Nepal Journal of Neuroscience

Disease Modifying Therapies in Multiple Sclerosis: A Review

Mohsin Raza — Fri, 21 Jul 2023 00:00:00 +0000

Multiple Sclerosis is a autoimmune demyelinating disease of the nervous system.This review paper evaluates disease modifying therapies for multiple sclerosis , a chronic autoimmune disease of the central nervous system. Over 20 Disease Modifying Therapies are available, categorized into injectables, oral agents, and infusions. The review covers their efficacy, safety, and tolerability. Established injectable Disease Modifying Therapies include interferon beta and glatiramer acetate, while newer oral agents like fingolimod offer conveniencewith more adverse effects. Infusions like natalizumab have higher efficacy but greater risks of adverse events. The paper also discusses newer Disease Modifying Therapies like cladribine. Treatment should be individualized based on patient characteristics, risk factors, and treatment goals. Overall, this review provides a comprehensive summary to aid clinicians in selecting appropriate Disease Modifying Therapies for Multiple Sclerosis.

“When the wind blows, it hurts” – Neuromylitis Optica presenting with Paresthesias and Allodynia

Justin Hicklin, Thomas Varkey, Luke Kloft — Fri, 21 Jul 2023 00:00:00 +0000

Patients with Neuromyelitis Optica Spectrum Disorder (NMOSD) and Multiple Sclerosis (MS) often present with similar, nonspecific symptoms. The relationship between the two diseases has long been disputed and their comparable presentation often creates a challenge in diagnosing patients on presentation alone. In this particular case, we present a patient that has symptoms, imaging, and a physical exam pointing to a likely diagnosis of MS. Further abnormal MRI findings continued high concern for possible early-stage MS. However, cerebrospinal fluid analysis yielded findings that revised the diagnosis to NMOSD. This particular case is intriguing as it demonstrates the importance of completeness of work up as this in the end changed the management of the patient.

A rare cause of Hemiplegia

Baishyak Renuji, Joby Jose, Karthika Ajith, Avin Eldho — Fri, 21 Jul 2023 00:00:00 +0000

Stroke is the most likely diagnosis considered, when an elderly patient comes to ER with acute onset hemiplegic weakness. Here we report a case of 82-year-old male, known diabetic and hypertensive with complaints of weakness of left upper limb and lower limb since one day. His MRI brain was normal and MRI cervical spine screening showed epidural hematoma which was later evacuated resulting in improvement of symptoms. SSEH is a rare entity, its presentation as hemiplegia is very rare and has not been reported previously. Keeping SSEH as a possibility in MRI brain negative patient presenting with hemiplegia can be beneficial to the patient as early surgical intervention will provide a good outcome.

Diastomatomyelia- A Case Series

Uddalok Das, Narayan Pandit — Fri, 21 Jul 2023 00:00:00 +0000

Diastematomyelia also called a split cord syndrome is a rare clinical entity. A group of concealed spinal dysraphisms includes this unusual congenital spinal cord defect known as diastematomyelia. The condition is characterized by a sagittal split of the spinal cord,conus medullaris, or filum terminale. There is often a fibrous, boney, or cartilaginous septum identified by dividing the two cords. It is frequently associated with other congenital anomalies of the spine and the cord. Presentation is varied from asymptomatic to lower back pain, lower extremity weakness, and spinal cord dysfunction. Imaging plays a vital role in the diagnosis. We present a series of 2 cases of diastematomyelia along with imaging features. One of the patients is also a case of Jarcho Levine syndrome (JLS) making it the eighth case in world medical literature to report a case of JLS with diastematomyelia.

Pathological Laughing in a Patient with a Pontine Tumor

Gopi Nepal, Pritam Gurung, Anik Jha, Durga Khadka, Resha Shrestha, Basant Pant — Fri, 21 Jul 2023 00:00:00 +0000

An 18-year-old man presented with the complaints of occasional headache and limb weakness associated with slurring of speech and purposeless laughing. Magnetic resonance imaging showed a diffuse altered signal intensity area involving the pons with asymmetrical expansion. The patient underwent surgical resection. Histology was suggestive of a WHO grade IV glioblastoma. Pathological laughing in a patient with a pontine lesion is a very rare presentation. Evidence for a mechanism of action underpinning how pontine lesions can cause such behavioral changes through the disruption of a network of cerebro-ponto-cerebellar pathways is discussed.

