An Epidemiological Case Control Multi-centre Trial on Cerebral Venous Sinus Thrombosis in Western Maharashtra, India.

INTRODUCTION: Cerebral Sino-Venous thrombosis (CSVT) is common in India; this country has a heterogeneous population. Genetically and physio-gnomically this population differs in their diet as well as in their environment. Despite these differences CSVT has been described from all quarters of India; a common factor embracing all these patients could be nutrition. OBJECTIVES: An epidemiological, case- control, multi-centre trial was carried out in patients of CSVT. A common factor underlying this could be nutrition which has not been highlighted in several studies. Hence, we studied the nutritional aspects of these patients. METHOD: 63 patients of CSVT and 62 controls enrolled prospectively and followed for a year were investigated with special emphasis on their nutritional status. RESULTS: The triceps skin fold thickness, energy baseline, serum Proteins, Albumin, Hemogram and Platelet counts were lower in patients than in the controls while serum Homocysteine, carbohydrates and fats were higher in patients than in controls. CONCLUSION: The results of this study confirm nutritional deficiencies in patients of CSVT and it begs the question of whether nutrition in any way is causal in CSVT. Larger multi-centric trials will help establishing causality. The study also shows that routine evaluation of thrombophilia factors and immunological tests are not necessary in CSVT.

Case Report: Chorea as a Rare Manifestation of Secondary Adrenal Insufficiency.

The word "chorea" comes from the Latin word "choreus," which means dancing movement. Chorea is defined as a hyperkinetic movement disorder characterized by uncontrolled, unintended, jerky, brief, irregular, random movements involving the limbs or facial muscles. Here, we discuss the case of a 48-year-old male with hypothyroidism for two years, which is well-controlled with medication. He presented with behavioral disturbances for the past seven months and choreiform movements affecting all four limbs, his tongue, and his face for the past six months. Investigations revealed hyponatremia and low serum osmolality. An MRI of the brain showed the empty sella sign. Further investigations revealed low levels of adrenocorticotropic hormone (ACTH), prolactin, and testosterone. Considering the diagnosis of chorea with euvolemic hyponatremia due to secondary adrenal insufficiency, the patient was started on tetrabenazine, trihexyphenidyl, oral hydrocortisone, and gradual correction of sodium level. The patient's condition improved during the hospital stay, and he continues to do well in routine follow-ups.

Benign Intracranial Hypertension: A Rare Manifestation of Neurosarcoidosis.

Sarcoidosis is an immune-mediated disease that can involve multiple systems. Sarcoidosis of the nervous system or neurosarcoidosis may present as cranial mononeuropathy, hypothalamic involvement, aseptic meningitis, granulomatous inflammation in the brain parenchyma or spinal cord, peripheral neuropathy, and, in rare cases, as myopathy and benign intracranial hypertension. The most common cranial nerve involvement is the facial nerve, which can present as unilateral or bilateral facial nerve palsy, often with recurrent episodes. Involvement of other cranial nerves such as the second and eighth cranial nerves has also been reported. Granulomatous inflammation in the spinal cord presents as myelopathy or radiculopathy. Peripheral neuropathy can manifest as mononeuropathy, mononeuritis multiplex, or generalized sensory-motor neuropathy. Carpal tunnel syndrome is more common in patients with sarcoidosis compared to the general population. Here, we describe the case of a 40-year-old female who presented with heaviness of the head and blurred vision, with a prior history of left-sided Bell's palsy. Bilateral papilledema was observed during the fundus examination. MRI of the brain revealed signs suggestive of benign intracranial hypertension. The cerebrospinal fluid (CSF) opening pressure was measured at 40 cmH2O. Biopsy of bilateral hilar lymphadenopathy indicated granulomatous inflammation consistent with sarcoidosis. The patient was started on steroids and acetazolamide, and she had a dramatic improvement in symptoms.

Neurolymphomatosis as an Initial Presentation of Non Hodgkins Lymphoma: A Case Report.

