Biomedical subjects

M D Winkelman

Publications and source records attributed to M D Winkelman.

9 recordsLinked to original sources

PubMed1992

Thalamic amnesia: Korsakoff syndrome due to left thalamic infarction.

In order to support the concept that a lesion of the thalamus is sufficient to cause a Korsakoff syndrome, we are presenting 5 patients, all of whom developed the syndrome after sustaining a left (dominant) thalamic infarction. Two patients had pure thalamic strokes followed by a permanent Korsakoff syndrome. One of these patients was studied with neuropsychometric testing, as well as with a modern MRI scan. In 2 other patients, clinical and imaging data indicate that infarction was not limited to the thalamus. Another patient had bilateral thalamic infarcts but only a temporary Korsakoff syndrome. Neuropathological data are needed to elucidate the exact anatomical substrate of dominant thalamic Korsakoff syndrome.

Aged↗

PubMed1992

Central nervous system complications of thermal burns. A postmortem study of 139 patients.

We report a retrospective, clinicopathologic study of 139 patients who died during treatment of a severe burn. Fifty-three percent of the patients had central nervous system (CNS) complications-infections, cerebral infarcts and hemorrhages, metabolic encephalopathies, central pontine myelinolysis, and cerebral trauma. Children and adults were equally affected. Sixteen percent of the patients had a CNS infection. Candida species, Staphylococcus aureus and Pseudomonas aeruginosa caused almost 80% of them. S. aureus and candida caused cerebral microabscesses and septic infarcts. P. aeruginosa caused meningitis and infarcts due to meningitis. CNS infections arose as a result of spread from a systemic source. The major risk factors for CNS infection were an extensive burn, S. aureus endocarditis, and a burn wound infection due to candida or P. aeruginosa. Patients with burns of less than 30% of the surface area of their body, those without a systemic infection, and those in the first week after their burn were at low risk. Eighteen percent of the patients had cerebral infarcts. In almost half the patients, the infarcts were caused by septic arterial occlusions or other complications of the burn, viz, disseminated intravascular coagulation (DIC) and septic shock. In only one-third of the patients were infarcts due to atherosclerosis, atrial fibrillation, or other causes prevalent in the general population. Intracranial hemorrhages were only one-fifth as frequent as infarcts and were due to DIC and thrombocytopenia, caused by bacteremia. Diagnosis during life was difficult, because the neurologic picture of focal cerebral lesions and meningitis was indistinguishable from that of metabolic encephalopathies, and because many patients had more than 1 neurologic complication. However, our results suggest that a clinical approach that includes analysis of risk factors for CNS infection, cerebral imaging, examination of cerebrospinal fluid, and tests for DIC can lead to a neurologic and microbiologic diagnosis in most patients.

Adolescent↗

PubMed1988

Central pontine myelinolysis in severely burned patients: relationship to serum hyperosmolality.

The rapid correction or over-correction of hyponatremia is believed by many to be the crucial factor in the causation of central pontine myelinolysis (CPM). Over a 17-year period we found CPM in 10 (7%) of the 139 burn patients examined postmortem but in only 10 (0.28%) of the 3,528 patients in the general autopsy population (p less than 0.001). Each of the burn patients with CPM had suffered a prolonged, nonterminal episode of extreme serum hyperosmolality, whereas most burn patients without CPM had not suffered such an episode. The histologic age of the lesions correlated with the duration of time between the hyperosmolar episode and death. Hypernatremia, hyperglycemia, and azotemia, alone or combined, accounted for the hyperosmolality. No single electrolyte or metabolic derangement was essential, as in at least one burn patient with CPM the serum sodium, glucose, or blood urea nitrogen was normal during the hyperosmolar episode. Hyponatremia was not present in any burn patient with CPM. We conclude that severely burned patients, like alcoholics, are especially susceptible to CPM, and that in burn patients with CPM there is a striking association with serum hyperosmolality. The data also suggest that the rapid correction of hyponatremia exerts its effects by causing an osmotic shift and not because of any specific property of the sodium ion.

Adolescent↗

PubMed1987

Intramedullary spinal cord metastasis. Diagnostic and therapeutic considerations.

The diagnosis of intramedullary spinal cord metastasis (ISM) is difficult, and treatment is usually ineffective. We review our own experience with ISM as well as the pertinent medical literature, and suggest a practical diagnostic and therapeutic approach. The problem of the diagnosis of ISM is essentially that of the differential diagnosis of a noncompressive myelopathy in a patient with systemic cancer. Most such patients prove to have ISM, meningeal carcinomatosis, radiation myelopathy, or paraneoplastic necrotizing myelopathy. Neurologic features of value in this differential diagnosis are pain, the tempo and mode of progression of symptoms, and tumor cells in the spinal fluid. Oncologic features of value are the location of the primary tumor, the past exposure to therapeutic radiation, cerebral metastases, and the extent of systemic metastatic disease. The myelogram in ISM is either normal or nonspecifically abnormal; therefore, the diagnosis must be made on clinical grounds. Although no single finding is diagnostic of ISM, a careful clinical analysis will lead to the correct diagnosis in most cases. Radiation therapy is effective treatment for ISM, but only if it is administered early, before paraplegia supervenes. Thus, the diagnosis should be made and treatment begun as soon as possible. Intramedullary spinal cord metastasis is often multifocal rather than solitary; therefore, whole-cord rather than local spinal radiation should be given, if possible. If local radiotherapy is chosen, the construction of the portal can be based on the myelogram or, in the event of a normal study, on the clinical localization of the tumor.

