Neonatal hypoglycemic brain - injury a common cause of infantile onset remote symptomatic epilepsy.

OBJECTIVES: To study the etiology of remote symptomatic epilepsy with onset in the first 3 years of life. Patients with neonatal hypoglycemic brain injury (NHBI), were further studied for risk factors and clinical features. METHODS: The study was conducted at a tertiary pediatric neurology service between May-August 2004. Consecutive patients were recruited prospectively. The probable etiological diagnoses were based primarily on cranial imaging. Two radiologists, blinded to the etiological diagnosis, reviewed the cranial imaging and suggested the likely etiology based on published imaging criteria. There were three categories i.e, (i) perinatal encephaloclastic conditions (PEC) e.g., hypoxic ischemic encephalopathy (HIE) etc, (ii) developmental (DV) e.g., tuberous sclerosis, etc and (iii) postnatal (PN) e.g., trauma, etc. Three risk factors (birth weight, type of delivery, feeding difficulty) were compared between NHBI and developmental etiology (DV) groups. Neurological findings were compared between the NHBI vs the other perinatal groups. Seizure details were studied only in the NHBI group. RESULTS: 63 boys and 37 girls were recruited. Mean age of seizure onset was 13.9 months. PEC were seen in 50 patients, DV in 28 patients and PN in 5. NHBI was seen in 23 patients and was the most frequent cause of epilepsy. Low birth weight (LBW), neonatal feeding difficulties and cesarean delivery were significant risk factors for NHBI vis a vis the DV group. Microcephaly, autism, visual impairment and apraxia of hand use were common while spasticity or dystronia were rare in NHBI. Spasms were the commonest seizure type. CONCLUSION: Neonatal hypoglycemia is the most common etiology of remote symptomatic infantile onset epilepsy. LBW, poor neonatal feeding and cesarean delivery are significant clinical correlates.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL): a case report with review of literature.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is an inherited arterial disease, commonly overlooked or misdiagnosed. We report a case of CADASIL in a 51 years old woman who presented with progressive subcortical dementia, recurrent ischemic events and seizures in the absence of known vascular risk factors of five years' duration. Her mother had a history of similar illness. Magnetic resonance imaging (MRI) of brain revealed subcortical and deep white matter hyperintense lesions within the cerebral white matter on T2-weighted images. DNA mutation of Notch 3 gene confirmed the diagnosis of CADASIL.

Idiopathic hypertrophic cervical pachymeningitis: a case report with 5 year follow up.

Idiopathic hypertrophic pachymeningitis is an extremely rare entity. It usually affects cranial meninges. The spinal form is further uncommon and presents as a chronic progressive disease. We describe a 42 year old female with isolated idiopathic hypertrophic cervical pachymeningitis who had a relapsing remitting course under observation for five years. Laminectomy and immunosuppressive therapy produced temporary and partial relief. The long term course and relevant literature is reviewed.

Intramedullary neurenteric cyst in mid thoracic spine in an adult: a case report.

Neurenteric cysts are very rare, particularly in adults. These are congenital intraspinal cysts of endodermal origin. A 67 years old man, presenting with backache and paraesthesiae of one and half years' duration, followed by subacute flaccid paraplegia, developing in a week is described. MRI revealed intramedullary cyst at T7. He underwent emergency thoracic laminectomy and complete excision of the cyst. Histopathology confirmed a neurenteric cyst. In view of their rarity, peculiarity in terms of age, location and presentation, we report this case.