Bangladesh Journal of Neuroscience

A Case Report of Reverse Split-hand Syndrome: An Overlooked Clinical Sign of Hirayama Disease

Sujan Saha — 2019-07-31

Hirayama disease (HD) is considered to be a relatively benign, slowly progressive and less disabling rare neurological disorder where flexion induced compressive ischemic lower cervical myelopathy causes selective anterior horn cell injury resulting weakness and atrophy of distal upper limb without any pyramidal, spinothalamic and posterior column disturbance. Herein, we report a young male with clinical and imaging features suggestive of Hirayama disease presented with dissociated hand muscle atrophy (hypothenar more affected than thenar) in both hands. This less recognized finding was previously termed as reverse split-hand syndrome just opposite to split-hand syndrome found in amyotrophic lateral sclerosis. We also observe electrophysiological correlation of reverse split-hand syndrome in HD.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 110-114

A Case of Subcortical Band Heterotopia Presented with Epilepsy and Speech Regression

Reaz Mahmud — 2019-07-31

Subcortical Heterotopia is a rare developmental disorder of human brain due to mutation in the DCX or LIS1gene.It is predominantly a disease of female. It usually present with refractory seizure and varying degree of mental retardation. Here a case of 22 years lady who presented with refractory seizure is reported. Her MRI revealed Double cortex and her EEG revealed Frontal intermittent rhythmic delta activity (FIRDA).

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 115-118

Clinical, Autonomic & Electrophysiological Features in Patients with Guillain Barre Syndrome in a Tertiary Care Hospital of Bangladesh

Maftahul Jannat — 2019-07-31

Background: Guillain-Barre Syndrome (GBS) is the most common cause of acute flaccid paralysis in the adult population. It is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis.

Objective: The aim of the study were to evaluate clinical profile, to assess autonomic involvement & electrophysiological findings in adult patients with GBS.

Methods: An observational, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS fulfilling the inclusion criteria were recruited as the study population. Detailed clinical examination, CSF study & nerve conduction study were done. Disability status was measured by Hughes functional grading scale. For autonomic assessment 35 adult healthy control were also included for comparison. Then following tests of autonomic nervous system were performed in both patient and control group 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire.

Results: The mean age of patients was 35±12 years (range18 to 65 years) with slight male predominance (58.1%). Major clinical presentation was weakness of all 4 limbs followed by sensory complaints (44.2%). 7% of the patient had breathing difficulty and dysphagia. Only 4.7 % had diplopia. Among the symptoms of autonomic dysfunction most common symptoms was constipation (30.2 %) followed by palpitation (14%), urinary retention (7%), lightheadedness and urinary incontinence (4.7%). Cranial nerve palsy was present in 34.9% of cases among them facial palsy was found commonly (27.9%), followed by bulbar palsy (7%) and ophthalmoplegia (4.7%). One patient (2.3%) had both facial palsy and ophthalmoplegia. AIDP, AMAN and AMSAN subtypes comprised 32.6%, 37.2% and 20.9% of cases respectively. Regarding autonomic dysfunction variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min on deep breathing (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (4.7%), and sinus arrhythmia (4.7%). Bowel bladder dysfunction was another autonomic dysfunction among them constipation 30%, urinary retention 7% and urinary incontinence 4.7% of cases.

Conclusion: GBS can be presented with variable presentation including autonomic dysfunction. In this study common clinical presentation was limb weakness & different patterns of autonomic dysfunction was found in patients with GBS. Common electrophysiological subtype was AMAN. So in addition to clinical & electrophysiological analysis autonomic evaluation is essential in every patients with GBS as autonomic dysfunction is one of important cause of mortality.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 57-62

Demographic and Clinical Pattern of Headache in Migraine Patients

Bipasha Majumder — 2019-07-31

Background: Migraine is one of the most common neurological disorder and fourth most important factor for debility in human. The presentation of migraine is complex. All patients do not have same features of migraine.

Objectives: The purpose of the study was to evaluate demographic and clinical patterns of headache in migraine patients.

Methods: A total of 30 migraine patients who were visited in the Headache clinic, Department of Neurology, BSMMU, Dhaka were enrolled for the study. Migraine patients diagnosed according to ICHD-3 (International Classification of Headache Disorders 3rd edition) criteria.

