October 2000: a 47 year old man with long-standing progressive tetraparesis.

A 47-year-old man with a six-month history of progressive tetraparesis and sphincter disturbances developed tetraplegy after two years and died. A cervical MRI revealed an ill-defined, enhancing lesion from C1 to C6, internally attached to the dura mater and compressing the spinal cord. At necropsy, eight whitish nodes and diffuse thickening of the dura mater from C1 to C6 were found. Histological studies revealed multiple en plaque lymphoplasmacyte-rich meningiomas. The radiologic and pathologic aspects of lymphoplasmacyte-rich meningioma are reviewed and the atypical features of this case, such as macroscopic appearance and the histological variant, are described.

[An en bloc cranio-orbitozygomatic approach: surgical technique and results]. / Acesso crânio-orbitozigomático em flape único. Técnica cirúrgica e resultados.

An en bloc cranio-orbitozygomatic approach is described. This technique was applied in seven patients (five basilar artery aneurysms, a trigeminal neuroma, a meningioma of the lesser sphenoid wing). The follow-up period ranged from 3 to 50 months. The patients were retrospectively analyzed from the cosmetic point of view, and submitted to computerized tomography with three dimensional and multiplanar reconstructions. The access provided a wider operative field with a shorter distance to the pathology and possibility of increased angulation of the microscope. The aneurysms could be clipped and the tumors completely removed in all cases. A frontalis muscle paralysis occurred in two cases, as well as a temporalis muscle atrophy in another two patients. There were no enophthalmos or bone flap displacements. The surgical technique is simple and do not require drills, electric saws and mini-plates.

[Brown-Vialleto-van Laere syndrome: report of 2 cases]. / Síndrome de Brown-Vialleto-van Laere: relato de dois casos.

Brown-Vialetto-van Laere syndrome is a rare hereditary or sporadic degenerative disorder characterised by progressive sensoryneural deafness, followed or accompanied by cranial nerve palsies. The anterior horn cells and the optic pathways may be involved in some cases. We report two cases, and comment the differential diagnosis and the relationships of this syndrome to the muscle spinal atrophies and the hereditary deafness.

Granuloma formation and arterial thrombosis following cotton wrapping of an intracranial aneurysm. A case report.

The authors report the case of a patient whose left middle cerebral artery aneurysm was wrapped with cotton. Occlusion of the middle cerebral artery, probably secondary to a foreign-body inflammatory reaction, developed late in the postoperative course. A computerized tomography scan revealed cerebral infarct, and an enhancing expansive lesion at the site of the aneurysm suggesting the formation of a granuloma. These findings are discussed and the literature is reviewed.

[Russel's syndrome. Report of a case with development of precocious puberty]. / Síndrome de russel. Apresentação de um caso com desenvolvimento de puberdade precoce.

The Russel's syndrome is characterized by emaciation and hyperactivity in children and is caused by hypothalamic expansive process. This report presents the typical signs of the syndrome associated with growth hormone secretion disorders and precocious puberty pattern appearing at the age of four. The pathogenesis of the disorder is discussed and related to the hypothalamus-pituitary axis dysfunction caused by the tumor growth.

Leopard syndrome, a neural crest disorder: a case report.

A case of Leopard syndrome with full clinical expression is reported. In addition to the cardinal signs of the syndrome this patient presented some abnormalities which have not been previously described such as macroglossia, multiple dental anomalies, basilar impression and platybasia, megacolon, hypertrophy of clitoris and anal ectopy. The presence of dental anomalies and megacolon may represent involvement of the dental papillae and myenteric plexus favoring the view that the syndrome results from a derangement of the neural crest elements.

Leopard syndrome, a neural crest disorder: a case report.

