Primary intracranial rhabdomyosarcoma (PIRMS) is a rare neoplasm, which affects infants and young children. We report a rare case of a primary embryonal rhabdomyosarcoma of the cerebellopontine angle in a 7-year-old boy with clinical and radiological features mimicking a vestibular schwannoma. The patient underwent definitive surgery and radiotherapy and is recurrence free at 6 months. PIRMS commonly occur in parameningeal locations or metastasize to the brain from an extracranial primary. Gross total tumor resection followed by adjuvant radiotherapy remains the current standard of treatment; however, chemotherapy has also been tried with favorable results. The overall prognosis continues to remain poor.
The authors present a case of a 15-year-old male patient who presented with gradually progressive quadriparesis for 3 years. Magnetic resonance imaging of the spine was suggestive of heterogeneously enhancing mass lesion extending from cervicomedullary junction to conus. This holocord spinal tumor was excised in a single stage with standard microsurgical technique. In immediate postoperative period, the patient had deterioration in power in both lower limbs which improved in follow-up at 6 months. Histopathology of the tumor was suggestive of ependymoma. Holocord ependymoma is a rare entity; until now, only six cases have been described in the literature. To the author's best knowledge, this is only the second case of holocord ependymoma excised in a single stage.
Remote cerebellar hemorrhage (RCH) is an extremely rare and potentially devastating complication of supratentorial and spinal surgeries. While there are numerous postulates explaining the patho-physiology behind this phenomenon, including the most popular CSF over drainage theory, the exact cause for the same is still largely unknown. In this report, we present 2 cases of remote cerebellar hemorrhage encountered following 2 different surgical procedures. One patient had preceding pterional craniotomy for ruptured anterior communicating artery aneurysm while the other one developed RCH after placement of EVD. Both of them had history of poorly controlled hypertension, contrary to most reports where hypertension has not been found to be commonly associated with it. Moreover, while most cases have been reported to occur following supratentorial craniotomies and spinal surgeries, one of our patients developed the same after placement of the EVD, which, to the best of our knowledge, has not been reported earlier.
Objective: The aim was to investigate the incidence and pattern of neuroendocrine changes in cases of acute aneurysmal subarachnoid hemorrhage (SAH). Materials and Methods: Endocrine assessment was performed in 100 consecutive cases of acute aneurysmal SAH presenting within 7 days of ictus. The gonadotropic, somatotrophic, thyrotropic, and corticotrophic axes were evaluated for their possible dysfunction. Results: A total of 100 cases (38 males, 62 females; age range - 17-76 years; mean age - 43.6 years) of acute SAH were studied. The aneurysms were located in the anterior circulation (n = 95) and posterior circulation (n = 5). The most common hormone deficiency was of growth hormone (n = 67), followed by gonadotrophin (n = 50), corticotrophin (n = 49) and thyrotrophin (n = 35). Hyperprolactinemia was noted in 10 cases. One-pituitary hormone axis deficiency was noted in 26 cases while 67 cases had two or more pituitary hormone axes dysfunction. A total of 93 cases had hormonal dysfunction in one or more pituitary hormone axes, and seven cases had no hormonal dysfunction. Conclusions: Endocrine dysfunction occurs in 93% cases of acute SAH and multiple pituitary hormone axes dysfunction occurs in 67% cases. It is suggested that hormonal evaluation should be considered as part of management of acute SAH.
Endometriosis involving the neural axis is extremely rare. A twenty-five-year-old woman presented with acute onset paraparesis with bladder involvement of five-day duration. We missed her history of cyclical back-ache related to menses at the emergency room. Magnetic resonance imaging (MRI) of spinal cord showed an intramedullary tumour with bleeding at conus-epiconus level. She was operated in the emergency department with laminectomy-durotomy and tumour decompression. Histopathology of the tumour was suggested endometriosis.
Dermato fibrosarcoma protuberance (DFSP) of scalp is a relatively uncommon soft tissue neoplasm constituting less than one percent of all DFSP [4]. Locally aggressive nature of lesion, potential intracranial spread and lack of adequate scalp tissue for reconstruction, makes this lesion unique and its proper management challenging. All these features mandate that the management of DFSP scalp,especially extensive and recurrent lesions should be a team approach with close co-operation between the neuro-surgeon and the reconstructive surgeon.
A 54-year-old male presented with the history of headache and vomiting. MRI of the head showed right posterior temporal mass which was surgically excised. Histopathological examination revealed features of glioblastoma with pigmented cells. The pigment was demonstrated to be melanin which was confirmed by special stains and immunohistochemistry. This is the first description of glioblastoma with melanotic differentiation reported in the literature. The relevant literature is briefly reviewed.
Agenesis of internal carotid artery (ICA) is a rare anomaly. The contralateral dominant artery has a high incidence of aneurysms. A 50-year-old male patient had aneurysmal subarachnoid hemorrhage due to rupture of a paraclinoid aneurysm. He also had an anterior communicating artery (Acom) aneurysm and the contralateral ICA was absent. To the best of the authors knowledge, the combination of agenesis of unilateral internal carotid artery with the unique association of a paraclinoid and an Acom aneurysm has not been previously reported. A meticulous assessment of the collateral blood circulation prior to surgery is mandatory in these cases for avoiding postoperative ischemic injury.
