A series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 53% of patients and dramatic improvement in the others. Surgical observations led us to propose a new anatomical classification according to the anatomical relationship between the hamartoma and the adjacent hypothalamus and third ventricle. Endoscopic disconnection seems to be a very safe way to treat hamartomas in intraventricular locations.
Artificial models of cerebral aneurysms for medical training and testing of medical devices were constructed from corrosion casts of the main cerebral arteries of a human specimen. Three aneurysms with a variety of shapes were simulated at typical locations. Rigid and soft models were made of silicone using the “lost wax” technique. The transparent silicone models were anatomically accurate and reproducible copies of human vascular casts. These models could be connected in a closed circuit that used an electric pump to simulate pulsatile flow. Endovascular procedures and surgical clip application were performed under fluoroscopic or direct visual control. Surgical clipping, endoluminal coil manipulation, and consecutive hemodynamic changes were visualized by digital subtraction angiography and direct observation. The model provides trainee surgeons with an understanding of clinical conditions. New medical devices, such as platinum coils, would be experimentally implanted in the model under stable conditions. These anatomically accurate and reproducible models of cerebral vasculature and aneurysms are valuable for medical testing, training, and research.
Neurons and glial cells respond to extracellular hyperosmolarity by accumulating small organic solutes, called “osmolytes.” Na+/myo-inositol is one of the major organic osmolytes in the brain and Na+/myo-inositol cotransporter (SMIT) regulates extracellular Na+/myo-inositol content. Subarachnoid hemorrhage (SAH) is an osmotic stress-inducing event of the brain. The expression of SMIT messenger ribonucleic acid (mRNA) and protein was investigated with in situ hybridization and immunohistochemistry in rat brains with SAH induced by endovascular perforation. SMIT riboprobe was raised from a 490-bp rat SMIT complementary deoxyribonucleic acid. Anti-SMIT antibody was raised in rabbits. SMIT mRNA was expressed strongly in the cortex, hippocampus, and hypothalamus of the perforated side at 6 to 24 hours after SAH. Mild upregulation was noted in the contralateral cortex, hippocampus, and hypothalamus. The ventral aspect of the pons showed mild upregulation. Microautoradiography and immunostaining showed SMIT expression mainly in the neurons, but also in some non-neural cells in the hippocampus. The present results indicate that diffuse osmotic stress occurs in the host brain after SAH.
A one-year, 9-month-old boy presented with pediatric head trauma associated with unusual accessory cranial sutures. Radiography demonstrated unusual bilateral longitudinal linear bone defects extending from the foramen magnum to the mendosal sutures, and bilateral transverse linear bone defects around the foramen magnum. No swelling or soreness were found in the occipital area, and there was no past history of head trauma. Therefore, the bone defects were considered to be accessory cranial sutures. Complex developmental patterns of the occipital bone and the considerable normal variation of sutures may simulate fractures around the foramen magnum. The present case of accessory cranial sutures is another example.
A 32-year-old woman was brought to the emergency room with hemiplegia on the left and consciousness disturbance. Her prior medical history and the circumstances of the onset were unknown. Brain computed tomography showed intracerebral hemorrhage (ICH) with a midline shift of more than 10 mm in the right parietal lobe. Cerebral angiography failed to show any vascular anomalies. Urine analysis with the triage system, a qualitative screening test for psychotropic drug abuse, showed positive reaction for amphetamines. Subsequent laboratory examination confirmed a highly elevated serum concentration of methamphetamine. The patient underwent evacuation of the hemorrhage via a craniotomy, and was discharged 40 days after admission. Abuse of illegal drugs including amphetamines among young adults is increasing in many developed countries, and the suspicion of possible drug abuse should always be raised in young patients with angiographically negative ICH. A urinalysis screening test for psychotropic agents should be a part of routine emergency room diagnostic procedures for such patients.
An 11-week-old male infant presented with intracerebral hemorrhage associated with coagulopathy manifesting as left hemiparesis, lethargy, and vomiting. Computed tomography demonstrated extensive right frontoparietal intracerebral hemorrhage extending into the ventricular system. Liver function tests revealed abnormal values of transaminases and bilirubin. Blood coagulation studies showed prolonged prothrombin time (PT) and activated partial thromboplastin time (APPT). PT and APTT immediately normalized after the administration of vitamin K and fresh frozen plasma. Right parietal craniotomy and evacuation of the hematoma were performed because of the deterioration in consciousness and left hemiparesis. No vascular abnormality was observed in the hematoma cavity. After surgery, he became alert and the left hemiparesis improved. There is a risk of intracerebral hemorrhage due to vitamin K deficiency even if prophylactic administration of vitamin K was given. Surgical treatment should be considered for the treatment of infantile spontaneous intracerebral hemorrhage, especially if neurological deterioration is present.
A 20-year-old male presented with an osteosarcoma in the right parieto-occipital bone occurring as a painless occipital lump which had rapidly enlarged in the 6 months prior to admission. The neuroimaging appearance resembled intraosseous meningioma. Gross total resection of the tumor was achieved. The final histological diagnosis was osteosarcoma. Osteosarcomas of craniofacial region have a better prognosis than those of the skeletal bones, and distant metastasis is rare. Local recurrence is the most significant factor contributing to poor outcome. Complete excision with negative margins is the key to a better outcome. Adjuvant therapy may be an option in cases of incomplete excision. Advances in target chemotherapy may diminish the significant morbidity associated with these lesions.
A 57-year-old man presented with a posterior fossa hemangioblastoma associated with primary hyperparathyroidism. The hemangioblastoma was completely removed. Further imaging found parathyroid and thyroid tumors which were resected. Hyperparathyroidism might be a manifestation of von Hippel-Lindau (VHL) disease, but sequencing of the VHL gene of the constitutional deoxyribonucleic acid of the hemangioblastoma was negative. The present association is rare, but the hemangioblastoma and the parathyroid adenoma may have some germ-line mutation in common.
A 33-year-old male presented with a rare intracranial oculomotor schwannoma not associated with neurofibromatosis manifesting as an 8-month history of right oculomotor nerve paresis. Neuroimaging examinations revealed a 4-cm mass in the right crural cistern. The tumor was totally removed. The right oculomotor nerve paresis resolved 7 months after surgery. Removal of the oculomotor or trochlear schwannoma usually results in parent nerve paresis, but subcapsular tumor resection in our case probably allowed complete recovery of the nerve function.
A 32-year-old female presented with a rare case of pituitary abscess manifesting as homonymous hemianopsia. Serum prolactin level was slightly high (40.8 ng/ml). Magnetic resonance (MR) imaging showed the content of the lesion as homogeneously isointense on the T1-weighted images and hyperintense on the T2-weighted images. The capsule of the lesion, which appeared thin and smooth, was enhanced by gadolinium. Dural enhancement around the sella turcica was also recognized. Thallium-201 single photon emission computed tomography (201Tl SPECT) showed homogeneous high accumulation in the pituitary region on both the early and delayed images. The lesion was treated via a transnasal-transsphenoidal approach. The cystic lesion contained pus and the capsule consisted of normal pituitary gland with inflammatory changes. The patient was treated with antibiotics for 3 weeks and the pituitary abscess was cured completely. Pituitary abscess can be differentiated from pituitary adenoma as lesion with a homogeneous high uptake on 201Tl SPECT on both the early and delayed images, and no enhancement of the central portion on MR images.