Neurolymphomatosis (NL) is a rare clinical disease where neoplastic cells invade the cranial nerves, roots, plexus, or other peripheral nerves in patients with hematologic malignancy mainly Non-Hodgkins Lymphoma(NHL). Primary NL occurs as the first manifestation of a hematologic malignancy. We report a 68-year male who presented to us with low backache and burning paraesthesia in the back of both lower limbs followed by a left foot drop. The clinical and electrophysiological examination was suggestive of bilateral lumbosacral radiculopathy involving L2-S1 roots. Plain MRI of the lumbosacral spine was normal. F18FDG PET CT Scan revealed increased uptake in both L5 and left L3 roots. Contrast-enhanced MRI of the lumbosacral spine showed marked fusiform thickening and enhancement of both L5 and left L3 roots CT-guided Biopsy from left L5 root, lymph node, and bone marrow was suggestive of large B cell lymphoma-germinal center cell type. The diagnosis was neurolymphomatosis secondary to NHL.

Acute Subdural Hematoma: A Rare Manifestation of Cerebral Venous Sinus Thrombosis.

Cerebral venous sinus thrombosis (CVST) usually presents with headaches, seizures, and signs and symptoms of raised intracranial pressure (ICP). Risk factors for CVST commonly include hypercoagulable states such as pregnancy and the peripartum period, dehydration, vitamin B12 deficiency, hyper-homocysteinemia, coagulation factor deficiency, antiphospholipid antibody disease, oral contraceptive pill intake, etc. CVST with venous hemorrhagic infarction is commonly reported, but only a few cases have been reported in the literature of CVST presenting as SDH. Here, we present a case of a 28-year-old female who presented with an acute onset of severe headache, vomiting, and bilateral papilledema on fundus examination. She had a prior history of oral contraceptive pill intake. An MRI brain venogram suggested CVST involving the superior sagittal sinus, right transverse, and a few cortical vein thromboses with subdural hematoma (SDH) in the frontal-parieto-temporo-occipital region on the right side. The patient was treated with anticoagulants and antiepileptics and had significant improvement in symptoms with the resolving SDH on subsequent scans.

Neuromyelitis Optica Presenting as Non-Communicating Hydrocephalus: A Case Report.

Neuromyelitis Optica (NMO) is a demyelinating disease predominantly involving optic nerves, spinal cord and peri-ventricular regions which are rich in Aquaporin-4 receptors. Aquaporin-4 (AQP4) antibodies are implicated in the pathogenesis of NMO. Association of hydrocephalus ( communicating and non communicating) with NMO is very rare. We report a case of 32 years old female patient who presented with 2 months history of progressive headache, visual obscurations and gait imbalance . Clinical examination revealed bilateral papilloedema with preserved visual acuity. She had truncal and gait ataxia. Rest of the examination of nervous system was normal. MRI brain showed non- communicating hydrocephalus and T2 and FLAIR hyperintensities in periventricular and periaqueductal regions. AQP4 antibodies were positive in serum and negative in cerebrospinal fluid(CSF). Ventriculo - peritoneal shunt was placed and she was treated with steroids and azathioprine. Her headache and visual symptoms improved. However, after 8 months she presented with acute optic neuritis of right eye which was treated with intravenous methylprednisolone and plasmapharaesis.

Psychiatric Symptoms and Fatigue in COVID-19 Survivors.

