Skull-base temporal encephalocele: Hidden cause of temporal lobe epilepsy.

In the present study patients with previously diagnosed MRI-negative temporal lobe epilepsy (TLE) on long-term video electroencephalography (VEEG) monitoring were re-evaluated with high resolution 3T MRI brain to look out for a skull base temporal lobe encephalocoele (TE). A total of 234 VEEGs were analyzed. TLE had been diagnosed in 104 patients based on semiology, ictal, interictal EEG data, and brain positron emission tomography (PET) studies. Of these, 99 patients had temporal lobe abnormality (78 had mesial temporal sclerosis, 8 had tumor, 3 had focal cortical dysplasia, and 10 had mixed pathology). Out of the five 1.5T MRI-negative TLE patients, two patients were diagnosed with TE on subsequent 3T MRI brain scans and one patient underwent electrocorticography-guided tailored resection for complete removal of epileptogenic tissue; with Engels class I seizure freedom at one year follow-up. We propose that TE should be carefully searched for, as a cause of refractory TLE, using high-resolution MRI sequences.

Massive benign osteoblastoma of the suboccipital bone and foramen magnum region.

Benign osteoblastoma is an uncommon primary bone tumor frequently found in the vertebral column and long tubular bones, and rarely occurring in the calvarium. A case of a massive benign osteoblastoma of the suboccipital bone and foramen magnum region in a 9-year-old boy is reported. He presented with progressively worsening nuchal pain and headaches secondary to a bony lesion in the suboccipital and foramen magnum region. Computed tomography (CT) of the brain showed a large midline occipital/suboccipital bony lesion extending to either side (R > L) and extending from the torcula till the foramen magnum region, causing moderate obstructive hydrocephalus. The atlas was uninvolved by the tumor. In addition, the cerebellum was pushed anteriorly squashing the fourth ventricle. The tumor was completely resected with wide margins via a suboccipital route. At follow-up after 7 years, the patient was asymptomatic, and CT imaging demonstrated no recurrence. The occurrence of benign osteoblastoma in the suboccipital bone and foramen magnum region has not been reported earlier in the pediatric population. Surgical extirpation of the lesion with wide margins is advocated and can produce an excellent long-term outcome. Serial vigilant follow-up along with sequential imaging is advocated even in cases with complete resection to detect early recurrence and possible malignant transformation.

Tuberculous temporal brain abscess mimicking otogenic pyogenic abscess.

Tuberculous brain abscess is a rare manifestation of central nervous system tuberculosis. We report the case of a tuberculous temporal lobe abscess in a 14-year-old female child that mimicked an otogenic pyogenic brain abscess. The patient had no prior history of tuberculosis. She had chronic otitis media and presented with signs of raised intracranial tension. Radiological imaging was suggestive of an acute pyogenic left temporal lobe abscess. A left temporal craniotomy was performed and the abscess was completely excised. Histological examination was consistent with a chronic abscess, and bacterial cultures were negative. A left radical mastoidectomy was also carried out. However, she presented with repeated abscess formation at the same site over the next 8 weeks, which was refractory to surgical therapy and broad-spectrum antibiotic administration. Furthermore, the purulent exudate showed strong positivity in the PCR test for tubercular bacilli. After administration of antituberculous treatment, she showed gradual clinical and radiological improvement. At follow-up after 2 years, she is asymptomatic. CT imaging at 2 years showed total resolution of abscess. Tuberculous abscess in the temporal lobe following otogenic infection has not been reported in the pediatric population. Although rare, the possibility of tuberculous etiology should be borne in mind as a differential diagnosis of acute abscess of otogenic origin, especially in endemic areas where the incidence of chronic otitis media as well as tuberculosis is high. The pathogenesis and treatment of tuberculous brain abscess in children is reviewed in light of the current literature on the subject.

Supratentorial convexity dural-based cavernous hemangioma mimicking a meningioma in a child.

A case of a convexity dural-based cavernous hemangioma in a 15-year-old male child is reported. The child presented only with headaches and had no neurological deficits. MRI features were suggestive of a meningioma. The tumor was completely resected and histology was conclusive of a hemangioma. At follow-up after 2 years, the child is asymptomatic and MRI showed no evidence of the lesion. Supratentorial convexity dural-based hemangioma in a child is rare and hitherto only 1 case has been reported in the world literature. Complete resection of the lesion is curative and long-term prognosis is excellent. The uniqueness of the present case is the supratentorial convexity dural-based location, innocuous presentation and unusual radiological features on MRI. The clinical profile, radiological features and management of cavernous hemangiomas in a child are discussed and the current literature is briefly reviewed.

