BACKGROUNDCranial neuropathies are frequently seen by neurologist. The causes are many and evaluation presents a unique challenge for the diagnostician. The differential diagnosis is broad and includes infections, inflammatory conditions, cavernous sinus lesions, and many life-threatening processes like neoplastic meningitis A knowledge of the anatomy of the cranial nerves and their involvement in pathologic processes is therefore one of the corner stones of neurologic diagnosis. Just as with any other neurologic presentation, the first step in the evaluation requires correct localization. Processes affecting multiple cranial nerves may involve intramedullary structures of the brainstem as well as their extramedullary course. The majority of the literature regarding aetiologies of multiple cranial neuropathies consists of case reports or small case series with very few reported large series. The largest reported series to date of multiple cranial neuropathies was that of Keane.

Visual loss is a common presenting symptom in the Outpatient Departments of Neurology, Ophthalmology and General Medicine disciplines. The present prospective study aims at evaluating 48 patients with partial or total visual loss as the predominant or only presenting symptom and with no associated ocular problems like cataract, glaucoma or diabetic retinopathy. The neurological causes were identified, analysed and tabulated; 39% of patients were below 20 years of age and there was 3:2 ratio of female versus male prevalence. Nearly 66% had acute visual loss and 35% had chronic visual loss. Majority of patients (48%) had profound visual loss limited to finger counting; 6% had normal visual acuity associated with field defects. The visual evoked potentials were abnormal in all cases with acute and sub-acute presentation and in some patients with sudden or chronic presentation also. The VEP abnormalities consisted of prolonged P100 latencies, loss of amplitudes or poor wave morphology. Cranial CT scan was done in 36 patients and was abnormal in 83% patients. It showed intracranial tumours in 8% patients and infarcts in another 8% patients. Cranial MRI scan was done in 12 patients and was normal in 83% patients. The aetiological considerations and treatment protocols followed are discussed. The limitations of such study in the present setup are also highlighted.

The Eye-of-the-Tiger sign is a radiological sign that refers to abnormal low signal intensity in the globus pallidus with a central longitudinal zone of high signal as seen on T2-weighted MRI images. The sign is considered the distinctive radiologic feature of Neurodegeneration with Brain Iron Accumulation (NBIA) type 1, also known as Pantothenate Kinase Associated Neurodegeneration (PKAN). NBIA is a group of inherited neurologic disorders, in which iron accumulates in the basal ganglia resulting in progressive extrapyramidal symptoms. Clinical features and severity may vary with the age of onset and the nature of the underlying mutation. PKAN can present as early onset and late onset forms. We report a few case studies with varying clinical phenotypes and showing imaging features of eye of tiger. Two patients did not develop the characteristic features of PKAN even after five years of follow-up.

BACKGROUNDStroke constitutes a significant health problem in paediatric population. The reported incidence of childhood stroke is 2-6/100000 children per year in the past 10 years. The impact of childhood stroke can easily be realised in terms of economic, social and psychological burden related to disability of affected children and long-term sequelae that include social and economic sequences as well as neurologic and cognitive disabilities and epileptic disorders. The identification of aetiology and risk factors of childhood stroke is important as many of these are age and population specific. Brain infarcts and brain haemorrhages are significantly less common in patients younger than 20 years as compared to adult patients older than 65 years; and their clinical aetiologies also differ markedly from older patients. The risk factors of young stroke include congenital cardiac defects, cerebral vascular defects and various genetic and metabolic disorders. The commonest aetiologies in developed countries encompasses cerebral arteriopathies, congenital or acquired cardiac disease and serious systemic infections. Haematological factors such as thrombophilias also contribute. The commonest CNS infections associated with stroke can be categorised into viral, bacterial, parasitic and fungal in origin. Metabolic factors, AVM and intracranial aneurysm are also potential risk factors. There is no population based published data about the incidence or prevalence of paediatric stroke in India. In the hospital based studies from India, paediatric strokes have constituted less than 1% of all paediatric admissions and 5-10% of all stroke. We attempted to study the clinical profile and risk factors of childhood stroke in South Indian population attending our hospital.