Biomedical subjects

A Sotrel

Publications and source records attributed to A Sotrel.

29 records · Page 2Linked to original sources

PubMed1987

Meningeal melanocytoma. Case report and review of the clinical and histological features.

A melanin-producing, encapsulated, histologically benign-appearing tumor of the trigeminal nerve was surgically removed from a 9-year-old boy. It recurred 6 months later and was then subtotally resected and treated with radiation therapy. The clinical and pathological features of this and 10 similar cases reported in the literature are reviewed. These tumors are best categorized as meningeal melanocytomas.

Adult↗

PubMed1986

Thrombotic cerebral vasculopathy associated with herpes zoster.

We describe the clinical, radiographic, and pathological findings in 3 patients with large-vessel cerebral vasculopathy following herpes zoster. Two of the patients were studied at postmortem examination, and a brain biopsy was performed in the third. Each of the 3 patients suffered thrombotic occlusions of large vessels without notable inflammatory or granulomatous changes following trigeminal or segmental herpes zoster infection. In the 2 autopsied patients, varicella-zoster virus (VZV) antigens were detected by immunoperoxidase staining within the media of the affected cerebral arteries. Little or no inflammation was associated with the foci of the VZV antigens. These studies provide evidence that the vasculopathy following herpes zoster may result from direct VZV infection of the artery and the in situ thrombosis can develop within the infected vessels in the absence of clear inflammatory vasculitis.

Adult↗

PubMed1986

Progressive polyradiculopathy in acquired immune deficiency syndrome.

We studied three patients with acquired immune deficiency syndrome (AIDS) and progressive polyradiculopathy. Postmortem examination of one patient disclosed extensive necrosis, inflammatory infiltrates, and focal vasculitis of spinal roots. Typical cytomegaloviral (CMV), intranuclear, and intracytoplasmic inclusions were noted within enlarged endoneurial and endothelial cells. Progressive polyradiculopathy is an unusual complication of AIDS; CMV may be the causative agent in certain cases.

Acquired Immunodeficiency Syndrome↗

PubMed1985

Facioscapulohumeral dystrophy presenting in infancy with facial diplegia and sensorineural deafness.

Six patients are described in whom facial diplegia occurred in the first year of life, with subsequent development of facioscapulohumeral dystrophy. All had severe progressive disability prior to adolescence. Facial involvement did not include extraocular muscles. All six patients had a sensorineural hearing loss. Evidence of a mildly affected parent was found in three families. Progressive and severe facioscapulohumeral dystrophy accompanied by facial diplegia and sensorineural hearing loss may represent a separate genetic form of facioscapulohumeral dystrophy.

Child, Preschool↗

PubMed1983

Subacute sclerosing panencephalitis: an immune complex disease?

Vascular osmiophilic deposits of entangled tubular profiles were demonstrated by electronmicroscopy in the cerebral tissue of a patient with subacute sclerosing panencephalitis. Measles viral antigen, IgG, IgM, and C3 were found in the same distribution, indicating that the deposits represent immune complexes. To our knowledge, this is the first demonstration of immune complexes in the vessels of the central nervous system.

Adult↗

PubMed1983

Childhood Köhlmeier-Degos disease with atypical skin lesions.

We report clinical and pathologic findings in a 16-year-old boy whose disease began in infancy with maculopapular skin lesions, followed by cyclic nodular cutaneous eruptions, intermittent enlargement of liver and spleen, episodic abdominal pain, and sporadic unexplained fever. Subsequently, various ophthalmologic disturbances, along with a multitude of neurologic signs and symptoms, dominated the clinical picture. The CNS bore the brunt of pathologic changes, characterized by widespread leptomeningeal fibrosis, ventricular enlargement, and multiple brain infarcts. Striking intimal thickening led to narrowing or occlusion of almost all the medium-sized and small extraparenchymal arteries.

Arterial Occlusive Diseases↗

PubMed1982

Cerebral hemangioma with glial neoplasia (angioglioma?). Report of two cases.

