Isolated Acute Cerebrovascular Involvement in COVID-19 without Fever and Respiratory Symptoms: An Indian Perspective.

BACKGROUND: Stroke in COVID-19 has been reported in critically ill patients globally. Stroke as a singular manifestation of COVID-19 in absence of typical symptoms (fever, cough and dyspnea) is under- recognized. OBJECTIVE: Comparative study of clinical and laboratory parameters of COVID-19 stroke patients without typical symptoms at onset with stroke cases without COVID-19 infection. METHODS: 28consecutive stroke patients, eight with coronavirus infection and twenty without COVID-19 admitted to neurology department of a tertiary care centre of North West India between 20 June,2020 and 19 July,2020 were enrolled in this retrospective study. RESULTS: COVID-19 patients had higher frequency of seizures (4[50%]) vs 2[10%];p= 0.03)and altered mental status(6[75%] vs 6[30%] p= 0.04). Severity of ischemic stroke(NIHSS >20, 3[75 %] vs 2[18%])and mortality(p=0.04)despite comparable vascular risk factors for stroke between the two groups was higher in COVID-19 patients. Three out of four COVID-19 young strokes died. Two females with COVID-19 did not develop any typical symptoms, six males(75%) developed fever with dyspnea after a mean delay of 2.7 days(Standard deviation 1.7) from stroke onset. All six patients who developed fever subsequently expired. Inflammatory markers (neutrophil to lymphocyte ratio;p<0.001and ESR: p<0.001), transaminases(p=0.038) and creatinine (p=0.009) were significantly elevated in COVID-19 patients. CONCLUSION: Isolated cerebrovascular involvement can be a presentation of COVID-19.Stroke severity and mortality is higher in COVID-19 with young strokes being no exemption. Development of fever was associated with clinical worsening. COVID-19 pandemic is far from over in India, such atypical presentations need to be recognized early and warrant stringent diagnostic protocols.

CT Angiographic Evaluation of Pattern and Distribution of Stenosis and its Association with Risk Factors Among Indian Ischemic Stroke Patients.

BACKGROUND: Stroke is the major cause of disability and third major cause of death. Ischemia causes about 80% of stroke cases; stenosis and occlusion are the predominant cause of stroke. Our purpose for this study was to evaluate the pattern and distribution of stenosis and its association with risk factors in Indian ischemic stroke patients and in other countries, for comparison. MATERIAL/METHODS: We evaluated 60 patients after ischemic stroke with CT angiography. The degree of stenosis was measured by the North American Symptomatic Carotid Endarterectomy Trial (NASCET). The results were analyzed by descriptive statistics and chi-square test. RESULTS: Out of 60 cases, 32 cases were positive for significant stenosis and a total number of stenotic segments was 45. Out of 45 stenotic segments, 29 were single stenotic segments (16 intracranial and 13 extracranial) and 16 were multiple stenotic segments (8 intracranial and 8 extracranial). In the total number of stenotic segments (single and multiple), there were 24 (53.33%) intracranial and 21 (46.67%) extracranial. The most commonly involved intracranial stenosis segment was MCA, present in 10 (41.6%) out of 24 intracranial segments. Most commonly involved extracranial stenosis segment was ICA, present in 14 (66.6%) out of 21 extracranial segments. Diabetes is found to be the most common risk factor of intracranial stenosis while hypertension and hypercholesterolemia are the major risk factors of extracranial stenosis. CONCLUSIONS: In the Indian population, intracranial stenosis is more common than extracranial one, anterior circulation stenosis is more common than posterior circulation stenosis; single stenosis is more common than multiple stenosis.

Herpes zoster meningoencephalitis complicated with peripheral vascular disease: an uncommon presentation of a common disease.

Herpes zoster is reactivation of the varicella zoster virus that has remained dormant in the dorsal root ganglia since an earlier episode of chickenpox. Herpes zoster has variable clinical presentations, but meningo-encephalitis is not frequently encountered. There is growing evidence of both large and small vessel involvement in immunocompetent and immunocompromised patients, in contrast with the previous opinion that immunocompetent patients have vasculopathy in the large vessels while immunosuppressed patients have vasculopathy in the small vessels. We present the case of a patient in whom herpes zoster meningoencephalitis was complicated with multifocal vasculopathy with peripheral vascular disease; this is an unusual co-occurrence.

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.

Spectrum of Posterior Cerebral Artery Dissection: A Retrospective Observational Study and a Critical Review.