Results: Out of 30 patients mean age was 30.63±10.95 years with age range 15-60 years in migraine patients. Female were more common. Positive family history was present in 56.7% patients. Common associated symptoms were photophobia, phonophobia (96.7%) and nausea (83.3%) in migraine patients. Common precipitating factors were stress and sunlight (90%) followed by journey (80%) and insomnia (73.3%). A major portion of migraine was without aura (73.3%) and the ratio of aura to without aura is 1: 2.75. Major portion of migraine patients were complained of 4-6 attacks/ month (46.7%) which was followed by 1-3 attacks/month (36.7%). Most of the migraine patients complained as moderate headache (60%) followed by severe headache (40%).

Conclusion: This study concluded that migraine is a disease of younger age group and it affects female more commonly than male, pattern of headache in migraine patients is unique.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 63-68

Infarct Pattern in Patients with Varying Degrees of Internal Carotid Artery Stenosis

Md Abdullah Al Muzahid — 2019-07-31

Background: Internal carotid artery (ICA) is one of the commonest site stenosis in patients with ischemic stroke. There is difference in the distribution of stenosis among different sites of cerebral infarct.Volume and severity of cerebral infarct may also depend on the degree of stenosis. To plan efficient evaluation and treatment of individual patient of ischemic stroke, the responsible clinician must be familiar with the relative probability of finding occlusive lesions at various sites within the vascular tree.

Objective: The objective of this study was to evaluate the angiographic pattern of ICA stenosis among different types of cerebral infarct.

Materials and Methods: We evaluated 53 ischemic stroke patients from indoor, outdoor, stroke and neuro intervention clinic, BSMMU. CT scan and/ or MRI of brain were done to each patient to confirm the diagnosis. After vascular imaging, the degree of stenosis was measured by the NASCET formula.

Results: Cervical segment of ICA was most commonly [n=45(84.9%)] encountered site of stenosis and total occlusion of ICA was always observed in cervical segment. Among patients with moderate stenosis (n=15) of ICA, 6(40.0%) presented with subcortical infarction, 4(26.7%) presented with lacunar infarction and 5(33.0%) presented with territorial infarction. In case of severe stenosis (n=23), territorial, lacunar and watershed infarct were 9(39.1%), 8(34.8%) and 2(8.7%) respectively. Whereas total occlusion (n=15) of ICA presented as either territorial infarction [n=11(73.3%)] or watershed infarction [n= 4(26.7%)]. These differences between severity of stenosis and subtype of infarct were also significant (p-value = 0.003).A total of 25 patients presented with territorial infarction, mostly MCA territory [n=23(92%)].

Conclusion: With increasing severity of stenosis, the infarct burden rises. ICA stenosis of e”70% mainly presented as territorial infarction whereas, watershed infarct was an indicator of severe stenosis.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 69-77

Prognostic significance of Intracerebral Hemorrhage Score in predicting 30-day mortality in Chittagong Medical College Hospital

Md Shawkat Emran — 2019-07-31

Background:Prognosticating the outcome of Intracerebral Hemorrhage (ICH) at the time of admission is important to customize treatment in a cost-effective manner in such cases. ICH sore is a widely used prognosticating tool but yet not evaluated in our setting. This study was aimed to assess the prognostic factors influencing outcome and validating the ICH score for prediction of 30-day mortalityin hospitalized patients with ICH.

Materials and methods:This prospective observational study was conducted in Chittagong Medical College Hospital, Bangladesh among 105 consecutively admitted patients aged 18 years and above with a computed tomography evidence of spontaneous ICH. ICH score was calculated soon after confirmation of diagnosis. Primary outcome measure was 30-day mortality after admission. Modified Rankin Scale (mRS) was used to assess outcome at discharge and at 30-day follow up.

Results:A total of 104 patients were analyzed. Mean age of this cohort was 59.30±19.91 years. At 30 days all 27 patients with an ICH score of 0 survived, whereas those having scores of 1, 2, 3, and 4 had 5.9%, 33.3%, 46.2% and 88.9% mortality, respectively. ICH score was good for discriminating 30-day mortality with having an area under the ROC curve of 0.886 (95% CI:0.816-0.956; p<0.001]. For patients scoring above 2, the rate of poor functional outcome (mRS score e”4) approaches 100%. On the other hand, 18.5% of patients with score of 0 and 64.7% of patients with a score of 1 are not functionally independent after 30 days.

