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PubMed2005

Pineal region giant cell astrocytoma associated with tuberous sclerosis: case report.

Tuberous sclerosis complex is a genetic disorder characterized by the development of hamartomas in multiple organs including the brain, skin, eye, kidney, and heart. Neurological features include seizures and mental retardation. Cortical tubers and subependymal nodules are the characteristic intracranial lesions of tuberous sclerosis. Subependymal giant cell astrocytomas, typically located adjacent to the foramen of Monro, can enlarge and cause symptomatic ventricular obstruction. The authors describe the case of a 3-year-old boy with a history of tuberous sclerosis and retinal lesions who presented with an enlarging enhancing pineal region mass. Via an infratentorial supracerebellar approach, the mass was removed using both the operative microscope and a rigid neuroendoscope. Pathological examination showed a giant cell astrocytoma. To the authors' knowledge, this is the first reported case of tuberous sclerosis associated with a giant cell astrocytoma of the pineal region. Diagnostic considerations are discussed.

Astrocytoma↗
PubMed2003

Intraventricular meningiomas: a review of 16 cases with reference to the literature.

BACKGROUND: Primary intraventricular meningiomas are notably rare with an incidence of 0.5 to 3% among all intracranial meningiomas. We present a series of 16 patients with histologically verified intraventricular meningiomas, the clinical and neuroradiological findings, and surgical results. METHODS: The charts of the patients including surgical records, discharge letters, histologic records, follow up records and imaging studies were analyzed retrospectively from 1978 to 2001. RESULTS: Collectively, 110 primary intraventricular tumors were surgically resected in our neurosurgical department from 1983 through 2001. There were 16 patients with primary intraventricular meningiomas. Thirteen (81.3%) of these tumors were located in the lateral ventricles, one (6.2%) in the third and two (12.5%) in the forth ventricle. Mean age was 47.1 years and there was no difference in incidence between the sexes. The most common presenting symptoms and signs were headache, mental change, and hemianopia. Other symptoms included gait ataxia, vertigo, hemiparesis, double vision, aphasia, and alexia/agraphia. On CT and MR studies most of the lateral ventricular meningiomas were located in the trigone (11 cases), two tumors originated at the level of foramen of Monro or in the frontal horn. The tumors were approached via a transcortical parieto-occipital (11 cases), transcallosal (3 cases), or median suboccipital (2 cases) route. Total removal was achieved in 15 out of 16 cases. There was no operative mortality. Clinical improvement was seen in symptoms like headaches, paresis, cerebellar signs and visual disturbances, whereas mental change showed less improvement. CONCLUSIONS: Although intraventricular meningiomas are quite rare, they represent an important differential diagnosis of intraventricular neoplasms. They can reach a substantial size by the time of diagnosis, yet total removal can be achieved in most cases.

Adult↗
PubMed2000

Cerebral embolization presenting as delayed, severe obtundation in the postanesthesia care unit after total hip arthroplasty.

Catastrophic neurologic events occur rarely postoperatively and must be diagnosed quickly. A 63-year-old woman who had undergone uneventful total hip arthroplasty experienced obtundation after admission to the postanesthesia care unit. Cranial magnetic resonance imaging revealed multiple lesions consistent with ischemia or infarction, and fat cerebral embolism was diagnosed. We describe the numerous complications that may occur in patients in the postanesthesia care unit and review the differential diagnosis of altered mental status in such patients. Paradoxical cerebral fat embolization must be considered in the differential diagnosis of altered mental status after pelvic or long bone fracture or lower extremity major joint replacement, and this condition may occur despite normal pulmonary function and no patent foramen ovale or right-to-left intracardiac shunt. Magnetic resonance imaging with T2-weighted sequences is the cranial imaging study of choice for early evaluation of patients with sudden multifocal neurologic deficits and suspected fat embolism syndrome.

Anesthesia Recovery Period↗
PubMed2006

Anastomosis between the inferior alveolar artery branches and submental artery in human mandible.

The mental artery displays several branches internal to the anterior region of the mandible as confirmed by macroscopic observation and computed tomography. The inferior alveolar artery formed complex branches and divided into mental and incisive branches, which were found in the right internal side of the mandible of one male cadaver (88 years old). The branches of these two arteries ran through the bony lingual canal to the lingual foramen between the canine and premolar region of the inner surface of the mandible body, where they emerged to enter the mylohyoid and anterior belly of the digastric muscles and communicate with the submental artery. The observation of the anastomotic artery is considered important for surgical placement of dental implants in the mandibular region.

