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Biomedical subjects

U Roessmann

Publications and source records attributed to U Roessmann.

15 recordsLinked to original sources

Agenesis of the corpus callosum: a study of the frequency of associated malformations.

Review of 11 cases of agenesis of the corpus callosum studied at our institution revealed a high incidence of associated anomalies. None patients had associated malformations of the central nervous system, 6 involving the pyramidal system. Eight cases were associated with malformations in the rest of the body. Review of completely examined cases from the literature yielded 47 examples of associated malformations. These were varied and without consistent pattern, with the possible exception of facial abnormalities.

Adolescent

Apert's syndrome with central nervous system anomalies.

The pathologic anatomy of the central nervous system is described in a patient with Apert's syndrome. Multiple developmental anomalies of the brain were noted including disturbances of rhinencephalic organization. The association between maldevelopment of the rhinencephalon and the face is well known. Cranial vault malformations may also be associated with maldevelopment of the rhinencephalon.

Acrocephalosyndactylia

Vermian hematoma in a four-year-old child.

An unusual case of a vermian hematoma from the rupture of an angioma in a four-year-old child is presented. The lesion was treated successfully with complete recovery.

Cerebellar Neoplasms

Hydranencephaly in vertebral-basilar territory.

A case is presented of almost complete destruction of the cerebellum secondary to a hemorrhagic event in utero. Lesions consistent with hydranencephaly were found in the territories of the vertebral-basilar circulation. Ependymitis and aqueduct occlusion secondary to the intraventricular bleeding resulted in intrauterine hydrocephalus formation.

Anencephaly

Kearns-Sayre syndrome with hypoparathyroidism.

The history, clinical findings, and postmortem examination of a patient with Kearns-Sayre syndrome are presented. In addition to the usuual symptoms, the patient also had hypoparathyroidism. There is increasing evidence that the syndrome is frequently associated with involvement ofthe endocrine system. Central nervous system involvement consists of a spongy encephalopathy.

Brain

A morphologic study of intracerebral hemorrhage in a case of acute leukemia.

Morphologic studies have thus far failed to demonstrate the nature of the vessel involved in the brain hemorrhages of patients with acute leukemia. A detailed study of such hemorrhages was carried out in a patient with leukemic phase of mycosis fungoides. Plastic-embedded lesions showed that blast cells blocked the lumen of the capillary, leading to severe dilation and rupture of the vessel. The rheologic properties of blast cells in vessels of critical diameters seem to be an important factor in the pathogenesis of intracerebral hemorrhages.

Acute Disease

Subependymoma: a case report with ultrastructural study.

A case history illustrating the potential clinical significance of subependymoma is presented. Fine structural studies indicate that the tumor is composed of cells having the cytoplasmic features of ependyma, astrocytes, and transitional cells. Its composition and structure are alike those in the adult mammalian subependymal layer.

Aged

Lower motor neuron disease with spinocerebellar degeneration.

A patient with polymyositis responded initially to steroid therapy. A muscle biopsy disclosed features of primary myopathy and group atrophy. The patient became refractory to therapy and died with relentlessly progressive weakness. The autopsy disclosed lower motor neuron involvement and degeneration of the spinocerebellar tracts. There was loss of Purkinje cells, which may have occurred secondary to an anoxic episode prior to death. The case is unique because of the limited involvement of the lower motor and spinocerebellar systems.

Cerebellum

Diffuse "anoxic" myelopathy.

Pathologic changes and distribution of lesions of the spinal cord were studied in 16 patients who suffered from "anoxic" episodes. The lesions were symmetrical and limited to the gray matter. The vulnerability of the spinal cord was most marked in the lumbosacral region, although almost every nucleus throughout the spinal cord was subject to damage.

Adolescent

Pathogenesis of central nervous system infiltration in acute leukemia.

The distribution pattern of leukemic infiltrates was studied in 31 cases of acute leukemia with CNS involvement. Dura mater involvement was found in 93% (29/31) of the cases; arachnoid, 71% (22/31); perivascular cuffing, 37% (17/31); parenchymatous, 16% (5/31). Dura matter infiltrates were the sole manifestation in nine cases; Infiltration of the arachnoid in the absence of dural infiltration was rare (9%). The anatomic evidence supports the concept that leukemic cells infiltrate by way of perivenous adventitial tissue connecting the dura mater and subarachnoid space. It is likely that this pathway leads directly from the bones of the skull into the brain parenchyma.

Brain

Chronic tonsillar herniation: an attempt at classifying chronic hernitations at the foramen magnum.

A system is presented for the classification of chronic herniations of the cerebellar tonsils in the absence of space-occupying intracranial lesions, based on a survey of the literature and 13 own cases. The Arnold-Chiari malformation in adults typically involves herniation of the cerebellar tonsils instead of herniation of the vermis as is typical when it occurs in infancy. Identification of chronic tonsillar herniation with the Arnold-Chiari malformation in adults was thought to require at least one other sign of the Arnold-Chiari complex, e.g. a medullary deformity. Cases for which chronic herniation and sclerosis of the cerebellar tonsils present as the only nervous lesion are classified as "chronic tonsillar herniation". Such cases may manifest with neurological symptoms during adult life, or they may be found incidentally at autopsy. Attention is drawn to the occurrence of chronic tonsillar herniation in 7 infants and children where it apparently represents a cause of sudden unexpected death. The overlap between chronic tonsillar herniation and the Arnold-Chiari malformation of adults is discussed in regard to the frequence of associated osseous anomalies, hydrocephalus and syringomyelia.

Adolescent

Massive intracerebellar hemorrhage in low-birth-weight infants.

The clinical and pathologic observations of massive intracerebellar hemorrhage (destruction of at least one-third of cerebellar tissue) are described in six low-birth-weight infants. In all infants, severe progressive apnea associated with a falling hematocrit were the prominent clinical features. Four infants were asphyxiated at birth. Some degree of cerebellar hemorrhage (macroscopic or microscopic) was observed in 21% of 157 newborn brains examined at autopsy. The cause of massive intracerebellar hemorrhage is unknown, but may result from deforming pressures on the skull secondary to perinatal trauma. A close follow-up of cerebellar function in low-birth-weight infants is important.

Birth Weight

Arterial dysplasia with ruptured basilar artery aneurysm: report of a case.

An 11 year old girl died unexpectedly as a result of spontaneous subarachnoid hemorrhage originating from a ruptured giant fusiform aneurysm of the basilar artery. The aneurysm had developed as a long standing complication of arterial fibromuscular dysplasia. This discussion describes the light and electron microscopic findings in the aneurysm and the histologic examination of abnormalities in the muscular arteries of the viscera.

Aneurysm