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M Blaivas

Publications and source records attributed to M Blaivas.

71 records · Page 4Linked to original sources
PubMed1993

Biopsy results in a kindred with Lafora disease.

We studied biopsy results in a kindred with the Lafora form of progressive myoclonic epilepsy. Four members of a family with known consanguinity presented as teenagers with seizures, myoclonus, dementia, and ataxia. After the diagnosis was established by brain biopsy in the first patient, many efforts were made to obtain a tissue diagnosis in the three other patients. Lafora bodies were absent in most of the skin biopsy specimens in three patients and in liver biopsy specimens from two patients. In cases of Lafora disease, where a reasonably certain clinical diagnosis can be established, supported by biopsy proof in some family members, repeated biopsy specimens even at advanced stages of the disease may be negative. These findings suggest that negative skin or liver biopsy specimens in patients with progressive myoclonic epilepsy should not exclude the diagnosis of Lafora disease.

Adolescent↗
PubMed1993

Polyneuropathy associated with IgA monoclonal gammopathy of undetermined significance.

Although polyneuropathies associated with IgM and IgG monoclonal gammopathies have been well described, polyneuropathy with IgA monoclonal gammopathy of undetermined significance (MGUS) is less commonly seen and has not been well studied. We reviewed the clinical and electrodiagnostic features of 5 such patients, and the sural nerve biopsy findings in 4 of them. One patient was diabetic, while 4 were free of other diagnoses commonly associated with neuropathy. Clinical presentations were varied. Electrodiagnostic and histological studies ranged from primary demyelination to primary axon loss to a mixed axonal/demyelinating picture. Three patients who were treated appeared to respond to prednisone or intravenous gamma globulin, despite clear clinical, electrodiagnostic, and histological differences. We conclude that the polyneuropathy associated with IgA MGUS is heterogeneous, similar to that in IgM and IgG MGUS. A trial of immunomodulating therapy appears to be warranted in such patients if the neuropathy is sufficiently severe.

Adult↗
PubMed1992

Ligamentum flavum hematoma. Report of two cases.

Two patients presenting with signs and symptoms suggestive of nerve root compression secondary to extradural masses were found to have ligamentum flavum hematomas. Both patients had neurological deficits preoperatively and regained normal function postoperatively. There was no significant antecedent injury in either case. The symptom course was longer than that for spontaneous epidural hematoma. In one case, there was remodeling of bone, initially suggesting either infection or tumor.

Adult↗
PubMed1991

Muscle fiber branching--difference between grafts in old and young rats.

Large numbers of branched muscle fibers occur in the freely grafted rat extensor digitorum longus muscle. The ratio of branched/non-branched muscle fibers in grafts is much higher in old (24 months) than in young (4 months) host rats. Cross-age transplants show that the proportion of branched muscle fibers is related to the age of the grafted muscle and not to the age of the host. This is in contrast to mass and maximum isometric tension, in which the age of the host, rather than the age of the grafted muscle, is the determinant of the success of the muscle graft.

Aging↗
PubMed1991

Acute pandysautonomic neuropathy.

Acute pandysautonomic neuropathy is characterized by severe postganglionic sympathetic and parasympathetic dysfunction, with relative or complete sparing of motor and sensory function. Of four reported cases with sural nerve biopsies, two were normal and two abnormal, revealing loss of small myelinated and unmyelinated fibers. We present a patient with pandysautonomic neuropathy and elevated CSF protein whose sural nerve biopsy showed active axonal degeneration.

Acute Disease↗
PubMed1991

Orbicularis oculi muscle in children. Histologic and histochemical characteristics.

This is the first study devoted to the histologic and histochemical characteristics of the orbicularis oculi muscle in children to the authors' knowledge. The orbicularis muscle was compared with extraocular, facial, and limb striated muscle. Light microscopy showed the orbicularis oculi muscle to be much smaller and more loosely packed than skeletal limb muscles. It further showed these muscle fibers to have greater variation in fiber size and shape and more endomysial and perimysial connective tissue. Finally, analysis of the histochemical reactions showed the orbicularis oculi had a higher percentage of fast-contracting fibers (Type II). This study establishes the histologic and histochemical standard characteristics for the orbicularis oculi muscle in children. It was found that orbicularis oculi muscles have some histologic and histochemical features in common with other facial muscles and other features in common with extraocular muscles.

