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Biomedical subjects

C L Dolman

Publications and source records attributed to C L Dolman.

At least 37 records · Page 2Linked to original sources
PubMed1981

Fibromatosis of dura presenting as infantile spasms.

A 6-month-old boy developed emprosthotonic infantile spasms and right hemiparesis. CT scan revealed a large mass related to a distended right temporal horn which on craniotomy proved to be a hard white tumour of the tentorium cerebelli which could be only incompletely resected. Microscopically and ultrastructurally, this lesion proved to be a typical fibromatosis containing myofibroblasts which invaded the brain. The child made an excellent recovery and is well a year after surgery. This case is another example which argues for full investigation of cases of infantile spasms which usually carry such a dismal prognosis.

Cerebellar Neoplasms↗
PubMed1981

Malignant meningioma: clinical and pathological features.

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.

Adult↗
PubMed1980

Aging of the optic nerve.

Histologic studies were carried out on 300 optic nerves covering ages from birth to 96 years. The optic nerve is small and nearly unmyelinated at birth. It rapidly grows and becomes medullated. With advancing years, the leptomeninges and fibrous septa become broader and occupy an increasingly larger proportion of the cross-sectional area of the nerve. The axons progressively diminish, and this probably reflects primarily a loss of ganglion cells and would contribute considerably to reduced visual acuity in the older population. Other age-associated degenerative changes are corpora amylacea and lipofuscin in astrocytic cytoplasm. In the older population, scars, swollen axons, and Schnabel's cavernous degeneration become common, indicating a high incidence of vascular impairment.

Adolescent↗
PubMed1980

Spinal cord damage: a rare complication of purulent meningitis.

Three cases of spinal cord damage following acute bacterial meningitis are described. Two children survived with neurological sequelae, while one died. Autopsy showed extensive spinal cord necrosis. The possible mechanisms for this rare complication are discussed and a possible connection with transient cardiorespiratory arrest is suggested.

Child, Preschool↗
PubMed1980

Lymphocytes and urine in ceroid lipofuscinosis.

Pathognomonic ultrastructural inclusions were found in the lymphocytes of 16 patients with neuronal ceroid lipofuscinosis (Batten's disease) and in the urine of three of four of such patients. These inclusions remained type specific for each patient and were identical with deposits in nerve cells and other body tissues. They persisted in lymphocytes in short-term tissue culture, but disappeared with stimulation by phytohemagglutinin. Siblings had identical inclusions. None were present in heterozygotes. Examination of lymphocytes in the peripheral blood cells is an easy method to use in the diagnosis of ceroid lipofuscinosis, but it cannot be used to detect carriers.

Adolescent↗
PubMed1979

Neoplastic angioendotheliosis. The case of the missed primary?

Two patients are described, of whom one suffered from progressive dementia, the other with a picture suggestive of Guillain-Barré syndrome. Both were found at necropsy to have small vessels throughout the body clogged with malignant cells with resultant cerebral infarcts. The source in one case was a 1-cm tumor in the thyroid, in the other a microscopic focus in the pancreas. It is suggested that most cases described as neoplastic angioendotheliosis involving the brain represent vascular dissemination of an unrecognized primary carcinoma rather than a miraculously widespread malignant endothelial transformation.

Aged↗
PubMed1979

Congenital fibrosarcoma metastatic to the choroid.

A 2 1/2-year-old boy developed a choroidal metastasis from a congenital fibrosarcoma of the lower left limb that had been amputated shortly after birth. To our knowledge this is the first reported case of a congenital fibrosarcoma that metastasized to the choroid.

Amputation, Surgical↗
PubMed1979

Progressive rubella panencephalitis: clinical course and response to 'isoprinosine'.

This report describes the clinical course of a 16-year-old Canadian-born Chinese boy who has progressive rubella panencephalitis. The progression, the lack of response to 'Isoprenosine', and the additional finding of myopathy are discussed. The clinical and pathological features of this rare, progressive neurological disorder are also summarized. In view of the last major rubella pandemic occuring in the mid-60s, it is likely that during the next few years physicians will diagnose an increased number of patients with this distinct neurological entity.

Adolescent↗
PubMed1979

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. Pathological study of two cases.

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.

Cerebral Arteries↗
PubMed1978

Necropsy of original case of Lowry's syndrome.

The necropsy findings are reported on one of the original sibs of Lowry's syndrome. The child, who died at 7 years of bronchopneumonia, was severely dwarfed, microcephalic, and microphthalmic. The brain was small and extensively calcified, the cerebellar cortex hypoplastic, and the retinae atrophic. There were multiple skeletal and integumental and minor renal abnormalities. The lymphoid tissue was much reduced, and many arteries and arterioles were thickened and narrowed. This constitutes a unique constellation of lesions.

Autopsy↗
PubMed1977

Fine structure of cutaneous nerves in ganglioside storage disease.