INTRODUCTION: Psychiatric symptoms and fatigue are common after the coronavirus disease 2019 (COVID-19) illness. The cause of these symptoms is direct neuronal injury and indirect injury with immune-mediated inflammation. In addition, social factors also affect mental health. OBJECTIVE: We aim to compare psychiatric symptoms and fatigue between COVID-19 survivors and healthy controls. MATERIAL AND METHODS: We prospectively evaluated 100 COVID-19 survivors for anxiety, depression, positive affect, and behavior control using the Mental Health Inventory (MHI). Fatigue is assessed using the Modified Fatigue Impact Scale (MFIS) score. We compared them with 100 healthy controls. RESULTS: There was a significant statistical difference between the MHI score and individual components of MHI. Overall, MHI scores in cases and controls were 79.41 and 93.31, respectively, with a P value of less than 0.0001. Computed scores for anxiety, depression, behavior control, and positive affect of COVID-19 survivors showed statistically significant differences as compared to healthy controls. There was a weak association between hospital stay duration and poor MHI scores. Fatigue was significantly worse in COVID-19 survivors, with a mean score of 6.93 in cases and 5.35 in controls, with a P value of 0.0001. This was a cross-sectional study evaluating psychiatric symptom scores, but not establishing the diagnosis. It is suggested that appropriate treatment and counseling for these symptoms should be done. CONCLUSIONS: Psychiatric symptoms and fatigue were significantly more common in COVID-19 patients after recovery from acute illness. It is a major contributing cause of morbidity other than organic complications of COVID-19 and requires attention in management.

Predictors of disease severity in COVID-19 associated mucormycosis: impact of HbA1C levels, time lag to mucormycosis onset, and radiologic patterns of paranasal sinuses and spaces involvement.

BACKGROUND: Identifying early predictors of severe Covid-19 associated mucormycosis (CAM) can help improve management and treatment outcomes. OBJECTIVES: Primary: To identify clinical and radiological predictors of disease severity in CAM. Secondary: To describe patterns of central nervous system (CNS) involvement in CAM. METHODS: A total of 71 patients with CAM were included in the study. Based on the anatomical extent of involvement on MRI, patients were divided into three groups: Sinus (paranasal sinuses), Orbit (orbital spread), and CNS (CNS spread). Clinical parameters and radiological patterns of involvement of sinuses and extra sinus spaces were studied between the three groups. Patterns of CNS involvement were also described. RESULTS: A shorter time lag between COVID-19 infection and CAM, as well as high HbA1C levels, were found to be associated with severe disease. Involvement of the sphenoid, ethmoid and frontal sinuses, T1 hyperintense signal in the sphenoid, as well as bony involvement of the sphenoid sinus, were significantly associated with severe disease. Extra-sinus spread into pre/retroantral space, pterygopalatine fossa, and masticator spaces were also significantly associated with a severe disease course. The most common pattern of CNS spread was cavernous sinus involvement, followed by pachymeningeal spread and cranial nerve involvement. CONCLUSION: Early identification of the above-described predictors in patients presenting with CAM can help detect those at risk for developing severe disease. A longer duration of amphotericin, combined with a more aggressive surgical approach in selected cases, may lead to better long-term outcomes.

Acute Onset Flaccid Paraplegia with Monocular Diminution of Vision in a Case of Chikungunya Infection.

Chikungunya is a common tropical viral infection in India. The majority of patients have limited systemic manifestations. Neurological manifestations of chikungunya may be due to direct viral infection or immune mediated. We present a case of a 45-year-old male who presented with acute onset paraplegia with diminution of vision in the right eye. A detailed evaluation revealed a diagnosis of chikungunya myeloradiculitis with viral keratitis. The patient was treated with steroids followed by intravenous immunoglobulin and had a good recovery.

Novel transthyretin gene mutation in familial amyloid neuropathy in India: Case.

Familial amyloid polyneuropathy (PN), also known as amyloid transthyretin (TTR)-PN is an autosomal dominant adult-onset fatal disease, if not treated. It occurs due to mutations in (TTR) gene which leads to a faulty TTR protein which folds up to form amyloid and gets deposited mainly on nerves and causes length-dependent PN and autonomic dysfunction. We report a case of a 45-year-old female who presented with symptoms of painful peripheral neuropathy for 5 months, a history of deafness for 5 years, and cardiac pacemaker implantation 2 years ago for complete heart block. She denied any symptoms of autonomic dysfunction. Her brother with similar symptoms died of cardiac arrest at the age of 50 years. Clinical examination was suggestive of symmetrical sensorimotor PN. The nerve conduction study was suggestive of axonal sensorimotor PN. Abdominal fat biopsy was negative for amyloid. Sural nerve biopsy was suggestive of amyloid neuropathy. Genetic analysis showed c. 165G > T mutation encoding amino acid p. Lys55Asn on exon-4 of TTR gene. This mutation has not been reported from India.