AMP Deaminase↗
PubMed1990

Circadian rhythms of human pineal melatonin, related indoles, and beta adrenoreceptors: post-mortem evaluation.

Human pineal glands obtained from 77 post-mortem sources from various age groups and times of death were used to examine the 24-hour cycle of serotonin (5-HT), melatonin, N-acetylserotonin (NAS), and beta adrenoceptor density. Pineal glands were divided sagittally and a single half was used to measure 5-HT, NAS, and melatonin concentrations, while the remaining half from the same gland was employed to assess changes in the density of beta adrenoceptors on partially purified membranes. The results show that density of pineal beta adrenoceptors was relatively constant between midnight and 18.00 h and became significantly higher between 18.00 and 20.00 h as measured by ligand saturation binding experiments using (125-I) iodocyanopindolol. The receptor affinity of all of the samples assayed remained in relatively narrow range near 58 pM and only changes in the relative receptor density were apparent. The up-regulation of receptors coincided with an increase in the concentration of 5-HT that began to rise between 16.00 and 20.00 h and became maximal between 20.00 and midnight. NAS, the immediate precursor of melatonin, was also at maximal levels between 20.00 h and midnight. Both 5-HT and NAS began declining after midnight and this change corresponded to the maximal pineal gland concentration of melatonin between midnight and 4.00 h. It is therefore suggested that the up-regulation of beta adrenoceptors noted during the late afternoon and early evening hours corresponds to the increased synthesis of 5-HT and the subsequent conversion to NAS. These events are followed by the highest accumulation of melatonin after midnight and represent the synthesis of melatonin from its precursor NAS in a sequential pattern.

Adolescent↗
PubMed1989

Electrodiagnostic evolution of carcinomatous sensory neuronopathy.

Sensory neuronopathy is a well-recognized remote effect of carcinoma. We report the clinical and electrodiagnostic evolution of a sensory neuronopathy in a patient with carcinoma of the lung. Serial electrophysiologic studies suggest transformation from normal peripheral nerve function through early posterior root involvement to absent sensory nerve function. Diffuse motor conduction abnormalities occurred late in the disease, perhaps reflecting motor axon changes associated with disuse.

Carcinoma, Small Cell↗
PubMed1989

McArdle's disease presenting as treatment resistant polymyositis.

The broad spectrum and often incomplete presentation of polymyositis frequently confound its distinction from other myopathies. In 18 months of screening for myophosphorylase in all muscle biopsy specimens at our institution, 2 cases of McArdle's disease were discovered in patients previously thought to have refractory PM. Given the important treatment implications of this distinction, all patients with "refractory PM" in whom the diagnosis has not been firmly established should be screened for myophosphorylase deficiency.

Adult↗
PubMed1985

Distribution of chromogranin and S100 protein in normal and abnormal adrenal medullary tissues.

The distribution of chromogranin and S100 protein was studied in 30 adrenal pheochromocytomas and 19 normal adrenal medullary tissues. Immunostaining in the tumors was compared with staining in sections of histologically normal medullae. Chromogranin showed diffuse cytoplasmic staining in all chromaffin cells. Chromogranin staining was consistently more intense in normal medullae, while less intense staining was present in most tumors from all four groups. S100 protein was present in the cytoplasm and nuclei of sustentacular cells surrounding chromaffin cells and in nerve branches. Many S100 protein-positive cells were present in normal medullae, in the two hyperplastic medullae, and in pheochromocytomas from patients with multiple endocrine neoplasia, type 2. Very few sustentacular cells were present in the other pheochromocytomas. These results indicate that S100 staining may be helpful in separating pheochromocytomas in patients with multiple endocrine neoplasia, type 2 disease from benign and malignant sporadic tumors.