In vestibular schwannomas (VS), the tumour size, as well as the size of the cystic component, have a considerable bearing on the outcome. This study addresses the differences between the cystic and solid variants of giant vestibular schwannomas. The study included 62 patients with giant VSs, of which 40 were solid and 22 were cystic (those in which cystic component greater or equal to 30% of the total tumour volume). The cystic tumour group was further divided into type A (31-60% volume of the cyst within tumour), type B (61-90% intra-tumoural cyst volume) and type C (more than 90% volume of the cyst). The clinicoradiological features, operative findings, histopathological characteristics and outcome of surgery of the two groups were compared. The mean duration of symptoms for the solid and cystic tumours were 21.1 and 26.2 months, respectively. However, six patients with cystic tumours showed recent and rapid neurological deterioration after a protracted existence. Papilloedema, lower cranial nerve involvement, facial paraesthesias and preoperative hydrocephalus were significantly more in cystic tumours. Total excision was achieved in 38 of the solid and 18 of the cystic tumours. VIIth nerve preservation was higher in the cystic lesions [solid 33/40 (82.5%), cystic 21/22 (95.4%)]. Myxoid degeneration, lobular growth patterns and cellular atypia were more prominent in the cystic variants. The giant vestibular schwannomas were associated with a higher incidence of cystic degeneration than has been reported for smaller tumours in literature. In cystic lesions, VIIth nerve preservation was higher due to early decompression of the lesion that facilitated in early identification of the VIIth nerve, except in patients with type C cystic tumour.
Solitary intraventricular metastasis is an extremely rare entity with poor outcome following surgery. A 26 years old male presented with features of raised intracranial pressure. An extensive intraventricular tumor was diagnosed as septal glioma on imaging. A gross total microsurgical excision of a vascular infiltrative tumour with subependymal infiltration resulted into marked pneumocephalus and progressive oedema of white matter. The patient died on 4th postoperative day the biopsy of tumor specimen revealed a metastatic papillary adenocarcioma. Venous infarct was thought to be the cause of an extensive white matter infarct. Literature is reviewed to explore the primary sites, clinical manifestations and causes of mortality in these cases.
AIM Efficacy of DWI in detecting ischemic injury following anterior communicating artery aneurysmal SAH is studied MATERIAL AND METHODS In this prospective study ,16 patients within 5 days of their ictus were included. Preoperative CT scan excluded an intracerebral infarct; an angiogram determined the extent of vasospasm; MR studies were done to detect cerebral ischemia and the diffusion coefficient (ADC) was calculated. Patients underwent surgery (n=12) or endovascular coiling (n=4). Postintervention CT scan within 24 hours and on day 5 determined radiological outcome as good or poor depending on the absence or presence of infarction. Clinical outcome at follow up (2-6 months) was regarded as good or poor when the patient was independent or dependent regarding daily activities, respectively,. RESULTS Restricted diffusion occurred in 8 (80%) of poor and in 1 (16.6%) of good grade patients (p < 0.05). In patients with good radiological outcome (n=8), DWI restriction occurred in 2 (25%) patients. In patients with poor radiological outcome(n=8), DWI restriction occurred in 7 (87.5%) patients . At 2-6 month follow-up, 7 (77.8%) of 9 patients with restricted diffusion and 2 of 7 patients with no DWI abnormality showed a poor clinical outcome CONCLUSION Clinical and radiological outcome at follow-up is related to the preoperative DWI.
Intracranial aneurysms in childhood account for 1-2% of intracranial aneurysms.[1],[2] These aneurysms have unique characteristics that make them different from those in adults. These differences are evident in their epidemiology, location, clinical spectrum, association with trauma and infection, complications and outcome.
AIMS: To report management results of vestibular schwannomas (VS) treated surgically in our institute, with particular reference to completeness of tumor excision, facial nerve and hearing preservation and complications of surgery. STUDY DESIGN AND SETTINGS: Retrospective study of 259 patients treated during the years 1988 to 2002. MATERIALS AND METHODS: The facial nerve function and hearing assessment was done according to House-Brackmann [HB] grading and pure tone audiometry (PTA) respectively. All patients were operated by retro-mastoid sub-occipital approach. RESULTS: Most patients had large tumors and had no useful hearing (90%), had disabling cerebellar ataxia (88%) and presented with features of raised intra-cranial pressure (45%). Large sized tumors were in 41.3% and giant sized tumors were in 56% cases. Complete tumor excision was carried out in 96.5% and anatomical preservation of facial nerve was achieved in 79.2% cases. Hearing preservation was achieved in 8 patients. Cerebrospinal fluid leak with or without meningitis and transient lower cranial nerve paresis were common complications. The mortality was 6%. CONCLUSIONS: With experience, complete tumor excision with good facial nerve preservation can be achieved in large tumors. Hearing preservation is difficult in bigger tumors. Prevention and control of infection was a major concern.
Thirty-four patients (18 men, 16 women, age range 40-80 years) with primary degenerative dementia, diagnosed by DSM-III (American Psychiatric Association 1980) criteria, were studied. All patients underwent thorough examination that included electrocardiogram (ECG) , electroencephalogram (EEG) , computed tomogram (CT), chest x-ray, blood count, serum electrolytes, blood urea and sugar, urine analysis, liver function tests, serological tests for syphilis, etc., which were designed to rule out any diseases that could cause or contribute to the dementia. The control population consisted of 34 age-matched, nonneurological and nonpsychiatric patients (25 men and 9 women). After informed consent, lumbar puncture was