Two patients are reported who had intracerebral mass lesions composed of hemangioma and glial neoplasm. After excision, one recurred as an oligodendroglioma, and the remnant of the other remained static over a 5-year period. These lesions may represent a subgroup of cerebral hemangiomas that have the biological potential for future glial neoplastic growth. Reference is made to experimental work with polyoma virus which can induce cavernous hemangiomas in the central nervous system in mice, and which is a papovavirus. Other papovaviruses can induce ependymomas in hamsters.

Adult↗

PubMed1981

Muscle phosphofructokinase deficiency: abnormal polysaccharide in a case of late-onset myopathy.

A 61-year-old woman with muscle phosphofructokinase (PFK) deficiency had mild limb weakness all her life but no cramps or myoglobinuria. For 5 years the limb weakness progressed. In muscle, PFK activity was 1% of normal and glycogen concentration was elevated (2.13%). By light microscopy, a minor component of the accumulated glycogen appeared as PAS-positive, diastase-resistant inclusions in 10% of muscle fibers. The inclusions had a filamentous fine structure that resembled the abnormal long-chain glycogen of brancher enzyme deficiency. Iodine absorption spectra of both the inclusions and a diastase-resistant fraction of isolated glycogen resembled amylopectin. The abnormal polysaccharide in PFK deficiency may be related to greatly elevated concentration of muscle glucose-6-phosphate, an activator of the chain-elongating enzyme glycogen synthase.

Child↗

PubMed1980

Intercostal muscle reflexes and sleep breathing patterns in the human infant.

Breathing variability and apnea characteristic of rapid eye movement (REM) sleep was investigated in a newborn infant with complete interruption of intercostal to phrenic neural pathways due to intrapartem transection of the cervical spinal cord. Breath-to-breath variability in inspiratory duration (TI), breath duration (Ttot), tidal volume (VT), and ventilation (VI) was significantly greater in REM than in quiet sleep and was similar to the variability in these parameters seen in normal infants. In addition, brief periods of diaphragmeatic apnea were observed during REM sleep. The phenomenon of shortened TI during airway occlusion previously attributed to intercostal-to-phrenic reflexes was examined in the quadriplegic infant and in seven healthy term infants. The frequency of this response was increased when airway occlusion was delayed until after onset of inspiration. Shortening of TI by occlusion occurred no less frequently in the quadriplegic than in the control infants. The constant paradoxical inward movement of the rib cage during inspiration observed in the quadriplegic infant suggests that supraspinal innervation of intercostal muscle limits such paradoxical movements in the normal infant. The quadriplegic infant's end-expiratory volume was consistently above his passive functional residual capacity, as inferred from respiratory volume and pressure measurements.

Birth Injuries↗

PubMed1979

Infantile atlantooccipital instability. The potential danger of extreme extension.

During early infancy, adventitious sliding and slipping movements between the vertebral column and skull are possible in the cadaver. In ten of 17 infants, the posterior arch of the atlas inverted through the foramen magnum during extension of the head on the atlas, resulting in the anatomic potential of bilateral vertebral artery compression. These anatomic conditions may be the basis for a chain of events that contributes to death in some neonates and infants with conventioanl diseases and may be one source of unanticipated death.

Atlanto-Occipital Joint↗

PubMedDate not supplied

Neocortical dendritic pathology in human partial epilepsy: a quantitative Golgi study.

We used a computerized image-analysis system to perform a quantitative analysis of rapid Golgi-impregnated pyramidal neurons of the third cortical layer of histologically normal cerebral cortex surgically removed from patients with partial epilepsy. Various parameters of 51 neurons from 9 patients and 29 neurons from 5 age-matched controls were compared. Dendritic spine density decreased progressively with increasing duration of seizures, and dendritic swellings were most numerous in epilepsy cases of uncertain etiology and in patients with seizures of longer standing. Neurons from seizure cases showed fewer dendritic branching points and fewer proximal dendritic branches than those from controls, suggesting a simplified dendritic architecture. These findings indicate that neurons in cortex distant from the primary site of epileptogenic activity may be undergoing subtle, progressive degeneration, which may explain the propensity of chronic epilepsy patients to have increased seizure activity and interictal behavioral and cognitive aberrations.

Adolescent↗