Background and Aims: Intracranial arterial dissections commonly involve the vertebrobasilar system leading to subarachnoid hemorrhage (SAH) or cerebral infarction attributable to a dissecting aneurysm of the vessel or occlusion of the lumen depending on the depth of dissection. However, isolated posterior cerebral artery dissections (PCADs) are rare and sparsely reported in the literature. Methodology: A retrospective multicentric observational study was carried out after collecting data from 14 patients admitted with PCAD in three hospitals of Kolkata, Jaipur, and Patna within the period of July 2021 to June 2022. Results: The median age of the population was 48.5 years, and 64.28% were females. SAH was the most common presentation with dissecting aneurysms in all patients barring one, who presented with a left occipital infarct consequent to ipsilateral PCAD. Among the 14 patients, three patients denied endovascular intervention and were lost to follow-up; one patient with an occipital infarct and another patient with a dissecting left P3 aneurysm, which underwent spontaneous thrombosis, were managed conservatively. Among the nine patients scheduled for endovascular coiling, one patient succumbed before intervention and one patient succumbed to sepsis in the postoperative period. A complete recovery was noted in six patients, whereas residual neurodeficits were present in three patients. Among the six patients who had an uneventful recovery at the end of 3 months, five patients had an endovascular intervention. Conclusion: PCAD may present with large-scale neurodeficits and is associated with high morbidity and mortality, hence necessitating prompt management. Conservative management is preferable for consequent infarcts, whereas endovascular management is desirable in cases of dissecting aneurysms, which usually tend to have a favorable outcome if intervened early.

A rare case of cerebral proliferative angiopathy with acute cerebral infarction and facial capillary malformation.

We are describing a case of a 14-year-old girl who developed acute cerebral infarction which was documented on diffusion-weighted magnetic resonance imaging (DWI). On detailed evaluation, diagnosis of cerebral proliferative angiopathy (CPA) was made. Incidentally, she had capillary malformation on the forehead contralateral to cerebral vascular malformation. To our knowledge, this is the first case of CPA in the literature where DWI abnormality was due to acute cerebral infarction.

Clinico-radiological features of cerebral proliferative angiopathy: A large case series from India.

BACKGROUND: Based on angiographic features, a rare subgroup, different from classical brain AVMs, called cerebral proliferative angiopathy (CPA) was suggested by Lasjaunias et al. in 1989. There are only few reports of CPA described from Asia. Liu et al. has described 5 cases of CPA from China. The objective of study was to evaluate the clinico-radiological features of patients with CPA in our cohort and to find out the differences between our study and the study by Lasjaunias and Liu et al. METHODS: We retrospectively analysed cases of CPA from March 2012 to April 2020. All radiological images of the patients with AVMs were studied and findings suggestive of CPA were identified. We found 10 patients with CPA out of 272 cases of AVM (10/272) and described the various demographic and clinico-radiological features. RESULTS: The age of the patients with CPA in our series varied from 14 to 53 years and the mean age was 28.6 years. There was male preponderance (60%) with male to female ratio of 3:2. The most common presentation was seizure (70%). Intracranial haemorrhage was present in five patients. One patient had cerebral infarction. CONCLUSIONS: There were substantial differences in clinico-radiological profile of patients with CPA in our cohort as compared to the study by Lasjaunias et al. and Liu et al. in form of male dominance, higher rate of ICH and seizure. In our knowledge, this is the second largest series of CPA worldwide and the first largest series in Asia.

Carotid cavernous fistula: Redefining the angioarchitecture.

OBJECTIVE: Numerous classification schemes have been used for carotid cavernous fistula (CCF), each describing some aspect of the disease process but none of them provides a complete description of the fistula including its clinical features, natural history, arterial and venous architecture. METHODS: Retrospective clinical and radiological review was done for all the patients diagnosed with CCF and treated at our institute. The CCF were classified according to the proposed API-ACE classification along with Barrow and Thomas classification. RESULTS: Overall 28 patients (M=21, F=7) were diagnosed and treated during the 6-year period. 89.2% of CCF developed following an episode of head injury. Orbital symptoms were the most common presenting complaints. Barrows type A was the most predominant subtype (n=24) and most of the patients (n=23) demonstrated decreased ipsilateral carotid filling. Combined anterior and posterior drainage pattern was the most common drainage pattern and anterior drainage was more commonly observed than posterior drainage. CONCLUSIONS: API-ACE classification helps to better understand and classify the angioarchitecture of CCF which could help better understand the clinical manifestations and guide in appropriate endovascular approach selection for treatment.

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.

Endovascular management of intracranial pseudoaneurysm: an institutional experience.