Skin punch biopsies of six children suffering from infantile or late onset Tay-Sachs disease, juvenile Sandhoff disease, or GM gangliosidosis type I, contained axons which, when viewed with the electron microscope, were distended by large amorphous black deposits. These are nonspecific residual bodies. Their large numbers indicate severe disturbance of the nerve cell and may be part of the dying back process. The three cases with Tay-Sachs disease had also axonal zebra or complex membranous bodies which appeared to be specific. Cytoplasmic vacuolation of other cells was a feature in the patient with GM1 gangliosidosis. Biopsies of three parents were negative.

Axons↗
PubMed1977

Progressive cerebellar ataxia, spasticity, psychomotor retardation, and hexosaminidase deficiency in a 10-year-old child: juvenile Sandhoff disease.

During the course of investigating a 10-year-old boy because of progressive deterioration of intellectual functioning, ataxia, and hemiplegia, an absence of serum hexosaminidase activity was noted. A skin biopsy examined by electron microscopy showed axonal accumulations of dense osmiophilic deposits. Because of the patient's age at onset and the slowly progressive nature of his ilness, we are reporting an atypical juvenile case of Sandhoff disease.

Age Factors↗
PubMed1977

Pineocytomas presenting as subarachnoid hemorrhage. Report of two cases.

Two cases of pineocytoma are reported in patients whose initial symptoms resembled a subarachnoid hemorrhage. The underlying tumor became obvious only later in the disease. Several episodes of subarachnoid bleeding occurred. These are the first documented cases of pineocytoma with this type of clinical presentation.

Adolescent↗
PubMed1977

Gestational development of brain.

Eighty normal human brains varying from 22 weeks' gestation to 1 month postnatal life were graded according to convolutional development and compared with the microscopical development of kidneys and the gestational age as given by the mother. Excellent correlation was obtained between the gross appearance of the brain and the microscopical appearance of the kidney. The history of the mother mostly, but not always agreed with the anatomical appearance, which was considered a reliable guide to the gestational age of the infant.

Anthropometry↗
PubMed1976

Krabbe's leukodystrophy without globoid cells.

Krabbe's infantile cerebral sclerosis with a prolonged course was present in a boy who became increasingly hypertonic during infancy and had an increased protein level in the spinal fluid. At 4 years he showed significant growth failure, profound mental retardation, spastic quadriplegia, bilateral optic atrophy, and depressed tendon reflexes. Conduction velocity in motor fibers of the median nerve had become progressively impaired. Autopsy at 5 years 10 months showed severe leukodystrophy with demyelination and gliosis. No stored breakdown products or globoid cells were seen in the brain. Galactosyl ceramide beta-galactosidase was virtually absent, and hardly any myelin was demonstrable on chemical and electron microscopic studies. The presence of globoid cells may not be essential for the pathologic diagnosis of Krabbe's leukodystrophy in the presence of appropriate enzyme deficiency.

Brain↗
PubMed1975

Hereditary mental depression and Parkinsonism with taurine deficiency.

An unusual neuropsychiatric disorder inherited in autosomal dominant fashion occurred in three successive generations of a family. Symptoms commenced late in the fifth decade in six affected patients and led to death in four to six years. The earliest and most prominent symptom was mental depression not responsive to antidepressant drugs or electroconvulsive therapy. This was accompanied by exhaustion, sleep disturbances, and marked weight loss. Later in the disease, symptoms of parkinsonism appeared, and respiratory failure occured terminally. The most recently affected family member was investigated biochemically late in his illness. Concentrations of taurine were greatly diminished in plasma and cerebrospinal fluid, and at autopsy, all regions of brain examined had a markedly reduced taurine content. Since taurine is a putative inhibitory synaptic transmitter, deficiency of brain taurine may possibly have caused the psychiatric and neurological manifestations of this disorder.

Age Factors↗
PubMed1975

Optic nerve hypoplasia with hypopituitarism. Septo-optic dysplasia with hypopituitarism.

Four children had optic nerve hypoplasia with hypopituitarism, and their clinical picture varied with age. The newborn had apnea, hypotonia, seizures, hyopglycemia, and prolong jaundice. The young infant had defective vision, behavioral delay, hypotonia, and seizures. Except for a mildly receding lower jaw and a high-arched palate, the appearance of the patients was not unusual. The fasting blood glucose level was mildly depressed. In two cases the liver was palpable and results of liver function tests were abnormal. The older child, who was blind and mentally retarded, had growth failure. The extent of the pituitary hormone deficiencies was variable, including diabetes insipidus. The septum pellucidum was not invariably absent. Clinical and pathological findings indicate that the brain lesion might be more diffuse than hitherto recognized. Early recognition of this syndrome and timely intervention might diminish serious sequels.

Abnormalities, Multiple↗