryptococcal meningitis is an important and a fatal neuroinfection. Early diagnosis and treatment is of utmost importance in reducing morbidity and mortality. MATERIALS AND METHODS: Data of patients with laboratory-confirmed cryptococcal meningitis seen in tertiary care hospital were reviewed. Details of demographic profile, clinical data, laboratory parameters, complications, and in-hospital mortality were studied. RESULTS: Among 97 patients with cryptococcal meningitis (79 men, 18 women), 88 were HIV-positive, two were diabetic, and seven were sporadic. Their age ranged from 23 to 67 years (39.16 ± 9.49). Additional pathogens for meningitis were identified in 24 patients. Headache was the most common symptom (91%) followed by fever (66%), vomiting (51%), altered sensorium (31%), and seizures (20%). Neurological deficits included cranial nerve palsies (28), motor deficits (11), sphincter disturbances (5), and sensory involvement in four patients. Complications included renal dysfunction (20%), dyselectrolytemia (20%), seizures (16%), hypersensitivity (7%), and hepatic dysfunction (5%). Favorable outcome was seen in 72 patients, 13 remained unchanged, and 12 died. Rapid clinical progression, low cerebrospinal fluid (CSF) cell count and protein were associated with higher mortality. CSF cell count and protein were lower in patients who had isolated cryptococcal meningitis compared to those with additional pathogen. Mean sugar levels were higher and duration of illness was shorter in HIV-negative individuals. CONCLUSION: Cryptococcal meningitis is common in patients with AIDS. Effective and early antifungal treatment carries a good prognosis. On rapid evolution of the disease, decreased CSF cell count and protein heralds poor prognosis and warrants initiation of early specific treatment.

Intact thumb reflex in areflexic Guillain Barré syndrome: A novel phenomenon.

INTRODUCTION: Areflexia is one of the cardinal clinical features for the diagnosis of Guillain Barré syndrome. However, some patients may have sluggish proximal muscle stretch reflexes. Presence of thumb reflex, a distal stretch muscle reflex has not been documented in Guillain Barré syndrome. MATERIALS AND METHODS: We prospectively evaluated thumb reflex in Guillain Barré syndrome patients and age matched controls from April to September 2013. RESULTS: There were 31 patients with Guillain Barré syndrome in whom thumb reflex could be elicited in all (24 brisk, 7 sluggish), whereas all the other muscle stretch reflexes were absent in 29 patients at presentation and the remaining two had sluggish biceps and quadriceps reflexes (P = 0.001). Serial examination revealed gradual diminution of the thumb reflex (P < 0.001). Rapid progression of weakness was associated with early loss of the thumb reflex. CONCLUSION: Thumb reflex, a distal stretch reflex is preserved in the early phase of Guillain Barré syndrome.

Uncommon dyselectrolytemia complicating Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) and hypokalemic paralysis are common causes of acute flaccid quadriparesis and specific therapeutic interventions differ. Simultaneous occurrence of severe hypokalemia in patients with GBS at the time of presentation can cause diagnostic and therapeutic dilemma. Presence of hypomagnesemia with hypokalemia in patients with GBS can be perplexing and pose further challenges. Evaluation for preexisting inherited or other associated metabolic disturbances is needed in the presence of such complex dyselectrolytemia. We report the rare association of GBS with severe hypokalemia and hypomagnesemia in a 41-year-old male presenting with acute flaccid quadriparesis and the therapeutic challenges faced.

Electromyographic and histological features of postpartum hypernatremic rhabdomyolysis.

INTRODUCTION: Rhabdomyolysis results from many causes including hypernatremia. Postpartum hypernatremia with osmotic cerebral demyelination is a rare cause of reversible rhabdomyolysis. Electromyographic studies in postpartum hypernatremia have not been reported. MATERIALS AND METHODS: Electromyography (EMG) was performed in five women with postpartum hypernatremia and muscle biopsy was performed in one of them. RESULTS: Among the five women presenting with postpartum hypernatremia associated with marked elevation of serum creatine kinase, four had quadriparesis. All had varying degrees of encephalopathy at admission and recovered without residual deficits after gradual correction of hypernatremia. Needle EMG revealed fibrillations with positive sharp waves in five patients and myotonic discharges in three patients. Serial EMG in one patient revealed the occurrence of transient fibrillations, positive sharp waves and myotonic discharges. Muscle biopsy revealed extensive rhabdomyolysis in one patient. CONCLUSION: EMG in hypernatremic rhabdomyolysis revealed spontaneous activity including fibrillations, positive sharp waves and myotonic discharges along with myopathic potentials. Electromyographic findings depend on the interval from the onset and the degree of rhabdomyolysis.

