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.

Mitochondrial Leukodystrophy: an Unusual Manifestation of Leigh's Disease. A Report of Three Cases and Review of the Literature.

Leigh's disease is an inherited, progressive neurodegenerative disorder of infancy and early childhood. This metabolic disease is biochemically and genetically a heterogeneous disorder with defects involving various enzymes involved in the respiratory chain mechanism. Due to the multitude of enzyme defects known to occur in patients afflicted with Leigh's disease, this condition is known to have a variable clinical, pathological and radiological pattern of presentation. Isolated deficiency of cytochrome oxidase (COX) enzyme is one of the commonest abnormalities seen in patients afflicted by Leigh's disease. A primary white matter pattern of involvement representing a mitochondrial leukodystrophy is rare. Symmetric hyperintensities on T2WI involving the subthalamic nuclei and brainstem have been reported in patients with COX deficiency with SURF 1 mutations and are considered almost a hallmark of Leigh's disease with COX deficiency. We describe three cases of Leigh's disease with a primary white matter involvement diagnosed at our institution on the basis of clinical features, radiological appearance and laboratory findings.

Magnetic resonance imaging of Asians with multiple sclerosis was similar to that of the West.

Magnetic resonance imaging (MRI) of the brain is the most important paraclinical diagnostic test in multiple sclerosis (MS). The appearance of MRI in Asians with MS is not well defined. We retrospectively surveyed the first brain and spinal cord MRI in patients diagnosed to have MS, according to Poser's criteria in seven regions throughout Asia to define the MRI changes among Asians with MS. There were 101 patients with first brain, and 86 with first spinal cord MRI, 66 of whom had both. The brain MRI showed a mean of 17 lesions per patient in T2 weighted images, mostly asymptomatic. Almost all the lesions were in the white matter, particularly in the juxtacortical, deep and periventricular white matter. A third of the lesions were greater than 5 mm, 14% enhanced with gadolinium. There were more supratentorial than infratentorial lesions at a ratio of 7.5: 1. Ninety five percent of the spinal cord lesions were in cervical and thoracic regions, 34% enhanced with gadolinium. The lesions extended over a mean of 3.6 +/- 3.3 vertebral bodies in length. Fifty (50%) of the brain and 54 (63%) of the spinal MRI patients had the optic-spinal form of MS. The MRI of the optic-spinal and classical groups of patients were similar in appearance and distribution, except that the optic-spinal MS patients have fewer brain but longer and more severe spinal cord lesions. In conclusion, the brain and spinal cord MRI of Asian patients with MS was similar to that of the West, although, in this study, Asian MS patients had larger spinal cord lesions.

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.

Isolated cysticercal infestation of extraocular muscles: CT and MR findings.

PURPOSE: We sought to document the appearance of isolated cysticercal infestation of single extraocular muscles on MR and CT studies, and to compare these findings with results of histopathologic examination. METHODS: Six MR and three CT examinations of the orbits of six patients were reviewed. Histopathologic confirmation of the diagnosis was available in three patients, and response to specific medical therapy was available in one. In all, the imaging findings were considered highly suggestive of cysticercal infestation. RESULTS: Typically, the affected extraocular muscle showed fusiform enlargement of its belly and contained a well-defined, spherical cyst with a nodule attached to its wall. The mural nodule was identified in all six cases with varying degrees of visibility. It was best seen on the CT examinations and in all cases in which contrast material had been administered. The nodule and the enlarged muscle showed intense enhancement on the contrast-enhanced studies. Imaging studies of the brain showed no evidence of cerebral cysticerci in any of the patients. CONCLUSION: The MR and CT appearance of isolated infestation of single extraocular muscles by the larva of the pork tapeworm Taenia solium is quite characteristic and often diagnostic of this condition.