Clinical Characteristics, Etiology, Recanalization Rates and Neurological Outcomes in CVT: A Prospective Cohort Study.

Background: Recanalization rates in cerebral venous thrombosis (CVT) and its effect on neurological outcome have been debated worldwide and are inadequately addressed in studies from India. Our objective was to study the clinical profile of CVT and determine recanalization rates with its predictors and its effect on outcome. Methods: A prospective single centre cohort study on 101 patients with radiologically confirmed acute CVT between October 2018 and June 2021 was conducted. Anticoagulation was given for 3-12 months or lifelong for thrombophilias. Recanalization status of vessels was assessed between 3-6 months and at 12 months after ictus. Outcome was defined as favorable (mRS 0-1) or unfavorable. Patients with atleast one CT/MR venogram on follow up were included. Results: Of the 101 enrolled patients, 83 completed study protocol. Mean age of patients was 34.2 ± 11.7 years. Clinical characteristics included headache (75.9%),seizure (66.2%), altered mentation(20.4%) with clustering of cases during summers. Transverse- sigmoid sinuses were predominantly involved (66.2 %) followed by superior sagittal sinus (SSS,65.0%).Commonest etiologies were thrombophilia (27.7%) and postpartum state (15.6%). Complete recanalization was achieved in 67.4%, partial in 26.5% and no recanalization in 6.02% at end of 12 months. Recanalization rates improved from 83.09% between 3-6 months to 93.9 % at 12 months. Median time to last follow-up was 12months and at last follow up 95.1% had favorable mRS with recurrence in two patients with raised factor VIII levels. Conclusion: Recanalization occurred in more than 90% of CVT patients. Isolated superior sagittal sinus thrombosis and age <50 years were predictors of complete recanalization. Most patients, except few achieved a favorable mRS.

Comparison of Clinical and Radiological Features of Aquaporin4 (AQP-4) Antibody Positive Neuromyelitis Optica Spectrum Disorder (NMOSD) and Anti Myelin Oligodendrocyte Glycoprotein (Anti-MOG) Syndrome-Our Experience from Northwest India.

Background: More and more cases of myelin oligodendrocyte glycoprotein (MOG) antibody are being diagnosed with the availability of laboratory tests helping us to know the differing patterns from AQP-4 antibody disease and we need to understand the natural course, treatment, and prognosis in a better way. Objectives: Neuromyelitis optica spectrum disorder (NMOSD) and anti-MOG syndromes are immune-mediated inflammatory demyelinating conditions of the central nervous system (CNS) that mainly involve the optic nerves and the spinal cord. We conducted this study to compare demographic, clinical, laboratory, and radiological features of AQP-4 antibody and MOG antibody positive patients. Methods: A single-centre retrospective observational study from a large tertiary care university centre of Northwest India conducted during 2019--2021. We screened all patients presenting with acute CNS demyelinating attacks and recruited total 47 patients of which 25 were positive for AQP4 antibody and 22 were positive for MOG antibody. No patient tested positive for both antibodies. Data were collected using a standardized format including demographic, clinical, laboratory, and neuroimaging data. Results: In our study, total 47 patients were included, amongst which 25 patients were AQP4 antibody and 22 patients were MOG antibody positive. Though there was no gender preponderance, pediatric patients were more frequently affected in MOG antibody positive group. In AQP-4 antibody positive patients, myelitis was most common presenting clinical feature followed by optic neuritis (ON), simultaneous ON with myelitis, and brainstem syndrome. In MOG antibody positive group, myelitis was the commonest phenotype followed by ON, brainstem syndrome, and cerebral syndrome. The neuroimaging revealed involvement of medulla mainly area postrema, cervicodorsal spinal cord and extension of cervical lesion up to brainstem more commonly in AQP4 antibody group, on the other hand involvement of upper brainstem (midbrain and pons), cortex, and conus was more common in MOG antibody group. Conclusion: We have made an attempt to find differentiating features in AQP-4 vs. MOG antibody positive cases but they were of no statistically significance value as the numbers were small. Further larger studies may prove helpful in planning better strategies in two groups.