Aged, 80 and over↗
PubMed1982

[Sibling case of osteosclerosis with cranial nerve symptoms].

We experienced two cases of "Osteosclerosis" who were 12 and 15 year old sisters. Previous reported cases of this disease are 50 cases and among them only one patient was reported in Japan. Osteosclerosis seems to be inherited as an autosomal recessive trait. Parental consanguinity is also observed. The peculiar facies are evident in infancy, characterized by broad, flat nasal bridge, ocular hypertelorism and prognathic, broadened mandible. Commonly, they have cutaneous syndactylies in bilateral hands and feet, especially between the second and third finger and toe. Roentgenographically, hyperostosis with osteosclerosis can be observed in systemic bones, particularly the calvarium is greatly thickened. Since such a bony change occurs most severely at the base of the skull, important clinical symptoms of this disease are cranial nerve palsies resulting from obliterations of unilateral or bilateral several cranial nerve foramina. In many cases deafness due to progressive encroachment upon the middle ear cavities and auditory nerve canals appears early in infancy. Transient palsy of the facial nerve occurs somewhat later, and bilateral facial paralyses are usually permanent in adulthood. In some cases optic atrophy and visual field defect due to compression of the optic nerves are late complications. Other ocular symptoms are strabismus, nystagmus and exophthalmos. Anosmia and trigeminal nerve palsy are less common. Lower cranial nerve symptoms can not be noted but the reason is unclear. Chronic headache, convulsion and mental retardation are occasionally present. They are considered as a result from increased intracranial pressure due to progressive diminution of the cranial capacity. By same mechanism, several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum in early adulthood. Then, some reporter puts emphasis on prophylactic opening of the foramen magnum in all adult cases.

Adolescent↗
PubMed1998

Generator sites of early scalp potentials evoked from the three trigeminal branches.

OBJECT: The aim of this study was to seek evidence about the generators of the first three components of the scalp's early trigeminal evoked potentials (TEPs) obtained by stimulation of the supraorbital (SW1, SW2, and SW3), infraorbital (W1, W2, and W3) and mental (MW1, MW2, and MW3) nerves. METHODS: Simultaneous scalp and depth recordings were measured during surgical procedures in which thermorhizotomy and microvascular decompression were performed. CONCLUSIONS: Direct evidence was found that the origin of MW1 lies in the mandibular nerve at the foramen ovale, whereas the origin of W1 in the maxillary nerve at the foramen rotundum and the origin of SW1 in the ophthalmic nerve at the superior orbital fissure could only be inferred. The generators of SW2, W2, and MW2 were found to be on the nerve root at a distance of 10 mm from the pons. Calculations based on conduction velocity suggested that the generators of SW3, W3, and MW3 were inside the brainstem, at distances between 16 mm and 20 mm from the root entry zone. Recordings obtained in eight patients with discrete surgical lesions of the trigeminal pathway confirmed the sites of origin of the early components and further proved that only the fastest group of fibers is responsible for scalp responses.

Electric Stimulation↗
PubMed1995

Non-progressive familial congenital cerebellar hypoplasia.

A syndrome is reported of congenital non-progressive, gradually slightly improving, ataxia in 3 out of 5 male sibs, issues of a first-order consanguineous mating. Additional characteristic features included: moderate microcephaly, generalised muscle weakness and hypotonia, nystagmus, and moderate mental retardation. A pyramidal syndrome of hyperreflexia and Babinski signs, without any spasticity, became manifest in the 2nd or 3rd year of life. In all three, the caudal part of the vermis was absent, the enlarged IVth ventricle opening up via Magendie's foramen into the cisterna magna. The middle and rostral vermian parts as well as the sagittal paravermian parts of the cerebellar hemispheres were hypoplastic. The differential diagnosis of this syndrome is analysed and the developmental pathogenetic mechanisms likely to produce the typifying distribution of aplasia are indicated.

Ataxia↗
PubMed2005

Severe epilepsy and pachygyria associated with peculiar facial traits characterize Fryns-Aftimos syndrome.