Retroclival arachnoid cyst with hemifacial spasm.

Arachnoid cysts are rare lesions occurring anywhere in the cerebrospinal axis. The sylvian fissure remains the most favoured site for their occurrence, followed by cerebellopontine angle, suprasellar, and quadrigeminal cisterns. Retroclival arachnoid cysts are very rare. We report a retroclival arachnoid cyst with bilateral cerebellopontine angle extensions with hemifacial spasm in a 26-year-old woman. The patient underwent surgery and her hemifacial spasm improved.

Intramedullary cervical neurenteric cyst mimicking an abscess.

We describe a cervical intramedullary neurenteric cyst in a 12-year-old male patient who presented with gradual onset and progressively worsening neck pain, spastic quadriparesis and impaired sensation in the C(2) dermatome. MR imaging revealed a well-defined peripherally enhancing cystic intramedullary lesion with a posteroinferior enhancing nodule at the C(2)-C(3) level mimicking an abscess. There was no evidence of spinal dysraphism. The lesion was completely resected through a posterior approach and the patient showed radical improvement in his symptomatology. At follow-up after 3 years, he was asymptomatic and the MR imaging showed no evidence of any residual or recurrent cyst. The case presented here is unique, since a spinal neurenteric cyst showing intense peripheral contrast enhancement mimicking an abscess is unusual. The radiological features, pathogenesis and surgical considerations in cervical intramedullary neurenteric cysts are discussed and the relevant literature is briefly reviewed.

Fourth ventricle arachnoid cyst.

We report four patients with fourth ventricle arachnoid cysts. There were two males and two females and the ages ranged from 18 months to 52 years (average 21 years). All patients presented with symptoms of raised intracranial pressure. Excision or marsupialization of the arachnoid cyst in this location was curative in all patients. The follow-up ranged from 1 to 14 years.

Posterior fossa dermoid cyst in a patient with Goldenhar syndrome.

A 7-year-old boy with Goldenhar syndrome was diagnosed to have a posterior cranial fossa dermoid cyst. The presence of such a combination of clinical entities has not been reported earlier. The embryonic dysgenesis causing midline posterior fossa dermoid and other anomalies observed in Goldenhar syndrome occur between the third and fifth week of intrauterine life and are probably interrelated. This report emphasizes the need for a high index of suspicion and the value of cranial imaging in a case with Goldenhar syndrome.

Glioblastoma multiforme following cranial irradiation and chemotherapy for acute lymphocytic leukaemia. Report of 3 cases.

The most common secondary neoplasms which occur following cranial radiation therapy are sarcoma and meningioma. The occurrence of glioblastoma multiforme following radiation and chemotherapy in acute lymphocytic leukaemia (ALL) is rare. We report 3 cases of glioblastoma multiforme in children developing 11-72 months following completion of chemotherapy/radiotherapy for ALL. The exact cause for the development of glioblastoma multiforme following therapy for ALL is not clear. A genetic predisposition may be essential for the occurrence of such a highly malignant primary brain tumour in leukaemia patients, irrespective of radiation and/or chemotherapy. The pathogenesis and surgical management are discussed, and the literature on the subject is reviewed.

Syringomyelia in infants secondary to mobile congenital atlantoaxial dislocation.

We report a characteristic pattern of 'waist-like' syringomyelia observed in three infants having mobile and reducible congenital atlantoaxial dislocation. The syringomyelia in at least two of these cases was diagnosed to be an intramedullary tumor. Following atlantoaxial fixation, all three patients had long-term recovery of their symptoms.

An atypical presentation of recurrent temporal lobe meningioma with external auditory canal mass.

Extracranial spread of recurrent meningiomas involving the middle ear is rare. We present the case of a 59-year-old woman with headache and swelling of scalp over the right temporal region. MRI revealed a lesion in the right temporal lobe suggestive of meningioma. She underwent complete surgical excision of the lesion followed by post-operative radiotherapy. After 1 year, she presented with right-sided otalgia and a middle-ear mass extruding into the external auditory canal. She was re-operated and histopathology was anaplastic meningioma. We are discussing this unusual pattern of recurrence in our patient with a review of literature.

Multiple intracranial aneurysms in a child with congenital cyanotic heart disease.

A 10-year-old child with transposition of the great arteries had multiple intracranial aneurysms. The child presented with pyogenic meningitis, and the investigations incidentally revealed numerous aneurysms involving both anterior and posterior circulation arteries. Angiography also revealed multiple fistulae between the internal and external carotid arteries. The child is under observation. The pathogenesis and management strategy of multiple intracranial aneurysms are discussed and the relevant literature is briefly reviewed.