Diagnosis, Differential↗

PubMed1986

Dialysis encephalopathy: neuropathologic aspects.

The clinical, epidemiologic, electroencephalographic, and toxicologic features of dialysis encephalopathy (DE) have been clarified, but the neuropathologic and clinicopathologic aspects of the disease have remained obscure. In three cases of DE associated with a dialysate of high aluminum content, spongy change restricted to the upper layers of the cerebral cortex was found. The spongy change was distributed through the cerebral cortex bilaterally and diffusely, but the left hemisphere was involved more severely and extensively than the right hemisphere, and the opercular portions of the frontal and temporal lobes were affected more than the remainder of those lobes. This topographic distribution correlated with the clinical defects in higher cortical functions, including aphasia. The spongy change consisted of vacuoles in the neuropil and inside nerve cell bodies. The vacuoles in the neuropil were located in dendrites, astrocytic processes, and, possibly, other structures. Thus, DE is a disease of neurons and astrocytes of the cerebral cortex. Although spongy change of the upper layers of the cerebral cortex is a nonspecific abnormality, when it occurs in the distribution described and in the absence of other diffuse cerebral diseases, it is characteristic of DE.

Adult↗

PubMed1985

Intramedullary spinal cord metastasis. A clinicopathological study of 13 cases.

A retrospective autopsy study of 627 patients with systemic cancer disclosed 153 patients with metastasis to the central nervous system (CNS) and 13 patients with intramedullary spinal cord metastasis (ISM). Thus, the frequency of ISM was 8.5% of cases of metastasis to the CNS and 2.1% of all cases of cancer. Bronchogenic carcinoma accounted for 11 cases of ISM, and breast carcinoma and melanoma for the other two. There were two distinct patterns of spinal cord involvement, indicating spread of tumor to the cord by two different routes. In nine of the 13 ISM patients a metastasis was found deep within the spinal cord, unassociated with leptomeningeal carcinomatosis; this most likely resulted from hematogenous spread of tumor from a pulmonary source. In the other four patients there was focal or multifocal direct extension of leptomeningeal metastatic tumor across the pia into the parenchyma of the cord. Only four of the 13 patients had a clinical myelopathy; in three of these four this was the presenting feature of an occult lung cancer.

Aged↗

PubMed1984

Unilateral right cerebral representation of reading in a familial left-hander.

Several recent hypotheses of cerebral functional organization in natural left-handers hold that language, including reading, is localized either unilaterally in the left hemisphere or bilaterally, but not unilaterally in the right hemisphere. A case of alexia without agraphia in a left-hander with an infarct in the right occipital lobe and splenium of the corpus callosum is reported. That a permanent and complete alexia resulted from a single, right hemisphere lesion indicated that reading was completely lateralized to that hemisphere. The case demonstrates that in some natural left-handers language functions can be unilaterally represented in the right hemisphere.

Aged↗

PubMed1983

Cerebellar degeneration caused by high-dose cytosine arabinoside: a clinicopathological study.

Twenty-four patients with leukemia or lymphoma refractory to conventional chemotherapy were given a course of systemic, high-dose cytosine arabinoside (3 gm/m2 every 12 hours for twelve doses). Four patients developed cerebellar degeneration during treatment. Ataxia of gait and limb movements, dysarthria, and nystagmus appeared five to seven days after the first dose, worsened over the next two to three days, and then remained stable for two to six days. Incomplete improvement occurred over the following one to two weeks. Postmortem examination disclosed loss of Purkinje cells in the depths of cortical sulci with relative preservation of those at the crests of folia and those in the most posterior inferior portions of the cerebellum. Other patients developed a mild, reversible cerebellar syndrome over the same time course as that of the irreversible disorder. Manifestations ranged from nystagmus alone to dysarthria and unsteadiness of gait without limb ataxia. We conclude that cytosine arabinoside at this dosage causes a cerebellar degeneration with characteristic clinical and pathological features. Among the present patients with refractory hematological malignancies, such degeneration occurred with an incidence of 16.7%, more than twice that reported in previous series.

Acute Disease↗

PubMed1983

Non-infantile neuronopathic Gaucher's disease: a clinicopathologic study.

Two siblings with Gaucher's disease developed a chronic, slowly progressive neurologic disorder in early adult life. Stimulus-sensitive myoclonus, generalized seizures, supranuclear gaze palsies, and cerebellar ataxia were the main clinical features. Autopsy disclosed perivascular Gaucher cells in the brain, and nerve cell loss and neuronophagia in the brainstem, cerebellum, and spinal cord. From these observations and the literature, there seem to be three clinically distinct neurologic syndromes in Gaucher's disease, depending on the age at which symptoms begin, but the neuropathologic changes are essentially the same in all of them. A modified classification of Gaucher's disease, based on these observations, is proposed.

Adult↗