We describe an 18-year-old boy with a frontofacial syndrome. The presence of severe epilepsy led to a neuroimaging investigation, which revealed a diffuse pachygyric (normal thickness and simplified folding) cortex that was bilateral and affected all supratentorial regions, sparing the medial and inferior aspects of the frontal lobe plus the posterior occipital region. In addition, an unusual skull base presentation with small posterior fossa, narrowed foramen magnum, and low torcular and tentorial insertions was documented. Dysmorphic ventricles and enlarged cavi of the septi pellucidum, vergae, and velum interpositum were also noted. This case shares many similarities with two patients formerly described by Fryns and Aftimos, who were previously classified as having mental retardation and multiple congenital anomalies syndrome. The existence of a new case corroborates the idea of a distinct condition with particular characteristic dysmorphic facial features and the presence of a specific malformation of cortical development associated with severe epilepsy. We believe that epilepsy and pachygyria play a central role in distinguishing this syndrome from others classified as frontofacial or cerebrofrontofacial syndromes, and we propose the term "Fryns-Aftimos syndrome" to name it.

Abnormalities, Multiple↗
PubMed2001

Migratory neurocysticercosis mimicking a third ventricular colloid cyst. Case report.

The authors report on a case of solitary neurocysticercosis located in the anterior third ventricle of a 21-year-old Hispanic man who presented with progressive headache, nausea, and mental decline. The appearance of the lesion on computerized tomography and magnetic resonance studies was typical of that of a colloid cyst. which was the presumptive preoperative diagnosis. A transcortical-transventricular microsurgical approach was used, and inspection of the anterior third ventricle via the ipsilateral foramen of Monro revealed no lesion. Inspection of the floor of the ipsilateral lateral ventricle revealed the intact cyst, which had migrated since the preoperative studies had been obtained. The lesion was removed intact, and histopathological examination confirmed the diagnosis of neurocysticercosis. The authors review the radiographic findings in this case and further illustrate the potential for neurocysticercosis to masquerade as other intracranial lesions.

Adult↗
PubMed1979

FAMILIAL VALVULAR PULMONIC STENOSIS INVOLVING THREE SIBLINGS.

This report describes the cases of three mentally and physically well-developed siblings, 12, 10, and 8 years of age, with varying degrees of isolated valvular pulmonic stenosis not related to age. The severest lesion occurred in the middle patient and was associated with a right-to-left shunt through a patent foramen ovale. The three children had no other siblings, and there was no history of congenital heart disease among close relatives. These cases support the conclusion that genetic factors play a significant role in the development of certain congenital cardiac disorders.

Journal Article↗
PubMed2002

A new fossil mustelid from the Miocene of South Dakota, USA.

A skull from the Barstovian of South Dakota has typical leptarctine characteristics, including robust zygomatic arches, double sagittal crests, grooves on the lingual side of the lower canines, and bony projections from the tympanic bullae. The robust mandibles and expanded masseteric fossa of this specimen indicate that it had large jaw muscles. Dental morphology and other characters lead us to agree with earlier suggestions that Hypsoparia is a valid genus. The morphology of Hypsoparia suggests that leptarctines were more herbivorous than most other Carnivora. Mustelids vary greatly in size and include 67 extant species in 25 genera. These species occupy many habitats, including fresh and salt water, and all land areas of the world except the West Indies, Madagascar, Sulawesi, Antarctica, and most oceanic islands (Nowak 1999). Qiu and Schmidt-Kittler (1982) considered Leptarctinae to be a subfamily of mustelids including Craterogale (North America, Middle Miocene), Trocharion (Europe, Middle Miocene), Hypsoparia (North America, Upper Miocene), and Leptarctus (North America and Asia, Lower to Upper Miocene). Leptarctus ranges from basal Hemingfordian strata to the Early Hemphillian (Lim 1999). The characters diagnosing Leptarctus as a mustelid include absence of M2, absence of a notch between the paracone and the metacone of the upper carnassial, and a reduced dentition with loss of P1 and p1. Among mustelids, Leptarctus also has many unique characters, including prominent double sagittal crests, a well-developed hypocone on P4, grooved lower canines, heavy zygomatic arches, and bony projections on the tympanic bullae (Lim 1999). Dorr (1954) erected a new genus and species of leptarctine mustelid from the Late Miocene of Montana, USA: Hypsoparia bozemanensis. Qiu and Schmidt-Kittler (1982) confirmed it as a taxon separate from Leptarctus. However, McKenna and Bell (1997) and Baskin (1998) synonymized H. bozemanensis with Leptarctus primus. The new specimen from the Miocene of South Dakota, USA, provides evidence that Hypsoparia is a separate genus from Leptarctus. We can describe this animal as follows: Systematic paleontology: class, Mammalia; order, Carnivora; family, Mustelidae (Fischer von Waldheim 1817); subfamily, Leptarctinae (Gazin 1936); genus Hypsoparia (Dorr 1954). Emended diagnosis: the muzzle short, deep, and wide compared with that of Leptarctus; small, round infraorbital foramen; relatively greater height of zygomatic arch; upper canine with anterior wear; P2 and P3 unicuspid with complete cingula; P4 and M1 longer than wide; labial side longer than lingual side on P4; anterior margin of M1 curving inward; mandibles larger and more robust than in L. primus; mandible deep with two mental foramina located below p2 and p3; masseteric fossa enlarged; m2 longer than wide; m1 with a broadly basined talonid; p3 and p4 with prominent cingula.