Bethlem myopathy: An autosomal dominant myopathy with flexion contractures, keloids, and follicular hyperkeratosis.

Bethlem myopathy and Ullrich congenital muscular dystrophy form a spectrum of collagenopathies caused by genetic mutations encoding for any of the three subunits of collagen VI. Bethlem phenotype is relatively benign and is characterized by proximal dominant myopathy, keloids, contractures, distal hyperextensibility, and follicular hyperkeratosis. Three patients from a single family were diagnosed to have Bethlem myopathy based on European Neuromuscular Centre Bethlem Consortium criteria. Affected father and his both sons had slowly progressive proximal dominant weakness and recurrent falls from the first decade. Both children aged 18 and 20 years were ambulant at presentation. All had flexion contractures, keloids, and follicular hyperkeratosis without muscle hypertrophy. Creatinine kinase was mildly elevated and electromyography revealed myopathic features. Muscle imaging revealed severe involvement of glutei and vasti with "central shadow" in rectus femoris. Muscle biopsy in the father showed dystrophic changes with normal immmunostaining for collagen VI, sarcoglycans, and dysferlin.

Familial Guillain-Barré syndrome: First Indian report.

Guillain-Barré syndrome (GBS) is the commonest acute immune-mediated peripheral neuropathy. Specific human leukocyte antigen types have been found in patients with axonal and demyelinating subtypes of GBS suggesting genetic susceptibility in the generation of GBS. However, familial occurrence of GBS is rare and 42 patients from 20 families have been reported. Majority of them are from European countries and two families have been documented from Asian countries, while none have been reported from India. Electrophysiological characterization in familial GBS has been limited. We report the clinical and detailed electrophysiological findings in two affected brothers with familial GBS from India who had GBS five years apart. Both of them had mixed axonal and demyelinating features in nerve conductions and had complete clinical recovery. Our report documents the first Indian familial occurrence of GBS. Detailed genetic and epidemiological studies are required to find the true prevalence of familial GBS.

Seasonal postpartum hypernatremic encephalopathy with osmotic extrapontine myelinolysis and rhabdomyolysis.

BACKGROUND: Neurological manifestations secondary to extrapontine myelinolysis and rhabdomyolysis caused by hypernatremia are infrequently reported. Occurrence of neurological manifestations due to spontaneous hypernatremia during postpartum period producing rhabdomyolysis and cerebral parenchymal MRI changes has not been previously reported. OBJECTIVE: Evaluation of clinical and radiological profile of postpartum hypernatremia. METHODS: Clinical, laboratory and radiological data of eleven women in postpartum period presenting with hypernatremic encephalopathy with or without muscle weakness were reviewed. RESULTS: Eleven puerperal women presented with encephalopathy due to hypernatremia from 2007 to 2009. Consciousness was altered in all the patients ranging from confusion to deep coma. Nine patients had quadriparesis, eight had corticospinal and corticobulbar dysfunction, six had ataxia, and seizures occurred in four patients. The patients had hypernatremia ranging from 158 to 199mEq/l with hyperchloremia, markedly elevated serum CK levels (2572 to 61,107U) and azotemia. Ten patients underwent MRI which revealed hyperintensity of corpus callosum in all the patients in T2, FLAIR and diffusion weighted sequences. Symmetrical hyperintensities were seen also in internal capsule, corona radiata, cerebellar peduncles and hippocampus in various combinations. Seven patients improved neurologically with reduction of serum sodium and CK levels while four patients died. CONCLUSION: Hypernatremia is a potentially lethal condition which can produce encephalopathy, osmotic demyelination and rhabdomyolysis. Prompt identification and appropriate management can improve the outcome in these patients.