Challenges and scope of movement disorder surgery in Nepal

Resha Shrestha, Pritam Gurung, Basant Pant — Fri, 21 Jul 2023 00:00:00 +0000

Movement disorders (MD) are clinical syndromes with either excess or paucity of voluntary or involuntary movements without weakness or spasticity. Hypokinetic MD includes primary and secondary Parkinsons disease and Parkinsons syndrome and hyperkinetc MD includes dystonia, tremors, choreas and dyskinesias. Although medical treatment is the mainstay of treatment modality, in selected cases surgical treatment is a safe option

A Rare Triple Neural Tube Defect- Case Report

Amit Kumar, Kislay Kumar — Fri, 21 Jul 2023 00:00:00 +0000

Multiple neural tube defects are a rare entity and it also includes anomalies like encephalocele, dermal sinus, myelomeningocele, and spina bifida. These occur due to defective closure of neural tube at multiple sites. There have been isolated cases of coexistence of triple neural tube defect in same subject and only 9 cases have been reported in available world literature.

Here, we present a rare case of 7 days old boy with three different NTDs- occipital encephalocele, upper cervical meningocele and lumbar myelomeningocele. Type II Chiari malformation, tethered cord, fenestration of posterior falx, hydrocephalus and syringohydromyelia accompanying the NTDs.

Intraventricular Meningiomas - Case Series and Review of Literature

Anand Shanmugaiah, R Raj Kumar — Fri, 21 Jul 2023 00:00:00 +0000

Introduction: Meningioma is the most common benign primary intracranial tumour after glioma. Meningioma occuring in intraventricular region is very rare. When located in intraventricular space, majority of intravemtricular meningioma (IVM) are located in lateral ventricles.

Material and methods: This is a Study of four cases of intraventricular meningioma in Madras medical College from January 2022 to December 2022. In this study, the symptoms, MRI findings, operative procedure, histology images, surgical outcomes and a detailed review is done from various literature.

Results: Among the various symptoms, headache, disturbance in vision, changes in cognition and speech disturbance were commonly found. The site of all the lesion was in the lateral ventricle. MRI was used to confirm the diagnosis before surgery. In our patients, the intraventricular meningioma was completely excised via parietal craniotomy or temporoparietal craniotomy based on the location of the tumour. The tumour was completely excised in all four patients. Histologically it was meningothelial meningioma in all 4 patients.

Conclusion: From our case series we observed that all the IVM were WHO grade I and total excision is possible in intraventricular meningiomas with properly planned surgical trajectory with minimum post operative morbidity.

The efficacy of intracisternal irrigation of papaverine on cerebral blood flow in patients with subarachnoid hemorrhage

Anand Shanmugaiah, R Raj Kumar, VT Suresh Babu — Fri, 21 Jul 2023 00:00:00 +0000

Background: Cerebral vasospasm remains the most common cause of death in patients after subarachnoid hemorrhage despite various treatments. This study was conducted to find the efficacy of intracisternal washing with papaverine on cerebral blood flow.

Materials and Methods: This study was performed on 40 patients and totally 120 arteries in 2022. Cerebral blood flow velocity changes before and after washing with papaverine were measured and analyzed.

Results: Twenty (20) patients with anterior communicating artery (ACOM) aneurysm and 20 patients with middle cerebral artery (MCA) aneurysm were assessed. Mean blood flow velocity before aneurysm and before washing in ACOM and MCA was 66.2±13.8cm/s and 62.86±7.3cm/s, respectively, which reached 20.45±4.17 cm/s and 32.1±7.7 cm/s, respectively after washing (P value = 0.016 and 0.024).

Mean blood flow after aneurysm and before washing in ACOM and MCA was 93.2±10.9 cm/s and 69.44±12.2 cm/s, respectively, which reached 33.29±4.2cm/s and 40.01±5.28 cm/s, respectively after washing (P value = 0.001 and 0.01).

Conclusion: Intracisternal irrigation with papaverine significantly reduces cerebral blood flow and relieves vasospasm.

Surgical outcome of brain arteriovenous malformation according to the location, A retrospective study

Fri, 21 Jul 2023 00:00:00 +0000

Background and Objective: The goal of this study was to analyze the association of brain arteriovenous malformations (AVMs) location and its outcome after microsurgical excision. The relation of outcome using modified Ranklin Scale (mRS) at time of discharge, early and last follow ups with respect to location of AVM.