Résumé La polyneuropathie amyloïde familiale (NP), également connue sous le nom de transthyrétine amyloïde (TTR) -PN, est une maladie mortelle autosomique dominante de l'adulte, si elle n'est pas traitée. Il se produit en raison de mutations du gène (TTR) qui conduisent à une protéine TTR défectueuse qui se replie pour former de l'amyloïde et se dépose principalement sur les nerfs et provoque une PN dépendante de la longueur et un dysfonctionnement autonome. Nous rapportons le cas d'une femme de 45 ans qui présentait des symptômes de neuropathie périphérique douloureuse depuis 5 mois, des antécédents de surdité depuis 5 ans et l'implantation d'un stimulateur cardiaque il y a 2 ans pour un bloc cardiaque complet. Elle a nié tout symptôme de dysfonctionnement autonome. Son frère présentant des symptômes similaires est décédé d'un arrêt cardiaque à l'âge de 50 ans. L'examen clinique évoquait une NP sensorimotrice symétrique. L'étude de la conduction nerveuse était évocatrice d'une NP sensorimotrice axonale. La biopsie de la graisse abdominale était négative pour l'amyloïde. La biopsie du nerf sural était évocatrice d'une neuropathie amyloïde. L'analyse génétique a montré c. Mutation 165G > T codant pour l'acide aminé p. Lys55Asn sur l'exon-4 du gène TTR. Cette mutation n'a pas été signalée en Inde. Mots clés: Neuropathie amyloïde familiale, tests génétiques, biopsie nerveuse, amylose à transthyrétine.

Rhino-Orbito-Cerebral Mycosis and COVID-19: From Bad to Worse?

Background: There has been an increase an alarming rise in invasive mycoses during COVID-19 pandemic, especially during the second wave. Aims: Compare the incidence of invasive mycoses in the last three years and study the risk factors, manifestations and outcomes of mycoses in the COVID era. Methodology: Multicentric study was conducted across 21 centres in a state of western India over 12-months. The clinico-radiological, laboratory and microbiological features, treatment and outcomes of patients were studied. We also analysed yearly incidence of rhino-orbito-cerebral mycosis. Results: There was more than five-times rise in the incidence of invasive mycoses compared to previous two-years. Of the 122 patients analysed, mucor, aspergillus and dual infection were seen in 86.9%, 4.1%, and 7.4% respectively. Fifty-nine percent had simultaneous mycosis and COVID-19 while rest had sequential infection. Common presenting features were headache (91%), facial pain (78.7%), diplopia (66.4%) and vison loss (56.6%). Rhino-orbito-sinusitis was present in 96.7%, meningitis in 6.6%, intracranial mass lesions in 15.6% and strokes in 14.8%. A total of 91.8% patients were diabetic, while 90.2% were treated with steroids during COVID-19 treatment. Mortality was 34.4%. Conclusion: Invasive fungal infections having high mortality and morbidity have increased burden on already overburdened healthcare system. Past illnesses, COVID-19 itself and its treatment and environmental factors seem responsible for the rise of fungal infection. Awareness and preventive strategies are the need of hours and larger studies are needed for better understanding of this deadly disease.

Neurosyphilis, A True Chameleon of Neurology.