Longitudinally extensive transverse myelitis: A retrospective analysis of sixty-four patients at tertiary care center of North-West India.

OBJECTIVES: To evaluate the demographic profile, clinical presentations, laboratory parameters and etiologies of longitudinally extensive transverse myelitis (LETM) patients in Indian population. PATIENTS AND METHODS: LETM is characterized by contiguous inflammatory lesions of spinal cord extending to three or more vertebral segments. Neuromyelitis optica (NMO) is the most common cause of LETM. In clinical practice, both LETM and NMO are thought to be synonymous with each other because of their very frequent association. Other causes of LETM are infective, neoplastic, autoimmune diseases and connective tissue disorders. All other causes should be ruled out before making the diagnosis of NMO in LETM patients. We conducted a retrospective study from August 2010 to February 2016 and analyzed various demographic profile, clinical presentations, laboratory parameters and etiologies in sixty-four patients of LETM. RESULTS: In our series, majority of the patients presented with acute bladder dysfunction and paraparesis. Twenty-one patients (32.81%) were clinically diagnosed as NMO, out of which thirteen patients were found to have positive serum NMO antibody. Other etiologies of LETM in our series were multiple sclerosis [9 patients], acute disseminated encephalomyelitis (ADEM) [6 patients], postinfectious [5 patient], subacute combined degeneration (SCD) [4 patient], tuberculous myelitis [4 patients], spinal arteriovenous malformation (AVM) [3 patient] and systemic lupus erythematosus (SLE) [3 patient], respectively. In nine patients, cause could not be ascribed despite thorough investigations. CONCLUSION: LETM is a heterogeneous disorder with a varied clinical feature, etiologies and outcome. Even the LETM patients who presented with optic neuritis do not necessarily have NMO. Therefore, the diagnosis of NMO should be made only after excluding other causes of LETM. The presence of NMO-Ab (Aquaporin 4-Ab) predicts the increased risk of recurrence of LETM or conversion to NMO.

Medullary Hot-Cross Bun Sign in Multiple System Atrophy-Cerebellar.

Multiple-system atrophy (MSA) is a progressive neurodegenerative disorder. It is characterized by dysfunction of multiple systems including autonomic, extrapyramidal, pyramidal and cerebellar. Pontine hot-cross bun (HCB) sign in MSA is a well-documented and highly specific entity. To the best of our knowledge, medullary hot-cross bun (HCB) sign has never been described in literature. Herein, we report a case of MSA-C with medullary HCB sign. Detection of medullary HCB sign might be a surrogate marker in the diagnosis of MSA-C, particularly in the absence of classical pontine HCB sign; however, further research will help to validate this sign.

A rare association of tuberculous longitudinally extensive transverse myelitis (LETM) with brain tuberculoma.

BACKGROUND: Longitudinally extensive transverse myelitis is characterized by contiguous inflammatory lesion of spinal cord involving three or more spinal segments. It is a well-recognized but rare presentation of Mycobacterium tuberculosis infection. CASE DESCRIPTION: We report a case of young boy diagnosed with multiple brain tuberculomas. He was on antitubercular drugs therapy for 2 months and became asymptomatic. On 2-month followup visit, the patient complained of acute onset progressive sensorimotor, spastic paraparesis with bladder dysfunction. Magnetic resonance imaging of spine showed longitudinally extensive transverse myelitis extending from thoracic spinal segment T2 to T10 level. He was treated with high dose intravenous methylprednisolone therapy and continued on combination of first line four antitubercular drugs. At 6-month followup, patient was able to walk with support. In our patient, clinical features, previous history of brain tuberculoma and spinal neuroimaging confirmed the diagnosis of tuberculous myelitis. The new onset longitudinally extensive transverse myelitis in our patient was may be related to paradoxical response to antitubercular therapy. CONCLUSIONS: Our case highlights that tubercular infection might be an important but overlooked cause of longitudinally extensive transverse myelitis. Therefore, clinicians should have a high index of suspicion to diagnose this potentially treatable cause especially in high-risk conditions like tuberculosis endemic areas, associated brain tuberculosis and HIV infection. Our case is unique because of paradoxical presentation of longitudinally extensive transverse myelitis in cranial tuberculomas, already on antitubercular treatment.

