Characterization of Ocular Sonography Findings and their Relationship to Clinical Features from a South Indian Cohort of Idiopathic Intracranial Hypertension.

Background: Idiopathic intracranial hypertension (IIH) typically manifests with headache, accompanied by papilledema and visual loss, and has a higher prevalence in females. In recent years, ocular sonography, particularly, measurement of optic nerve sheath diameter (ONSD), is being investigated for diagnosis of IIH. Methods: A total of 35 patients over the age of 18 years, fulfilling the modified Dandy's criteria for diagnosis of IIH were included. Patients underwent assessment with magnetic resonance imaging, lumbar puncture, and ocular sonography to measure ONSD and ocular arterial indices. Results: The mean ONSD values (in centimeters) in the right eye of patients with IIH was 0.57 ± 0.13, while it was 0.48 ± 0.03 in controls. In the left eye, the mean ONSD value (cm) was 0.59 ± 0.13 in patients with IIH and 0.48 ± 0.03 in controls. ONSD was significantly higher in cases compared to controls (P < 0.001, Welch test). Pulsatility index of the central retinal artery was significantly higher in cases compared to controls (P < 0.001, Welch test). Resistance index of the ophthalmic artery was statistically significant (P < 0.005, Welch test). Receiver operating characteristic curve analysis revealed a cutoff value of 5.1 mm on the right side and 5 mm on the left side had a sensitivity and specificity of more than 80% for IIH diagnosis. Conclusion: Our study provides insights into the utility of optic nerve sheath measurements and arterial indices in the diagnosis of IIH in a South Indian cohort. Further research is needed to fully understand the longitudinal relationship of these parameters and treatment outcomes in IIH.

Profile and Outcome of Prospective Non-Septic Lateral Sinus Thrombosis Patients from a Stroke Unit of a Developing Country.

Background: Due to effective treatment of middle ear infections there is a change in etiologies causing lateral sinus thrombosis (LST) and outcome. There is a paucity of literature describing homogenous group of patients with nonseptic LST (NS-LST). Objective: To describe the clinical profile, risk factors, outcome of patients of NS-LST seen at a single center from South India. Methods and Materials: Prospective, observational study of 100 patients of NS-LST patients, diagnosed by magnetic resonance imaging (MRI) seen at the stroke unit. Results: During 2 years, 100 patients of NS-LST (isolated: combined: 27:73) (male: female: 44:56), mean age: 31.45 ± 11.13 years, were seen. Subacute presentation (74%) with headache, seizures, focal deficits, and features of raised intracranial pressure were presenting features. Hyperhomocysteinemia (61%), anemia (57%), postpartum state (41%), OCP use (37%), and low VitB12 (32%) were commonly seen risk factors. Imaging with MRI compared withcomputerized tomography (CT) had better diagnostic sensitivity (100% vs. 67%), detection of parenchymal (81% vs. 67%)/hemorrhagic (79% vs. 74%) lesions, and cortical vein thrombosis (31% vs. 15.46%). Treatment with anticoagulation and supportive therapy resulted in good outcome (mRS (0-2)) at 3 months in 81%.There were four deaths, all during admission (one - isolated, three - combined) and 11 patients underwent decompressive surgery. Patients with low GCS level of sensoriumat admission, hemiparesis, combined LST, cerebellar involvement, and decompressive craniectomy had a poor outcome. Conclusion: This single-center large cohort study of NS-LST patients brings out the clinical features, risk factors (peculiar to developing countries), and the superiority of MRI in the diagnosis. Majority of patients have good outcome, with low mortality with 10% requiring decompressive surgery.

Treatment-related Fluctuations in Guillain Barre Syndrome and the Conundrum of Additional Cycles of Plasmapheresis.

INTRODUCTION: In Guillain Barre syndrome (GBS), worsening of weakness or disability after initial period of recovery or stabilization is described as treatment-related fluctuations (TRF). AIM: This study aims to describe the clinical characteristics and outcome of six patients with GBS and TRF. PATIENTS AND METHODS: Six patients with GBS fulfilling NINCDS criteria, evaluated at a tertiary care university hospital during 2008-2017, were diagnosed to have TRF. They form the basis of this report. RESULTS: All patients were men and their mean age was 40 years. At presentation, mean duration of illness was 15 days; the illness had plateaued in three and progressive in other three patients. Two of the four patients had variant GBS. Initially, five patients were treated with large volume plasmapheresis (LVPP) and one patient with methyl prednisolone. At 17-28 days after disease onset, three patients developed new neurologic deficits (bilateral facial paresis in two; paralytic ileus in one). Other three patients with worsening of limb weakness (medical research council sum score of >5) and disability (Hughes disability grade by ≥1) fulfilled Kleyweg's criteria for TRF. All the six patients were treated with the completion of five cycles or additional cycles of LVPP. CONCLUSION: Awareness about TRF is essential for correct diagnosis and management of patients with GBS.