A sindrome de Leopard e uma condicao autosoma dominante de variavel expressao fenotipica.Uma minoria apenas dos pacientes apresenta todos os componentes da sindrome, ou seja, lentigenes multiplas (L), alteracoes eletrocardiograficas (E), hipertelorismo ocular (O) estenose pulmonar (P), anormalidades genitais (A), retardo de crescimento (R), e surdez neurosensorial (D). Descrevemos o caso de uma crianca com todos os componentes da sindrome alem de outras anomalias nao descritas anteriormente, tais como macroglossia, alteracoes dentarias, megacolon, ectopia anal e hipertrofia do clitoris, impressao basilar e platibasia. A presenca de alteracoes dentarias e megacolon, representando envolvimento das papilas dentarias e do plexo mioenterico, derivados da crista neural,constitui nova evidencia que favorece a hipotese de ser a sindrome de Leopard resultante de anomalia dos elementos da crista neural

[Aphasia caused by paramedian lesion of the dominant hemisphere: report of a case]. / Afasia produzida por lesão paramediana do hemisfério dominante: registro de um caso.

A 41 year old woman with a ruptured left pericalosal artery aneurysm and hematoma in the inter-hemispheric fissure and paramedian region of the left hemisphere presented with mutism which turned to aphasia characterized by absent spontaneous speech, lack of paraphasias, relative preservation of repetition and evident preservation of comprehension. This picture conforms to the so called "trans-cortical motor aphasia". The fact that lesions of the paramedian region of the dominant hemisphere, in and around the secondary motor area, can produce aphasia is unknown to many neurologists and several such cases are probably misdiagnosed due to the transience of the major symptoms and to confusion with non-specific dementia.

[Surgical treatment of atlanto-axial subluxation by transoropharyngeal approach. Report of a case]. / Tratamento cirúrgico de sub-luxação atlanto-axial por via transorofaringea. Relato de um caso.

A case of atlanto-axial subluxation in a 17 year old boy with rheumatic disease is reported. The subluxation could not be reduced by cervical traction. Posterior fixation with acrilic and wire was unable to prevent the appearance of signs of spinal cord compression. The odontoid process was removed through a transoral approach leading to neurologic recovery. Treatment of atlanto-axial subluxation is discussed, emphasis being given to the indications for anterior descompression.

[Diabetes insipidus after myelography. Report of a case]. / Diabete insípido após mielografia. Registro de um caso.

The case of a 28 year-old man in whom diabetes insipidus appeared 24 hours after Pantopaque myelography is reported. This unusual complication, not previously reported, probably was caused by a hypersensitivity reaction or mechanical-irritative effect on the hypothalamic-hipophyseal area produced by suprasellar Pantopaque droplets, demonstrated by skull x-rays. The patient improved after being treated with steroids. Some serious complications of myelography can be prevented by avoiding supratentorial spillage of Pantopaque.

Subdural hematomas due to ruptured cerebral aneurysms: angiographic diagnosis and potential pitfall for CT.

Two patients with subdural hematoma secondary to rupture of intracranial aneurysms are described. Computed tomography and plain skull films are generally the examination of choice for the patient with significant head trauma. In selected patients, however, cerebral angiography in place of, or along with, computed tomography may be necessary to demonstrate an underlying cause for the subdural hematoma.

[Myotubular or centronuclear myopathy; report of a case and review of the literature]. / Miopatia miotubular ou centronuclear; relato de caso e revisão da literatura.

A case of centronuclear or myotubular myopathy in a 13-year-old boy who was admitted with hypotonia and congenital ptosis is reported. Clinical, electromyographic and pathologic studies are discussed. A review of the electron microscopic features and comparison with out findings is included. Review of the literature disclosed near 50 cases of centronuclear myopathy. The diferential diagnosis is discussed.

[Pituitary apoplexy after pneumoencefalogram]. / Apoplexia hipofisaria apos pneumencefalograma

A case of pituitary apoplexy following the performance of a pneumoencephalogram is reported. No such a case has been published in the literature, to the author's knowledge. The etiopathogenetic mechanism is discussed and a recomendation is made that surgery should be performed straight after completion of the pneumoencephalogram, should it show a significant supraselar extension of a pituitary adenoma, even in the absence of increased intracranial pressure.