Cerebral proliferative angiopathy with papilledema.

A young female presented with intermittent blurring of vision and mild to moderate headache for three months. Fundus examination revealed bilateral papilledema with secondary optic atrophy (right more than left). Computed tomography scan of brain showed a diffuse intraparenchymal vascular malformation in right parietooccipital region. Cerebral digital subtraction angiography (DSA) revealed diffuse nidus in right parietooccipital area, supplied by multiple branches of distal middle cerebral artery and anterior cerebral artery. Scattered-puddling appearance of contrast was noted in diffuse nidus and there were no definite arterial feeders. DSA was consistent with the diagnosis of cerebral proliferative angiopathy (CPA). The patient was started on acetazolamide 250 mg twice a day. At six months follow up, she was asymptomatic with resolving papilledema. CPA is a rare vascular malformation, any case report that gives further insight to it will be very useful to the scientific community. Herein, we describe an unusual association of CPA with papilledema.

Familial segmental spinal myoclonus: a rare clinical feature of Friedreich's ataxia.

INTRODUCTION: Friedreich's ataxia (FRDA) is the most common autosomal recessive inherited ataxia. It is characterized by onset before the age of 25 year, progressive limb and truncal ataxia, lower limb areflexia, extensor plantars, dysarthria and impaired posterior column sensations. Other important associated features are skeletal deformity, hypertrophic cardiomyopathy and diabetes mellitus. Most of the patients (98%) have an unstable homozygous trinucleotide (GAA) expansion in intron-1 of chromosome 9 and 2% patients are compound heterozygous for GAA expansion and point mutations. CASE DESCRIPTION: We observed an adolescence onset FRDA exhibiting spinal segmental myoclonus (SSM) in a family. Triplet repeat primed polymerase chain reaction (TP-PCR) demonstrated unstable expansion of >66 GAA repeats. CONCLUSIONS: SSM is a unique and rare manifestation of FRDA. This might be the first case report of SSM in FRDA patient.

Longitudinally extensive transverse myelitis as presenting manifestation of small cell carcinoma lung.

Longitudinally extensive transverse myelitis (LETM) is an unusual manifestation of systemic malignancy. It has been mainly reported with lung cancers and lymphoproliferative malignancy. LETM in systemic malignancy can be caused by either intramedullary metastases or paraneoplastic syndrome. We report an unusual case of small-cell carcinoma lung, who presented with LETM without having any cardinal manifestations of lung malignancy. This case report highlights the important differentiating features between intramedullary metastasis and paraneoplastic syndrome.

Reversible magnetic resonance imaging changes in a case of neuroleptic malignant syndrome.

Neuroleptic malignant syndrome (NMS) is a life-threatening neurologic emergency associated with the use of mainly typical antipsychotic drugs. It is characterized by fever, altered mental status, generalized rigidity, autonomic instability, myoclonus, raised creatine phosphokinase, rhabdomyolysis, and leukocytosis. Neuroimaging (brain computed tomography/magnetic resonance imaging [MRI]) is usually normal in most of the cases of NMS. Magnetic resonance imaging findings have not been well elucidated in NMS as yet. Very few cases have been reported worldwide. We herein, report a case of a 42-year-old patient of NMS, who presented to us with reversible changes in MRI brain. This case report highlights the possible MRI changes in NMS and their plausible mechanism.

