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.

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.

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.

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.

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.

Endolymphatic sac carcinoma of the right petrous bone in Von Hippel-Lindau disease.

A large endolymphatic sac papillary adenocarcinoma in a patient with Von Hippel-Lindau (vHL) disease is reported. A 31-year-old man was treated for a left eye retinal angioma 10 years previously and had been blind in that eye since. He was admitted with progressive tinnitus, lower cranial nerve paresis and ataxia. Investigations revealed a vascular and bone-eroding petrous tumour. The entirely extradural tumour involved a large part of the petrous bone and extended into the cerebellopontine angle. The vascular tumour was radically resected using a presigmoid approach after feeding vessel embolisation. The literature on this rare clinical entity is briefly discussed.

Trigeminal neurinoma associated with Chiari malformation and syringomyelia.

We report a 36 year-old woman who presented with headaches and hypoesthesia of the face. MRI revealed a large dumbbell shaped trigeminal neurinoma extending into both the middle and the posterior cranial fossae. In addition, there was a Chiari I malformation and syringomyelia. Within two months of tumor resection, both the Chiari malformation and the syringomyelia resolved. The association of an intracranial space-occupying lesion with Chiari malformation and syringomyelia is reviewed.

Petroclival mixed meningioma.

We present a 47-year-old lady with a large dumb-bell petroclival-cavernous sinus meningioma. The tumour was of mixed histopathology; the anterior part, in relationship to the cavernous sinus, was a syncitial meningioma and the posterior part in the petroclival region was a transitional meningioma. The two histological subtypes of meningioma within the same tumour displayed remarkable differences in their radiological features, nature of extensions, relationship with the adjoining structures and consistency. The clinical course of the two subtypes was also different. The unusual features of this case are reported and the relevant literature briefly reviewed.

Giant pituitary adenoma invading the clivus.

A 26-year-old man was admitted with complaints of worsening of vision for one month. Investigations revealed a large sellar and parasellar tumor mass invading into both the cavernous sinuses. The tumor invaded the clivus and both petrous apices. It was radically but partially resected through a transsphenoidal approach. Following surgery, the patient showed symptomatic improvement. The residual tumor was treated by radiation treatment. The case is discussed and the literature on the subject is briefly reviewed.

Surgery on anterior foramen magnum meningiomas using a conventional posterior suboccipital approach: a report on an experience with 17 cases.

OBJECTIVE: The advantages of a posterior "conventional" suboccipital approach with a midline incision over lateral, anterolateral, and anterior approaches to anteriorly placed foramen magnum meningiomas are discussed. METHODS: From 1991 to March 2000, 17 patients with foramen magnum meningiomas arising from the anterior or anterolateral rim of the foramen magnum underwent operations in the Department of Neurosurgery at King Edward Memorial Hospital and Seth G.S. Medical College. All patients were operated on in a semi-sitting position by use of a conventional suboccipital approach with a midline incision and extension of the craniectomy laterally toward the side of the tumor up to the occipital condyle. RESULTS: The patients ranged in age from 17 to 72 years, and the tumors ranged in size from 2.1 to 3.8 cm. The intradural vertebral artery was at least partially encased on one side in eight patients and on both sides in two patients. The brainstem was displaced predominantly posteriorly in each patient. A partial condylar resection was performed in two cases to enhance the exposure. Total tumor resection was achieved in 14 patients, and a subtotal resection of the tumor was performed in the other 3 patients. In one patient, a small part of the tumor was missed inadvertently, and in the other two patients, part of the tumor in relation to the vertebral artery and posterior inferior cerebellar artery was deliberately left behind. After surgery, one patient developed exaggerated lower cranial nerve weakness. There was no significant postoperative complication in the remainder of the patients, and their conditions improved after surgery. The average length of follow-up is 43 months, and there has been no recurrence of the tumor or growth of the residual tumor. CONCLUSION: From our experience, we conclude that a large majority of anterior foramen magnum meningiomas can be excised with a lateral suboccipital approach and meticulous microsurgical techniques.

Primary Ewing's sarcoma of the cranium.

OBJECTIVE: We analyzed the data for a series of 14 patients with primary Ewing's sarcomas of the cranium who were treated since 1985. Our aim was to assess the long-term outcomes and the selection of appropriate treatment methods. METHODS: The patients were reviewed with respect to their clinical presentations, treatment, and outcomes. Computed tomographic scanning of the brain was performed for all patients. Skeletal surveys with routine radiographs and technetium-99 bone scans to detect extracranial Ewing's sarcomas were performed for all patients. For all 14 patients, radical tumor excision was achieved surgically. All patients were then subjected to adjuvant multidrug chemotherapy and radiotherapy. The follow-up periods ranged from 8 months to 8 years (mean, 4.25 yr). RESULTS: The predominant presenting features were headaches, increased intracranial pressure, and scalp swelling. Excision was nearly total for nine patients and total for five patients. All patients experienced uneventful postoperative courses. One patient experienced a local recurrence, which was detected 2 years after surgery. This recurrent tumor was completely excised, and additional chemotherapy was administered. Eight of the 14 patients (57.1%) studied have survived 5 years or longer. CONCLUSION: Although primary Ewing's sarcoma of the cranium is a malignant bone tumor, it is associated with a good prognosis when treated with radical surgery, aggressive multidrug chemotherapy, and radiotherapy.

Bilateral vocal cord paralysis after anterior cervical discoidectomy and fusion in a case of whiplash cervical spine injury: a case report.

BACKGROUND: Bilateral vocal cord paralysis is a risk of anterior cervical discoidectomy and fusion. We discuss the mechanism of vocal cord paralysis and the precautions necessary to avoid this catastrophic complication. A rare case of bilateral vocal cord paralysis after anterior cervical discoidectomy and fusion (ACD/F) is reported. CASE DESCRIPTION: The patient, a 37-year-old male, was paraplegic, had bilateral intrinsic hand muscle weakness and sphincter involvement following a whiplash cervical spinal injury. A C5-C6 ACD/F for traumatic C5-C6 disc prolapse was performed. On the third postoperative day, he developed difficulty in coughing and a husky voice. Otolaryngological evaluation revealed bilateral vocal cord paralysis. He later required a tracheostomy that partially alleviated his major symptoms. CONCLUSION: In patients undergoing ACD/F, a mandatory preoperative evaluation of the vocal cords should be performed. An appropriate modification in surgical planning should be made if vocal cord palsy is diagnosed preoperatively to prevent bilateral vocal cord paralysis. Proper and judicious use of Cloward retractors is advocated.

An unusual post-traumatic occipitocervical pseudomeningocele: case report.

BACKGROUND: A rare case of massive post-traumatic subcutaneous pseudomeningocele probably communicating with the cisterna magna, is reported. CASE DESCRIPTION: An 8-year-old boy sustained a severe injury to the nape of the neck, after which he developed a huge local subcutaneous swelling containing cerebrospinal fluid (CSF). Communication of the subcutaneous CSF collection with the cisterna magna or any other site of dural fistula could not be identified. After a lumbo-peritoneal CSF shunt, the swelling resolved completely. CONCLUSION: The clinical features of a rare case of symptomatic post-traumatic cerebrospinal fluid pseudomeningocele are elaborated.