Lamotrigine Induced Severe Cutaneous Reaction.

Antiepileptic drugs are used commonly by physicians, neurologist and neurosurgeons in Nepal. Lamotrigine is an effective antiepileptic drug used as an add-on and monotherapy for a variety of seizure types in both adults and children. Rashes due to AED including SJS and hypersensitivity syndrome leading to hospitalization occur in approximately 0.33% of adults and 0.8% in children. Reactions due to LTG alone occur in around 5.7% of cases. We report probably the first case of this reaction in a 12-year old girl with a brief discussion on its etiology and management.

Cerebellar Mutism After Pineal Tumour Excision: A Case Report.

Cerebellar mutism syndrome (CMS) was first described by Rekate et al in 1985. This syndrome is a common complication of posterior fossa surgery in children with range of 11-29% and usually manifests as diminished speech, hypotonia, and ataxia. The cause is due to bilateral pertubation of the dentate nuclei and their efferent pathways by edema, perfusional defects, axonal damage or metabolic disturbances. Other rare causes of CMS like acute subdural hematoma of the posterior fossa, head injury, brainstem glioma surgery, meningitis and basilar artery occlusion have also been reported. CMS after supracerebellar resection of the pineal tumor is a very rare with very few cases reported. We report such a case in a 10- year old boy who underwent excision of a pineal tumor through the infratentorial supracerebellar route.

The Diencephalic Syndrome of Russell: A Case Report.

Diencephalic Syndrome (DS) is also known as Russells Syndrome. This is associated with marked emaciation, locomotor hyperactivity, vomiting, and absence of obvious neurological signs and loss of subcutaneous fat. A 15-month old child who presented with hyperactivity, loss of weight and failure to thrive since bi rth is reported. On Computed Tomography he had a large supra sellar mass with extension into the third ventricle causing gross hydrocephalus. He underwent biventricular shunting followed by microscopic near total excision of the tumor. The histopathology revealed it to be fibrillary meningioma. Although DS is uncommon it must be kept as a differential diagnosis in all children who fail to grow despite adequate intake.

Parietal Epidural Dermoid Mimicking a Scalp Abscess in an Infant: Case Report.

Scalp masses that involve the scalp or the cranium especially over the parietal bone are uncommon presentation in infants. We report a case of an 8-week old female child who presented with a left parietal scalp mass which had been progressively increasing since birth. CT scan revealed the mass extending into the cranium with underlying bone defect over the parietal area, with medial extension till the sagittal suture. Intraoperatively there was presence of thick white infected flaky material with small tufts of hair which was removed completely till the bone edges and from the epidural space. There was no recurrence postoperatively and the baby was discharged after a week of antibiotics. This case provides an example that simple looking scalp masses in infants and children should be managed with caution to prevent further morbidity.

Frontal recurrence of medulloblastoma five years after excision and craniospinal irradiation.

Medulloblastomas were originally classified under gliomas of the cerebellum until Bailey and Cushing in 1925 named these tumors as medulloblastoma. At present these tumors are classified under primitive neuroectodermal tumor. Surgical excision followed by craniospinal irradiation is the treatment of choice. A 13-year-old-girl operated for posterior fossa medulloblastoma 5 years ago presented with history of headache and vomiting on and off for 4 days in late August 2008. The MRI showed left frontal tumor which on excision was reported as medulloblastoma. Even after optimal treatment reports of recurrence abound in literature. The most common location is in the posterior fossa, followed by spinal, supratentorial, and uncommonly, systemic metastases. We conclude that medulloblastomas are highly aggressive tumor with high local recurrences if the initial excision is incomplete and that recurrence in the supratentorial area although uncommon is still a possibility. This mandates regular follow up of these children till adulthood to catch early recurrences and metastatic disease.

Spontaneous intracystic hemorrhage of the arachnoid cyst: a case report and review of literature.

Arachnoid cysts represent benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane and do not communicate with the ventricular system. We report a case of a years right handed lady, who presented to the emergency department with the complaints of headache and vomiting for one week CT scan showed extraaxial cystic lesion in the left fronto-parietal region. On the fifth day of admission, patient had sudden onset of severe headache associated with loss of consciousness for about 3-4 minutes with neck rigidity. A CT scan of head was repeated, which showed left fronto-parietal cystic lesion with intracystic bleed and SAH. Intraoperatively, there was intradural cystic lesion containing xanthochromic fluid with normal brain surface and there were no evidence of any vascular malformations. Marsupilization of the cystic lesion was carried out and she improved. The literature regarding arachnoid cyst with spontaneous intracranial haemorrhage is reviewed.

Cervical spondylosis in Nepalese porters.

Neck pain with or without radiculopathy and myelopathy is a very common problem in clinical practice. The incidence is believed to be higher in subgroups carrying load on their head. It has been reported in literature that radiographic spondylosis is appreciable in 25% to 50% of population by the age of 50 years and 75% to 85% by the age of 65 years. One hundred and nineteen lateral X-rays of cervical spine were analyzed as case-control study in patients between 40 and 50 years age with the objective of finding out proportion of cervical spondylosis in this age group and difference in the prevalence between porters and non-porters. Out of the 119 cases 54 (45.4.2%) were porters and 65 (54.6%) were non-porters. There were 98 (82.4%) males and 21 (17.6%) females. The overall prevalence of radiological cervical spondylosis was 69 (58%). The prevalence of cervical spondylosis was significantly lower (x2=14.795, p=0.0001) in porters in comparison to non-porters. The odds ratio was found to be 0.23 (0.10, 0.53) at 95% confidence interval indicating that portering significantly prohibited development of spondylosis. This study concludes that the overall prevalence of cervical spondylosis in the Nepalese population is slightly higher than in the Caucasian and contrary to other studies there is significantly lower prevalence of such degeneration among the Nepalese porters.

Traumatic posterior fossa extradural haematoma.

Posterior fossa extradural haematoma is known for the vague signs and symptoms and a notorious course that varies from recovery to sudden death. The incidence of posterior fossa epidural hematomas among intracranial epidural hematomas has been reported from 4% to 7%. Subsequently, PFEDH with low GCS or the haematoma of more than 10 ml were subjected to evacuation. Since the volume of the posterior fossa is limited, patients deteriorate early with the development of obstructive hydrocephalus, which is visible in the CT scan in only thirty percent of cases. A retrospective study of 43 cases was done in this Institute from May 1999 to December 2005. The males (98%) have a clear predominance over female patients (2%). Road traffic accidents accounted for the majority of the cases (80%), fall for the rest (17%) and one case due to a bullhorn injury. Vomiting was the most common symptom accounting for 67% of cases followed by transient loss of consciousness in 48% and headache in 34%. On arrival to the hospital 67% presented with a GCS more than 13, 28% with score of 9-12 and the rest 5% with GCS of less than 8. Out of the total 43 cases of PFEDH surgical evacuation was done in 33(76%) and conservative management in 10 cases (23%). A dichotomised Glasgow outcome score was used to measure the outcome. This was favorable in 27 of the 33 cases operated (81%), and 7 out of the 10 conservatively managed group (70%). Overall favorable outcome was found in 34 cases (79%) with overall mortality of the study being 7%.