Unilateral Papilledema in Cerebral Venous Sinus Thrombosis.

In the majority of patients with raised intracranial pressure, the papilledema is bilateral. Unilateral papilledema is rare in conditions causing intracranial hypertension, and it has been described in Foster-Kennedy syndrome and in some cases of idiopathic intracranial hypertension. It has never been reported in cerebral venous thrombosis. We report a young lady presenting with features of subacute onset of headache with seizures, on evaluation she had superior sagittal and bilateral lateral sinus thrombosis. The risk factors found on evaluation were Vitamin B12 deficiency and hyperhomocysteinemia. On optic fundus examination, she had swollen optic disc on the right side with normal fundus on the left side, confirmed with the orbital ultrasound B-scan and optic coherence tomography. Her magnetic resonance imaging showed features of raised intracranial pressure with thrombosis of the superior sagittal and bilateral lateral sinus thrombosis. She was treated with anticoagulation (heparin followed by oral anticoagulants), antiedema measures, and vitamin supplementation for hyperhomocysteinemia. She improved over time and was asymptomatic during follow-up. We discuss the possible mechanisms described in the literature for unilateral papilledema. This report highlights the need for carefully performing bilateral fundus examination so as not to miss the vision or life-threatening causes of a headache.

Preliminary data on utility of subcutaneous unfractionated heparin in patients with deep cerebral venous thrombosis.

Subcutaneous unfractionated heparin (SCUFH) has been proved effective in puerperal cerebral venous thrombosis (CVT), but its efficacy in the more serious form of the disease such as deep CVT patients (DCVT) unreported. We describe the outcomes of 37 (isolated:combined: 11:26) patients of DCVT diagnosed by MRI, treated with SCUFH in a tertiary care stroke unit. It was a prospective observational cohort study using 5000 U of SCUFH every 6 hourly for 10 days with oral Acenocoumarol started on day 7, with monitoring. The outcome was assessed by modified Rankin scale (mRS), National Institute of Health Stroke Scale (NIHSS) and Barthel's activities of daily life (BADL) at 3 months. The mean age of the cohort was 27.9 ± 9.7 years, females (n = 24) outnumbering the males (n = 13). Mean duration of symptoms being 10.2 ± 15.9 days. MRI showed vein of Galen and straight sinus involvement in 36 (97.3%) patients, with sparing of the basal vein of Rosenthal in 28 (75%). Thalamus 27 (73%) basal ganglia 21 (56.7%) were commonly involved areas with hemorrhagic lesions in 18 (48.6%) patients. The median NIHSS score at presentation was 11 (1-21). Mean duration of SCUFH treatment was 9.3 ± 1.3 days and the mean aPTT on day 7 was 49.3 ± 9.8 s (control 32-39 s), mean PT INR on day 13 was 1.5 ± 0.45. All the patients improved with no mortality in the study group. At 3 months, good functional outcome (mRS: 0-2) was observed in 94.6% (n = 35) of patients. Two patients had mRS-3. The median mRS (3{1-5} to 0{0-3}) and BADL (8{0-20} to 20{8-20}) improved at 3 months. Complications seen were thrombocytopenia-1, infection-6 and deep vein thrombosis of leg-4. Our preliminary data suggests that SCUFH is safe, effective treatment option in patients with DCVT in a stroke unit with minimal monitoring.

Episodic neurological dysfunction in hereditary peripheral neuropathy.

Episodic transient neurological symptoms are an important set of problems presenting to a neurologist in his routine practice. Occasionally, detailed clinical history including past and family history supplemented with focused examination can bring out a rare cause for such symptoms. We describe in this report in a young male presenting with episodic focal neurological dysfunction, with family history of similar episodes in mother and brother. Examination showed features of pes cavus and peripheral neuropathy for which patient was asymptomatic. Mother and brother were established cases of hereditary neuropathy. Imaging on multiple occasions showed reversible white matter abnormalities. Clinical suspicion of X-linked Charcot-Marie-Tooth disease type 1 (CMT1X) was confirmed with detection of mutation in Gap Junction B1 (GJB1) gene, which codes for connexin 32 protein (c.425G>A; p.R142Q hemizygous mutation). Though this mutation has been already reported in CMTX patients, it has not been associated with transient neurological dysfunctions. This is probably the first reported case of CMTX patient with transient neurological dysfunction from India, whose family members had similar episodes.

Intravenous thrombolysis in a patient with left atrial myxoma with acute ischemic stroke.

