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.

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.

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.

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.

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.

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.

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.

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.

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.

Kleine-Levin syndrome with comorbid iron deficiency anemia.

Kleine-Levin syndrome (KLS) is a rare chronic sleep disorder of unknown etiopathology, which typically occurs in adolescent males. Although the severity of symptoms and disease course varies between the KLS patients, it usually resolves spontaneously, but sometime comorbid conditions may worsen the symptoms. Herein, we report a case of KLS who presented with severe episodic hypersomnia. During episodes, the patient used to sleep as long as 20 h in a day, affecting his daily living activities. All the relevant investigations including electroencephalography, magnetic resonance imaging of brain and cerebrospinal fluid analysis were normal except for severe iron deficiency anemia (IDA). In our patient, the severity of symptoms worsened due to coexistent IDA. The treatment of IDA along with modafinil decreased the severity of symptoms and shortened the hospital stay during episodes. This might be the first case report of KLS with comorbid IDA.

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.

Infratentorial hemorrhagic cerebral proliferative angiopathy: A rare presentation of a rare disease.

Cerebral proliferative angiopathy (CPA) is a unique and rare vascular malformation with distinct clinico-radiological features. CPA is associated with low risk of hemorrhage as compared to classical cerebral arteriovenous malformations (AVMs). Infratentorial location of diffuse nidus is also an uncommon presentation. Infratentorial hemorrhagic presentation of CPA is a rare co-occurrence. Herein, we report a case of an elderly old male, who presented with acute onset severe headache, recurrent vomiting, vertigo and swaying toward left side while walking. Cerebellar signs in the form of scanning speech, nystagmus, impaired finger-to-nose incoordination test, dysdiadochokinesia, and limb ataxia on the left side were present. Magnetic resonance imaging (MRI) brain revealed diffuse vascular network with intermingled normal brain parenchyma and hemorrhage in the left cerebellum. Digital subtraction angiography (DSA) revealed diffuse, ill-defined, nidus in left posterior fossa involving the left posterior inferior cerebellar, anterior inferior cerebellar and posterior cerebral arterial territories. There were no definite arterial feeders. DSA showed scattered "puddling" appearance of contrast material in the widespread nidus and drained into the multiple ill-defined posterior fossa veins. The MRI brain and DSA findings were consistent with the diagnosis of the CPA. Treatment in our case was limited to supportive medical therapy because selective embolization was not feasible due to nonidentifiable arterial feeders. He was asymptomatic at 1-year follow-up. This case highlights a rare entity called CPAs, which have different clinical presentations, angiographic features, treatment options and patient outcome as compared to classical cerebral AVMs.