An institutional experience of 26 patients with Moyamoya disease: A study from Northwest India.

AIM: Moyamoya disease (MMD) is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD. MATERIALS AND METHODS: We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA) was done in 25 (96.15%) patients where as Digital substraction angiography (DSA) was done in 18 (69.23%) patients. RESULTS: Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH), 50% patients had ICH with intraventricular extension (IVE) and 30% patients had primary intraventricular hemorrhage (PIVH). 50 % of the patients had involvement of the posterior circulation. CONCLUSION: Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.

Primary intraventricular hemorrhage: clinical features, risk factors, etiology, and yield of diagnostic cerebral angiography.

BACKGROUND: Primary intraventricular hemorrhage (PIVH) is a rare neurological disorder, with bleeding confined to the ventricles only, without recognizable parenchymal or subarachnoid component. AIM: The purpose of this retrospective study was to identify clinical features, predisposing risk factors, etiology, radiological features and yield of diagnostic cerebral angiography in identifying the etiological causes. SETTINGS AND DESIGN: Records of patients admitted in neurology division were analyzed in a tertiary care teaching hospital. MATERIALS AND METHODS: We analyzed the records of 27 patients with PIVH evaluated and treated at our institute from August 2010 to April 2013. PIVH was diagnosed as hemorrhage in the ventricles only, detected by computed tomography scan without evidence of intraparenchymal, subarachnoid hemorrhage or intraventricular hemorrhage associated with trauma. CT angiography (CTA) alone was done in 10 patients (37.03%), digital subtraction angiography (DSA) in 2 patients (7.4%) and both CTA as well as DSA was done in 15 patients (55.5%). STATISTICAL ANALYSIS USED: Categorical and continuous data were analyzed using SPSS version 17. RESULTS: 17 (62.96%) patients were females and 10 (37.03%) were males with ratio of F:M= 1.7:1. Headache was the commonest mode of presentation (85.18%). Hypertension was most common predisposing factor (29.62%) followed by arterio-venous malformations (AVMs) (25.92%), moyamoya disease (MMD) (11.11%), lenticuostriate artery aneurysm (LSA) (11.11%), arterial dissections (7.4%) and dural arteriovenous fistula (dAVF) (3.7%). CONCLUSIONS: PIVH is rare and hypertension is important predisposing factor. Yield of cerebral angiography is high in diagnosing the etiology. AVMs and other rare etiological causes like MMD, LSA aneurysm, arterial dissection, and dAVF should be kept in mind with a high index of suspicion and warrants cerebral angiography in them, as some of the causes are potentially treatable.

Lenticulostriate artery aneurysm presenting as primary intraventricular haemorrhage.

Isolated or primary intraventricular haemorrhage (PIVH) is rare and usually caused by hypertension, vascular malformations, aneurysms and moyamoya disease. Lenticulostriate artery aneurysm (LSA) is also a rare entity, and LSA aneurysm rupture causing primary intraventricular haemorrhage is extremely rare. There are only a few case reports. LSA aneurysms can occur medially or laterally, proximally or distally, though distal lateral LSA aneurysms are more common. They are usually associated with various vascular conditions. We report two rare cases of LSA aneurysms presenting as PIVH, one being medial and other being lateral distal LSA aneurysm. Both were normotensives, and one was associated with arteriovenous malformation.

Rapid thrombosis of middle cerebral artery aneurysm after subarachnoid haemmorhage.

Spontaneous thrombosis of intracranial aneurysm is a rare event but is frequent after subarachnoid haemorrhage (SAH) and in fusiform or giant saccular aneurysms. We report a case of a 20-year-old man presenting with SAH due to rupture of a giant aneurysm of the middle cerebral artery. Initial CT angiography (CTA) revealed partially thrombosed MCA aneurysm but digital subtraction angiography performed 3 days later revealed complete occlusion of the aneurysm. Rapid thrombosis of aneurysm within 3 days has not been reported in literature so far.