OBJECTIVE: Pseudoaneurysms (PSAs) of the internal carotid artery (ICA) and vertebral artery are rare entities but with varied treatment options. PSAs can be spontaneous or secondary to trauma, infections, malignancies or iatrogenic. To find out the efficacy of various endovascular interventions in the management of ICA and vertebral PSAs. METHODS: The study included 14 patients diagnosed with intracranial PSAs who underwent endovascular interventions in SMS Medical College, Jaipur (Rajasthan) between June 2015 to January 2019. The clinical and radiological findings (computed tomography angiography and digital subtraction angiography) were reviewed and the consequent endovascular intervention carried out and their results were analyzed. RESULTS: Total 14 patients were studied out of which 8 (57.1%) were anterior circulation PSAs and 6 (42.9%) were posterior circulation PSAs There were 10 (71.4%) females and 4 (28.5%) males between the age of 9 to 65 years. Only 2 patients with PSA had past history of trauma. Coiling was done in 8 patients (57.1%), stenting in 2 patients (14.2%), parent artery occlusion in 1 patient (7.1%), glue embolization in 1 patient (7.1%) while coiling with glue in 1 patient (7.1%) and flow diverter in 2 patients (14.2%). Immediate and complete occlusion was achieved in 11 (78.6%) patients while 3 (21.4%) patients had subtotal occlusion. 11 patients under follow up till June 2019 did not report recurrence or new neurological deficit. CONCLUSIONS: Endovascular interventions is minimally invasive and safe treatment strategy for intracranial PSAs. The ultimate choice of technique depends on clinical and imaging characteristics.

Atypical Kleine--Levin syndrome: can insomnia and anorexia be features too?

BACKGROUND: Kleine-Levin syndrome is an uncommon disorder with recurrent episodes of hypersomnia, clearly associated with behavioral abnormalities like binge eating, hypersexuality and abnormal behavior. Many patients may not necessarily fulfill minimum criteria described for diagnosis. We aim to report such patients with atypical presentation resembling the Kleine-Levin syndrome. METHOD: We evaluated all patients at our clinic who had episodic disturbance in sleep and/or appetite lasting a few days to weeks, not necessarily fulfilling the International Classification of Sleep Disorders (ICSD) criteria for a diagnosis of Kleine-Levin syndrome, over 4 years. All clinical details, especially regarding sleep, appetite and behaviour during episodes, about prior and co-existing illnesses were noted. All patients were investigated with brain magnetic resonance imaging (MRI), electroencephalogram (EEG) and some with polysomnography. RESULTS: Eighteen patients (5 females, 13 males) ranging in age from 12 to 55 years (median 18 years) were included in the study. The median duration of symptoms was 1.5 years, and the median number of episodes in each patient was six. The range of episode length was 18-300 h with a mean of 91.2h. Fourteen patients had a history of hypersomnia, 3 had only insomnia and 3 had both during their episodes, while 5 patients reported hyperphagia, 11 reduced appetite and 2 no change in appetite. Ictal EEG revealed evidence of sleep, while polysomnography showed reduced rapid eye movement (REM) latency and normal sleep architecture during the episode. MRI was normal in all patients, except one who showed non-specific abnormalities. All patients showed improvement with carbamazepine. CONCLUSION: There are many patients with episodic alteration in sleep, appetite and behaviour with a course and treatment response similar to the classical Kleine-Levin syndrome, who otherwise do not fit the classical description for diagnosis of this condition.

Treatment of traumatic trigeminal-cavernous fistula by coil embolization and compression of carotid artery.

We report a case of a traumatic cavernous fistula supplied by a persistent primitive trigeminal artery. The process of treatment was unique in this case. Fistula was subcompletely occluded by coiling from primitive trigeminal artery. Residual fistula was helped to form thrombosis by compression of the carotid artery with hand in the procedure. Long-term follow-up was satisfactory. Traumatic cavernous fistula supplied by a persistent primitive trigeminal artery could be treated by embolization and temporal compression of the parent artery might be useful for residual minimal fistula.

A rare case of dural arteriovenous fistula presenting as primary intraventricular hemorrhage.

Primary intraventricular haemorrhage (PIVH) is rare. Dural arteriovenous fistula causing PIVH is extremely rare. We report a case of a 17 year old boy who presented with left hemiparesis, left lower motor neuron facial palsy and ataxia. His computed tomography head revealed primary intraventricular hemorrhage. Catheter super selective angiography revealed a dural arterio venous fistula with arterial feeder arising from the middle meningeal artery as well as from the inferior marginal tentorial artery. Glue injection led to successful disappearance of the fistula and eventual clinical recovery.

Lower limb onset Parry-Romberg syndrome: an unusual presentation of a rare disease.

Parry-Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and muscle wasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophy of right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. He was managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up.