BACKGROUND: Neurosyphilis (NS) is a rarely encountered scenario today. Manifestations are heterogeneous, and their characteristics have changed in the antibiotic era. A differential diagnosis of NS is not commonly thought of even with relevant clinical-radiological features, as it mimics many common neurological syndromes. OBJECTIVES: To study the manifestations of NS in the present era and the process of diagnosis. METHOD: The data of ten patients with NS was collected and analyzed. Their background data, clinical features, investigations, the process of reaching the diagnosis, management and outcomes were recorded. OBSERVATIONS AND RESULTS: The manifestations of NS in our cohort included six patients with cognitive decline/encephalopathy and one each with meningitis with cranial nerve palsies, cerebellar ataxia, myelitis and asymptomatic NS. The presence of Argyll Robertson pupil helped to clinch diagnosis in one patient. Treponemal tests were ordered in two patients only after alternative etiologies were looked at, to begin with, whereas in six patients treponemal test was requested as a part of standard workup for dementia/ataxia. CONCLUSIONS: NS dementia and behavior changes are mistaken for degenerative, vascular, nutritional causes, autoimmune encephalitis or prion disease. Meningitis has similarities with infective (tubercular), granulomatous (sarcoidosis, Wegener's), collagen vascular disease and neoplastic meningitis, and myelitis simulates demyelination or nutritional myelopathy (B12 deficiency). Rarely, NS can also present with cerebellar ataxia. Contemplate NS as one of the rare causes for such syndromes, and its early treatment produces good outcomes.

Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome.

BACKGROUND: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. AIMS: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients. METHOD: Retrospective data of MOG-IgG positive patients was collected. Demographics, clinical manifestations at onset and at follow up and relapses, anti AQ-4-IgG status, imaging and all investigations were performed, treatment of relapses and further immunomodulatory therapy were captured. RESULTS: In our 30 patients, F: M ratio was 2.75:1 and adult: child ratio 4:1. Relapses at presentation were optic neuritis {ON}(60%), longitudinally extensive transverse myelitis {LETM}(20%), acute disseminated encephalomyelitis {ADEM}(13.4%), simultaneous ON with myelitis (3.3%) and diencephalic Syndrome (3.3%). Salient MRI features were ADEM-like lesions, middle cerebellar peduncle fluffy infiltrates, thalamic and pontine lesions and longitudinally extensive ON {LEON} as well as non-LEON. Totally, 50% patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, Azathioprine, Mycophenolate and Rituximab were effective attack preventing agents. CONCLUSIONS: MOG-IgG related manifestations in our cohort were monophasic/recurrent/simultaneous ON, myelitis, recurrent ADEM, brainstem encephalitis and diencephalic Syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, LETM, LEON and non-LEON. Where indicated, patients need to go on immunomodulation as it has a relapsing course and can accumulate significant disability. Because of its unique manifestations, it needs to be considered as a distinct entity. To the best of our knowledge, this is the largest series of MOG-IgG disease reported from India.

Atypical Multiple Sclerosis with Antibody to MOG.

CASE PRESENTATION: We report a case of a young female who had magnetic resonance imaging (MRI) brain lesions typical of multiple sclerosis (MS) with cerebrospinal fluid (CSF) oligoclonal bands (OCBs) and definite multiple sclerosis based on revised McDonald criteria; however, she also had atypical features of mild pleocytosis, brainstem and cerebellar peduncle involvement apart from opticospinal (OS) involvement. She also turned out to be positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody; hence, she was diagnosed with atypical multiple sclerosis. This case highlights when to suspect atypical MS and its management approach. DISCUSSION: Typical MS cases are largely anti-MOG-negative. In a study of 50 Japanese cases, with anti-aquaporin 4 (AQP4)-immunoglobulin (IgG)-negative OSMS, just 2 were MOG-IgG-positive, but they had some features atypical for MS, such as bilateral optic neuritis, longitudinally extensive transverse myelitis, or moderate pleocytosis. In another study, antibodies to MOG were found in about 5% (5/104) of preselected adult patients with MS. Patients with MS with antibodies to MOG showed typical MS lesions on brain MRI with concomitant severe brainstem and spinal cord involvement and had a severe disease course with high relapse rates. CONCLUSION: In conclusion, any patient showing typical MS lesions on brain MRI with OCB present in CSF but has atypical features like mild pleocytosis with brainstem, cerebellar, or OS involvement should also be tested for autoantibodies to MOG, and if positive, then he/she would require aggressive treatment approach in the form of plasma exchange, if resistant to pulse steroid therapy, followed by either rituximab or natalizumab rather than trying other disease modifying therapies (DMTs).