Conclusion:In conclusion, the present study has demonstrated that the ICH score is a strong prognostic indicator of ICH outcomes (30-day mortality and 30- day functional outcome) among hospitalized patients in Bangladesh.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 78-85

Impact of Risk Factors on the Size of Ruptured Intracranial Saccular Aneurysms

Md Humayun Kabir Sarker — 2019-07-31

Background: Un-ruptured intracranial aneurysms (UIAs) are common and prevalence is about 2 to 8%. Several studies have shown that the decision to treat un-ruptured aneurysms should not be based on aneurysm size alone. A study suggest that treatment of UIAs smaller than 7 mm in hypertensive patients and smokers may be beneûcial.

Aim and objective: The goal of this study is analysis of correlation of age, gender, location of the aneurysm, history of hypertension and cigarette smoking, previous history of SAH with the size of ruptured aneurysms.

Materials and Methods: This hospital based observational cross-sectional study was conducted in the Department of Neurology & Neurosurgery, Dhaka Medical College Hospital (DMCH), Dhaka. Total 44 patients with SAH were taken by inclusion & exclusion criteria. The aneurysms size, site of location and aneurysm multiplicity was assessed by three-dimensional rotational digital subtraction angiography (DSA).

Results:The mean age of the study population was 49.24 ±11.5. About half of the population were within 51-60 years. The male female ratio was 1:1.2. Out of 44 population, 93.2% were presented with headache, 90.9% with vomiting. In this study aneurysms mean size was 5.72±4.010 mm. 93.2% of aneurysms were below 10 mm, 75.0% were below 7 mm and 50.0% below 5 mm. Size of ruptured aneurysm is small in hypertensive population and is significant (p-value 0.037). Aneurysm size was significantly (p- value 0.013) smaller in case of smoker. Mean aneurysm size in hypertensive smoker population was significantly (p-value-0.004) smaller than hypertensive non-smoker. Population with one risk factor had mean aneurysm size was 8.32±6.84 mm, two risk factors had 5.26±1.86mm, three risk factors had 4.79±2.05 mm and more than three risk factors had 2.85±1.43.

Conclusion: This study shows that more the risk factors, smaller the size of aneurysms. Therefore, history of hypertension, cigarette smoking, female sex, age and positive family history should be considered in the assessment of treatment of un-ruptured intracranial aneurysms.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 86-94

Study of Rare Cases of Congenital Myasthenic Syndrome in Bangladesh

Akter Banu — 2019-07-31

Congenital myasthenic syndromes (CMS) comprise a heterogeneous group of rare inherited diseases in which the neuromuscular transmission in the motor plate is compromised by one or more genetic pathophysiological specific mechanisms are characterized by fatigable weakness of skeletal muscle (e.g., ocular, bulbar, limb muscles) with onset at or shortly after birth or in early childhood; rarely, symptoms may not manifest until later in childhood. The diagnosis of CMS is based on clinical findings, a decremental EMG response of the compound muscle action potential (CMAP) on low-frequency (2- 3 Hz) stimulation, absence of anti-acetylcholine receptor (AChR) and anti-MuSK antibodies in the serum, a positive response to acetylcholinesterase (AchE) inhibitors and lack of improvement of clinical symptoms with immunosuppressive therapy. Pathogenic variants in one of multiple genes encoding proteins expressed at the neuromuscular junction are currently known to be associated with subtypes of CMS. The most commonly associated genes include: CHAT, CHRNE, COLQ, DOK7, GFPT1 and RAPSN. We studied on a sibling presented with progressive fatigability and fluctuating ptosis with frequent exacerbations of muscle weakness during infections since infancy. On both cases CT scan of chest were negative for thymoma, antibodies against the acetylcholine receptor (AChR) and the muscle specific kinase (MuSK) were negative and decremental response on electrophysiological study of Repetitive nerve stimulation (RNS) and EMG were consistent with disease of neuromuscular junction (post synaptic) and they were only on pyridostigmine for long time with marked improvement of symptoms and signs. Considering all scenario both of our cases mostly fits with the autosomal recessive, post synaptic CMS associated with Rapsyn deficiency.

Objective : As in Bangladesh, there is inadequate data on the epidemiological profile of CMS, our aim is to describe these cases for their rarity and the difficulty encountered in diagnosis as they are easily confused with Juvenile Myasthenia Gravis (JMG) and familial myopathies. As both the cases are very rare, it should be an original article.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 95-103

Understanding Hemifacial spasm: A Review Of An Uncommon Movement Disorder

Nayeem Anwar — 2019-07-31

Hemifacial Spasm is a rare condition and often misdiagnosed even by neurologists. Although etiology, pathophysiology is clear, treatment is unsuccessful in many cases. In this review article we focus on etiology, pathophysiology, diagnosis and management of this infrequent movement disorder.

Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 104-109