Methods: Demographic data, AVM characteristics, and treatment outcomes were evaluated in 47 bAVMs treated with microsurgery between November 2009 and April 2021. For this series, 47 patients were retrospectively reviewed. The mRS was used to assess functional outcome post-surgery with respect to its location

Outcomes: Forty-seven patients (32% female) and 68% male; average age 33.4 years) with brain AVMs were included in this review. Among the AVMs, the mean size was 5.5cm, 83% with hemorrhage, 63% with deep drainage, mean SM grade is 2.74. The non-eloquent AVM comprised of 25.5%. The most common eloquent location was language (36.2%), followed by sensorimotor (23.4%), visual 8.5% and coordination 6.4%. Motor mapping was done in all cases of brain AVM with sensorimotor eloquence. There was no statically significant difference in the baseline patient and AVM characteristics among different subtypes of eloquence. Favorable outcome (mRS 0-2) was seen on both eloquent (100% in visual) and non-eloquent AVMs. Age and AVM size were significant predictor for good overall outcome in last follow-up.

Conclusion: Although the sample size of this study is a limitation, the result of our case series demonstrated excellent surgical outcome for visual eloquence, good outcome in sensorimotor and language eloquence. Coordination eloquence had worse overall outcome.

Correlating magnetic resonance imaging characteristics with motor outcome in cerebral palsy children with white matter injury

Nilima Ananda Narkhede, Sushma Save — Fri, 21 Jul 2023 00:00:00 +0000

Introduction: Cerebral palsy (CP) is the term used to describe individuals with a movement disorder resulting from non-progressive disturbance to the developing brain. The diagnosis of CP is based on the presence of a motor deficit, and they manifest with reduced motor capacity. The most widely utilized classification system for the severity of motor limitations is the gross motor function classification system (GMFCS).

Material and Methods: We tried to describe the white matter injury on Magnetic Resonance Imaging (MRI) in children with cerebral palsy irrespective of clinical subtype. We also studied if there was association between white matter injury on MRI in children with cerebral palsy and motor outcomes-gross motor function classification system Expanded and Revised(GMFCS E &R). It was an observational, Cross sectional Descriptive study with analytical wing aged between 6 months to 12 years of age carried out for 8 months. Children diagnosed to have cerebral palsy on history and clinical examination were enrolled in the study.

Results: A total of 50 children diagnosed with cerebral palsy were enrolled in our study. Majority of the study sample were <5years old (29) and males (31). We found that symmetrical involvement, severe white matter loss in the hemisphere and callosal thinning were the strongest predictors of poor gross motor function. Bilateral, extensive WM (white matter) loss was more likely to result in quadriplegia, whereas volume loss in the posterior-mid WM more frequently resulted in diplegia. Asymmetrical involvement was associated with less extensive hemispheric involvement than symmetrical WMI (White matter injury). Cysts, deep grey abnormality, and Wallerian change in the PLIC (Posterior limb of Internal Capsule) and cerebral peduncles were all more commonly seen in association with unilateral and asymmetric patterns of WMI. All children with unilateral WMI functioned at GMFCS levels I or II or III whereas bilateral involvement was associated with wheeled mobility (GMFCS levels IV–V). Motor outcomes for children with asymmetrical WMI fell between those with symmetrical and unilateral WMI on MRI.

Conclusion: Laterality/ symmetry of WMI along with extent and location of WM loss can predict gross motor function in Cerebral palsy.

Cubital tunnel syndrome: A retrospective analysis of surgical management

Prabin Shrestha, Isha Dhungana, Manish Kolakshyapati — Fri, 21 Jul 2023 00:00:00 +0000

Introduction: Cubital tunnel syndrome (CuTS) is a condition of ulnar nerve dysfunction due to prolonged compression at the elbow often caused by cubitus valgus deformity of lateral epicondyle fracture. The main objective of this study is to share our experience of and to review the role of surgical decompression and anterior transposition (SDAT) of ulnar nerve in long-term relief of the symptoms.

Materials and Methods: Retrieval of case records of CuTS and retrograde analysis of data was performed. The inclusion criteria were availability of relevant clinical information, finding of severe nerve compression in nerve conduction study (NCS), insignificant MRI of cervical spine and SDAT. About 100 cases of ulnar nerve compression underwent surgical management from 2008 till 2021. Of them, only 54 fulfilled all the criteria. Minimum follow up period was 18 months after surgery.