Intravenous thrombolysis (IVT) is an accepted therapy in patients with acute ischemic stroke presenting within 3-4.5 hours of symptom onset. Selection of the patient for thrombolysis depends on the careful assessment for the risk of post thrombolysis symptomatic haemorrhage (6.2-8.9%) which may be fatal. Atrial myxomas which are the commonest tumors of the heart are associated with stroke due to tumor/clot embolism. There are very few case reports of IVT and its outcome in patients with atrial myxoma with stroke. Some have reported successful thrombolysis, while others have reported intracerebral bleeding. In this report we describe our experience of IVT in atrial myxoma patient with ischemic stroke and review the relevant literature.

Profile of patients with craniovertebral junction anomalies with posterior circulation strokes.

BACKGROUND: Craniovertebral junction (CVJ) anomalies are rare and treatable risk factors for posterior circulation strokes (PCSs). Most of the literature comprises case reports and from varied specialities. METHODS: Chart review of patients with PCS with CVJ anomalies admitted in a single tertiary stroke care unit. We describe their clinical profile, investigative findings, and therapeutic outcomes. RESULTS: We saw 7 patients (6 males, mean age 20.1 ± 12.0 years), all previously undiagnosed during the 6-year period. Three patients had a combination of atlantoaxial dislocation (AAD), basilar invagination (BI) and Klippel-Feil anomalies, 2 patients had os odontoideum with AAD, and 1 patient each had isolated AAD and BI. Clinically, they presented with first or recurrent episodes of stroke with neck pain. Examination revealed noticeable skeletal markers and neurologic deficits involving the arterial territory. Routine stroke risk factors were absent. X-ray of CVJ was abnormal and diagnostic in all the patients. Other imaging modalities had their own contributions for identifying associated defects, demonstrating pathophysiology, and management of these patients. Acute care with anticoagulation and supportive measures helped in recovery of all of them. Posterior fusion was done in 4 patients, and 2 patients are awaiting the surgery. During follow-up all patients have improved and none had recurrences. CONCLUSIONS: In case of a young patient with PCS (first/recurrent) in the absence of routine risk factors for stroke, careful physical examination and imaging with basic X-ray may help in the diagnosis CVJ abnormalities, which may be effectively managed by a multidisciplinary team to prevent recurrences.

Unilateral herpes zoster ophthalmicus with bilateral cerebral infarcts in human immunodeficiency virus seropositive patient.

Ischemic stroke is a recognized complication of herpes zoster ophthalmicus. Arterial involvement is usually seen on the side of the rash. It is thought to be due to vessel inflammation by the virus, which travels from the trigeminal ganglion. Few case reports of bilateral and distant site of zoster lesions with stroke in the brain have been described. These reports suggest possibility of contiguous vascular, cerebrospinal fluid (CSF) or hematogenous spread of the virus from the ganglion to the cerebral blood vessels. Therapeutically acyclovir, anticoagulation, and steroids have been used in the treatment of the zoster associated with stroke. We describe a case of immunocompromised patient with ipsilateral zoster ophthalmicus with bilateral anterior circulation strokes, who was treated with above measures and made successful recovery. This report also raises/supports possible CSF/vascular/hematogenous spread of the virus from the ganglion to involve cerebral blood vessels leading to the stroke.

Profile of patients with cerebral venous sinus thrombosis with cerebellar involvement.

BACKGROUND: Cerebral venous thrombosis (CVT) is a rare form stroke with diverse manifestations. There is very little information available regarding profile of patients with cerebellar involvement in CVT. We describe the clinical profile, investigations, and outcome of patients with cerebellar involvement with CVT. METHODS: We prospectively studied 6 (male:female: 5:1, mean age 29.3 ± 10.3 years) patients of CVT with cerebellar involvement (2 isolated cerebellar and 4 associated deep or supratentorial structures) among 330 CVT patients (1.8%) admitted in our stroke unit during a period of 3 years. RESULTS: The presenting features (mean duration 7.0 ± 4.7 days) were headache, vomiting, encephalopathy, ataxia, and papilledema. Initial diagnosis considered were neuroinfection in 2 patients, raised intracranial pressure in 2, subarachnoid hemorrhage in 1, and subacute ataxia in 1 patient. Computed tomography (CT) was diagnostic in 3 of 5 patients, whereas magnetic resonance imaging (MRI) and magnetic resonance venography were diagnostic in all; in addition, MRI was superior in detecting new lesions not visualized on CT, in better delineating hemorrhages and in predicting the age of thrombus. Patients were managed medically with anticoagulation, anti-edema, and anti-epileptics and supportive treatment. Two patients underwent posterior fossa decompression. Four patients made complete recovery at the time of discharge and 2 patients died (1 because of brain stem dysfunction and another because of postoperative pneumonia and septicemia). CONCLUSIONS: Cerebellar involvement in CVT is very rare, and high index of suspicion is required for diagnosis. Imaging with CT and MRI will help in confirming the diagnosis. Conventional anticoagulation is effective in treatment. Mortality was 33%, and there was no disability in survivors.