Adrenal Medulla↗
PubMed1985

Detection of HLA-DR antigens in paraffin-embedded thyroid epithelial cells with a monoclonal antibody.

The human Class II major histocompatibility (MHC) antigens, or Ia antigens, which are thought to regulate immune cell interaction, can be detected in paraffin-embedded tissues by immunoperoxidase staining with a recently developed monoclonal antibody (LK8D3). HLA-DR antigens were observed in lymphoid tissues, Langerhans cells of the skin, some epithelial cells, and pulmonary alveolar macrophages. The expression of HLA-DR antigens was analyzed in formalin-paraffin sections by immunoperoxidase in 86 normal and abnormal thyroid epithelial tissues. All patients with Hashimoto's disease (8/8) and most patients with Graves' disease (6/8) expressed HLA/DR antigens in the thyroid epithelial cells and in adjacent inflammatory cells. Most papillary carcinomas (12/18), including 3 of 5 follicular variant of papillary thyroid carcinomas, had HLA-DR antigens detected in epithelial cells; whereas medullary thyroid carcinomas (0/5), follicular carcinomas (0/5), and multinodular goiters (0/4) did not have detectable HLA-DR immunoreactivity. A few other thyroid lesions had HLA-DR antigens detected in epithelial cells, including anaplastic carcinomas (2/5), Hurthle-cell tumors (1/16), and thyroid lymphomas (2/2). Monoclonal antibody LK8D3 and two other commercially available monoclonal antibodies against HLA-DR-stained tissues equally well in cryostat sections, but only antibody LK8D3 was effective in formalin-fixed paraffin-embedded tissue sections. These results indicate that epithelial cells from thyroids of patients with autoimmune diseases commonly express HLA-DR antigens. The presence of HLA-DR antigens in most papillary thyroid carcinomas may be helpful diagnostically in cases of follicular variants of papillary carcinomas. The role of HLA-DR expression in autoimmune thyroid disease and in papillary thyroid carcinoma remains to be determined.

Antibodies, Monoclonal↗
PubMedDate not supplied

Pediatric macrophagic myofasciitis associated with motor delay.

BACKGROUND: Macrophagic myofasciitis (MMF) is a rare inflammatory myopathy characterized by accumulation of perifascicular macrophages without muscle fiber necrosis. Few sporadic pediatric cases have been described, and MMF is recognized as a possible reaction to intramuscular injections of aluminum-containing vaccines. The association of MMF and motor delay is unclear in the pediatric population. We report the clinical evaluation and follow-up of 4 young children with MMF and review of 4 cases previously reported of sporadic, pediatric MMF to better determine the possible association of sporadic MMF in children presenting with motor delay. PATIENTS AND METHODS: Described our 4 case reports in which we observed children presenting for evaluation of motor delay with unrevealing clinical and laboratory evaluations for common causes of motor delay and histopathological evaluations consistent with macrophagic myofasciitis. Muscle data was obtained by quadriceps muscle biopsy. RESULTS: Clinical presentations were similar in all children and were characterized by motor delay, hypotonia, and failure to thrive with an unrevealing evaluation for central nervous system disease, congenital, and mitochondrial myopathies. CONCLUSIONS: Our cases and those previously reported in the literature demonstrate MMF should be considered in the evaluation of children with failure to thrive, hypotonia, and muscle weakness, as clinical outcome appears to be favorable.

Biopsy↗
PubMedDate not supplied

Cerebellopontine angle invasive papillary cystadenoma of endolymphatic sac origin with temporal bone involvement.

The authors report the MR, CT, and pathologic findings in a case of invasive papillary cystadenoma originating in the endolymphatic sac and involving the temporal bone. This case illustrates characteristic imaging features of this lesion. The authors emphasize awareness of this entity with its aggressive temporal bone involvement as an aid to pathologic differential diagnosis.

Adenocarcinoma↗