Animals↗
PubMed1995

Tuberous sclerosis associated with histologically confirmed ocular and cerebral tumors.

The clinical, neuroradiologic, and pathologic features of an unusual retinal and cerebral giant cell astrocytoma in a 24-year-old man with tuberous sclerosis are reported. The patient was referred at 3 years of age because of partial seizures from the first months of life, severe mental retardation, and left microphthalmos. The microphthalmic eye presented slow growth from 9 years of age and was enucleated at age 18 years because of exophthalmos and pain. At age 23 years, the patient experienced sudden and severe headache. Magnetic resonance imaging revealed a voluminous cystic tumor in the region of the foramen of Monro, lateral ventricle, and basal ganglia of the right cerebral hemisphere. Pathologic examination of the enucleated eye and of the cerebral tumor disclosed the same histologic findings in both locations, a giant cell astrocytoma.

Adolescent↗
PubMed1985

Third ventricle gliomas. Report of 7 cases with benign clinical behaviour.

Neoplasms of the third ventricle are lesions arising within the ventricular cavity, often free but more often pedunculated. True third ventricle tumours are surgically removable. In a consecutive surgical series of 580 intracranial tumours the authors operated on 27 lesions of the third ventricle, 7 of which were benign gliomas. The histological diagnosis was polar spongioblastoma in 5 cases and fibrillar subependymal astrocytoma in 2 cases. The dominant clinical features were a slowly progressive or intermittently increased intracranial pressure syndrome, and mental and visual disturbances. CT and CSF contrastographic studies provided the essential diagnostic data for the therapeutic plan. CT scans at follow-up studies confirmed the successful removal of the lesions. A right trans-ventricular approach was employed in all cases. Depending on the size and position of the lesion, removal was performed through the foramen of Monro or through a sub-choroidal approach. In two patients a ventriculo-atrial shunt was necessary in spite of tumour removal. No surgical mortality occurred and patients are well at a follow-up time of from 2 to 6 years.

Adolescent↗
PubMed2004

Robotic techniques improve quality of life in patients undergoing atrial septal defect repair.

BACKGROUND: Minimally invasive cardiac surgery has emerged as an alternative to conventional, open surgery. Although most studies of robotically assisted cardiac surgery have reported morbidity and mortality, few have addressed outcome measures, such as pain and quality of life, which was the aim of this study. METHODS: Eleven patients with atrial septal defects (ASD), and five patients with patent foramen ovale, underwent repair using the Da Vinci system (Intuitive Surgical, Mountain View, CA). The Medical Outcomes Study Short Form Survey (SF-36), along with two additional questions, were administered to these patients on postoperative day 30, along with a similar number of patients who underwent ASD repair by mini-thoracotomy or sternotomy. Quality of life endpoints included bodily pain, vitality, mental health, general health, physical function, and social function. RESULTS: Robotic patients demonstrated significantly higher scores in 6 of the eight variables (p < 0.05). There was no significant difference in intensive care unit or overall hospital stay among the groups (p = NS). Robotic patients returned to work after 40.2 +/- 30.2 days, mini-thoracotomy patients after 45.6 +/- 27.9 days, and sternotomy patients after 51.7 +/- 40.2 days (p = 0.767). There were no significant differences in SF-36 scores between patients who underwent mini-thoracotomy and sternotomy approaches. CONCLUSIONS: Closure of an ASD can be performed safely and effectively via an endoscopic approach. Robotic technology minimized the degree of invasiveness, hastened postoperative recovery, and improved quality of life, although length of hospital stay was unchanged.

Adult↗
PubMed2006

Coronary arterial air embolus occurring during cesarean delivery.

We present a case of a severe systemic (paradoxical) air embolism occurring during spinal anesthesia for cesarean delivery in an otherwise healthy 35-year-old parturient. Uncomplicated spinal anesthesia and satisfactory surgical anesthesia were obtained; no sedatives were used and the patient was awake and alert and tolerating the procedure well. Immediately following infant and placental delivery (approximately 25 min after the spinal anesthetic was induced) the patient had acute onset of markedly decreased mental status, profound ventricular ectopy and labile blood pressure. The event lasted for approximately 10 min with spontaneous resolution. Neurologic status returned to normal by the end of the surgery, but electrocardiogram findings in the immediate postoperative period were consistent with myocardial ischemia and serial cardiac troponin levels confirmed myocardial injury. On postoperative day 1, an echocardiogram demonstrated the presence of a patent foramen ovale. The events in this case are likely to be due to paradoxical coronary and cerebral air embolism.

