Children with reduced sensitivity to pain: assessment of hereditary sensory neuropathy types II and IV.
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Biomedical subjects
Publications and source records attributed to K Berry.
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The records of 15 patients with a diagnosis of malignant meningioma were reviewed. In one of these patients, in whom invasion of the brain and pituitary gland was the only unusual feature, the tumor was reclassified as benign. Seven tumors, four hemangiopericytomas and two transitional and one syncytial meningioma, were considered to be only borderline-malignant despite necrosis and invasion of the brain, because of few mitoses and regular architecture. Of this group of patients, four men and three women, two are alive and well, three died after incomplete resections, and two succumbed to recurrent tumor that had become inoperable. The other seven patients, six men and one woman, had lesions classified as histologically frankly malignant, on the basis of marked anaplasia and numerous mitoses. These comprised three hemangiopericytomas and three syncytial and one fibrous meningioma. One of these patients is alive and well and the others are dead, three a a result of metastases. The initial clinical course of malignant meningiomas tends to be short but is otherwise indistinguishable from that of benign meningiomas. The chances of recurrence and eventual death are high, and extracranial metastases are not rare. The tumors are most often hemangiopericytomas, but not exclusively so, and men are particularly at risk.
Surgical closure of the glottis is reserved for patients who have suffered progressive loss of sphincteric and phonatory laryngeal function. In the prevention of life-threatening aspiration, a triple-layer closure is performed with use of a superiorly based sternohyoid muscle flap. Surgical closure is theoretically reversible should sufficient neuromuscular function return in any given patient.
Superficial temporal artery to middle cerebral artery (STA-MCA) anastomoses were examined at autopsy in two patients who survived 2 years and 5 1/2 years, respectively, after the cerebral revascularization procedure. Identification of the actual anastomotic sites at autopsy was rendered impossible because of marked fibrosis in the region of the surgery. However, in both patients intimal fibrosis and medial damage were noted in the STA immediately adjacent to the anastomosis. In the patient who survived 2 years, the lumen of the STA was only slightly compromised by these changes, but in the patient who survived 5 1/2 years, the artery was almost totally occluded. The MCA's adjacent to the anastomosis were widely patent and showed focal areas of intimal hyperplasia with no abnormalities of the media.
The authors report a case of inversion of part of the cerebral mantle into the frontal horn of the lateral ventricle following ventriculoperitoneal shunting for noncommunicating hydrocephalus. The abnormally positioned cerebral mantle continued to grow and caused no detectable neurological dysfunction.
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The known susceptibility of the Mongolian gerbil to cerebral infarction following unilateral carotid artery ligation has been attributed in the past to the demonstrated absences of an anastomotic supply between the anterior and posterior cerebral circulations. In a study of 34 adult gerbils exposed to such a procedure, 11, or 33%, developed severe neurological sequelae and succumbed to the procedure in less than 30 hr, whereas 23 animals survived with only minor or transient neurological signs. All animals displayed the expected lack of an anastomosis between the anterior and posterior circulations, but in addition the animals which survived the procedure were found to have a prominent early cross-connection between the anterior cerebral arteries, whereas the animals which succumbed had no such connection. Neuropathological changes in susceptible animals were apparent as early as 3 and one-half hr after ligation and consisted of edema, initially perivascular and then intraneuronal, slowed by acute necrosis. A variety of other vascular anomalies was encountered. We conclude that the peculiar susceptibility of Mongolian gerbils to cerebral infarction following acute unilateral common carotid artery ligation is not related primarily to lack o adequate collaterals between the anterior and the anterior cerebral arteries, but to the degree of adequate adequacy of communication between the anterior cerebral arteries. The critical difference may be more one of degree, i.e. the point at which the medial branches of the anterior cerebral artery fust to become anazygos vessel, rather than an actual difference in the pattern of distribution of the anterior cerebral arteries. The presence of other variation in vascular supply in a relatively small series suggests that results of similar studies of infarction and response to treatment be interpreted with caution.
Recently, the presence of thalamic neuronal cytoplasmic inclusions in patients with myotonic dystrophy has been reported. At the ultrastructural level, the inclusions were described "containing a fibrillar material within a limiting membrane studded on its outer surface with ribosomes". We have studied the brain of a 48-year-old woman with myotonic dystrophy. Many neuronal inclusion bodies were found within the thalamus, and examined in the electron microscope. Inclusions were found to have an electron density resembling that of nuclear chromatin, were not membrane-bound, and had an internal structure composed of parallel alternating dark and pale lines. We confirm the previously-reported existence of cytoplasmic thalamic neuronal inclusions in myotonic dystrophy, but differ in our observations of their ultrastructural appearance, and note that these inclusions bear no resemblance to previously described inclusion bodies.
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