Results: Among 54 cases, 20 were females, 34 males, and mean age was 31 years. Three most common clinical features were paresthesia, motor weakness and hand muscle atrophy. Symptomatic relief was achieved in 54/54 (100%) and complete motor recovery in 34/54 (63%) cases after SDAT. However, muscle atrophy recovery was not observed in any. There was no surgical complication, no features of recurrence nor was there re-surgery in any at least for 18 months after surgery.

Conclusions: SDAT provides long-term relief for CuTS without recurrence. Muscle atrophy is irreversible though other symptoms improve. Therefore, early surgery before appearance of muscle atrophy is advocated.

Quality of life in family members of patients of Schizophrenia and Bipolar Mood Disorder: A crosssectional study in a sample of the Indian population

Alisha S. Patil, Sanjiv S. Kale — Fri, 21 Jul 2023 00:00:00 +0000

Introduction: Schizophrenia and bipolar disorder are chronic mental diseases with far-reaching effects on those who suffer from them and people who care for them. Family members suffer physical and tremendous emotional distress, often reflected in daily activities and functioning. The quality of life deals with how well a person can do their daily tasks. We have tried to assess the quality of life of family members of people with schizophrenia and bipolar disorder.

Materials and Methods: A cross-sectional analysis was performed at a Tertiary Care Centre located in Maharashtra. The research was conducted over the course of six months. After gaining consent, 70 family members of patients diagnosed with schizophrenia as well as bipolar illness were chosen. Sociodemographic data were collected from participants and the quality-of-life instrument (WHO-QoL-BREF) was administered to them. Statistical analysis: SPSS-PC -25 version was used for data analysis. The Shapiro-Wilk test was used to investigate normal distribution. The difference in means between the groups was investigated using student t-test or Mann-Whitney U-test. Statistical differences between supplied proportions was calculated by the chi-square/exact Fisher’s test. Multivariate linear regression analysis was done to see the factors associated with quality of life. Statistics were deemed significant at a P <0.05.

Results: In all four dimensions of the WHO-QoL-BREF, those caring for patients with schizophrenia fared worse than those caring for patients with BMD, most noticeably in the physical category (p=0.001).

Conclusions: Interventions in the form of psychoeducation and provision of psychosocial support may enhance the welfare of caregivers.

Visual field defects in neuro-ophthalmological diseases at a tertiary hospital in Nepal

Fri, 21 Jul 2023 00:00:00 +0000

Purpose: This retrospective study aims to describe the pattern of visual field defects among patients presenting with neuro-ophthalmological diseases in a tertiary hospital of Kathmandu.

Materials and Methods: Medical records of patients undergoing Goldmann visual fields (GVF) for neuro-ophthalmological diseases from July 2019 to June 2021 were retrospectively reviewed. Patient demographics, laterality of the disease, clinical diagnosis and pattern of visual field defect were evaluated and entered and analyzed using SPSS version 20 using descriptive univariate and bivariate analysis.

Results: A total of 216 subjects (378 eyes) with the mean age 34.52±14.80 years were included in the study. The visual field defects were divided 4 categories: optic nerve disorders (76.4%, n=165), chiasmal disorders (17.6%, n=38), retrochiasmal disorders (2.8%, n=6) and miscellaneous (3.2%, n=7). Disc edema/papilledema was the most common diagnosis in optic nerve disorders with enlarged blind spot (79.1%) and constriction of visual fields (18.3%) as pattern of visual field defects followed by optic neuritis. Common lesions affecting the chiasma in our study were craniopharyngioma on whom 50% had the classical temporal field involvement (bitemporal-20%, inferotemporal-20% and superotemporal-10%) and pituitary lesions where 30% showed constriction of peripheral fields, 57.5% showed temporal field defects (mostly superotemporal) in both eyes. Retrochiasmal lesions were less common but all showed a bilateral and homonymous pattern of visual field defect.

Conclusion: Optic nerve disorders were the most common group of conditions undergoing visual fields. Understanding the varied patterns of visual field defects can aid in early diagnosis and guide timely treatment of common neuro-ophthalmological diseases.

Dr. Juha Hernesniemi, Visionary Neurosurgeon and Compassionate Healer

Prakash Kafle — Fri, 21 Jul 2023 00:00:00 +0000

Dr. Juha Hernesniemi, a visionary in the field of neurosurgery, passed away on June 27, 2023, at the age of 75. Born on May 3, 1948, in Helsinki, Finland, Dr. Hernesniemi dedicated his life to advancing the understanding and treatment of complex neurovascular disorders.