Adult↗
PubMed1990

[Cerebral white matter bundle alterations in patients with dementia of Alzheimer type and patients with multi-infarct dementia--magnetic resonance imaging study].

The widths of the anterior white matter bundle (AWM) and the interhemispheric (AWM-TER) and intrahemispheric (AWM-TRA) bundles at the level of the foramen of Monro on horizontal inversion recovery MRI scans were measured in 17 patients with dementia of Alzheimer type (DAT), 16 patients with multi-infarct dementia (MID) and 47 age-matched normal subjects (NOR). The area of the corpus callosum (CC) on midsagittal spin-echo MRI scans was also measured in 12 DAT, 11 MID and 36 NOR. The Mini-mental score in patients with DAT was 20.8 +/- 5.9, which was not significantly different from that of 22.9 +/- 5.4 in patients with MID. The width of AWM was not significantly decreased in either DAT or MID compared with NOR. However, the width of AWM-TER in DAT and MID showed a significant decrease from that in NOR. The width of AWM-TRA was decreased significantly only in DAT. Compared with NOR, the area of the CC in DAT and MID was decreased by 7.0% (p less than 0.05) and 6.5%, respectively. The white matter bundle has an important role in the connectivity of the brain. Although loss of white matter occurs in both DAT and MID, the change appears more diffuse in DAT than MID.

Aged↗
PubMed1979

Computed tomography in tuberous sclerosis--with special reference to relation between clinical manifestations and CT findings.

Concerning the clinical signs and symptoms, we noticed skin white macula in 87%, adenoma sebaceum in 53%, mental retardation in 75%, retinal phacoma in 54% and seizure in 92%. Numbers of nodules ranged from zero to 11, and showed no correlation with aging. Half of the cases showed slight ventricular dilatation, one quarter showed moderate dilatation and the remainder were normal. There was no definite correspondence between size of the ventricle and number of nodules. There were relatively many cases with normal mental state in the group with normal ventricles. In adult cases a large ventricle does not always mean poor prognosis in mental state. Almost all of the cases with cortical atrophy had mental retardation. In the group with moderate dilatation infantile spasms were the most frequent type of seizure. On the contrary, grand mal was most frequent in the normal group. As for the position of nodules there was no difference between the normal and dilated groups. Nodules were predominantly seen in the lateral aspect of the body, trigone of the lateral ventricle and adjacent to the foramen of Monro. Asymmetrical lateral ventricles were noticed in 18%. Incidence of laterality increased as dilatation proceeded. It was larger on the left side in 9 out of 11 cases. We have no reasonable explanation of this tendency. There was one case with a tumor which was verified by enhanced CT. There were five cases with brain anomalies.

Adolescent↗
PubMed1996

Colloid cyst of the third ventricle.

BACKGROUND: Colloid cyst of the third ventricle has rarely been reported in the ophthalmic literature. The incidence is about one per 1000 in asymptomatic patients and accounts for 0.55 to 2 percent of all intracranial tumors. Although it is an uncommon benign tumor, nearly half of symptomatic patients present with papilledema and hydrocephalus. Consideration of this intracranial tumor should be included in the differential diagnosis of papilledema and apparent unilateral disc edema. METHODS: A patient with highly asymmetrical papilledema and vague visual symptomatology involving the right eye was studied. Computer axial tomography (CT) without contrast enhancement revealed a colloid cyst of the third ventricle. Typical symptoms of the condition include headaches, nausea, vomiting, visual obscuration, transient diplopia and weakness of the lower extremities, which are due to acute hydrocephalus caused by increased intracranial pressure from cystic obstruction of the foramen of Monro. Intermittency of symptoms is characteristic of this lesion. RESULTS: The patient underwent frontal craniotomy with total tumor excision and reopening of the cerebrospinal fluid pathway. CONCLUSIONS: Colloid cyst of the third ventricle discovered incidentally on CT in asymptomatic patients with no evidence of hydrocephalus can be monitored periodically. If symptomatic, neurosurgery is usually indicated since prolonged increased intracranial pressure can lead to mental and physical deterioration as well as sudden death.

Brain Diseases↗