Search PubMed⌕ Search

Biomedical subjects

W Grisold

Publications and source records attributed to W Grisold.

107 records · Page 6Linked to original sources

The syndrome of continuous muscle fibre activity following gold therapy.

A 72-year-old man suffering from arthritis received a total dose of 500 mg sodium aurothiomalate during a period of 5 months. His clinical state then deteriorated and he had to be hospitalized. Upon admission he was bedridden, his level of consciousness was slightly impaired, he was confused and respiration was laboured. Continuous muscle activity was noted on all extremities and at first, erroneously, fasciculations were diagnosed. The EMG exhibited continuous muscle fibre activity consisting of duplets, triplets and multiplets. The discharges occurred in an irregular pattern; when various muscles were examined at the same time no synchronicity could be observed between muscle discharges. In the left m. deltoideus an increased percentage of polyphasic potentials was found, whereas mean duration of motor unit potentials was normal. Spontaneous activity remained unchanged during sleep and administration of intravenous diazepam or phenytoin. Blocking of ulnar nerve at either elbow or wrist level did not stop spontaneous activity in m. abductor digiti quinti. Ischaemia increased the amount of discharges after 7 min. Within 4 months after termination of gold therapy the patient's condition improved and he was discharged from hospital. Regular EMG follow-up after 8 months showed complete cessation of abnormal spontaneous activities. Nerve conduction velocities were normal except for markedly reduced compound action potential in peroneal nerves. Continuous muscle fibre activity as a side-effect of gold therapy is described.

Aged↗

Metastasis of solid tumors in extraocular muscles.

Three autopsy cases with discrete metastatic involvement of one or several extraocular orbital muscles by disseminated amelanotic melanoma (one case) and lobular mammary adenocarcinoma (two cases) associated with extensive meningeal involvement are reported. Clinical ocular symptoms including pain, exophthalamus, and diplopia occurred 6 months to almost 5 years after resection of the primary tumor; in two cases CT scan showed spindle-like enlargement of orbital muscles. Pathologic examination disclosed solid localized metastatic deposits in several extraocular muscles of one (breast carcinomas) or both orbits (melanoma), with diffuse invasion of striated muscle, but without necrosis, inflammation, or involvement of other orbital adnexa, eye ball, optic nerves, or orbital bone. Since no continuous invasion of orbital or intraocular structures by diffuse meningeal blastomatosis was histologically observed, rare metastatic involvement of extraocular muscles via hematogenic route is suggested.

Breast Neoplasms↗

[Combined treatment of malignant gliomas].

A controlled study of 226 age-matched patients with histologically proven grade 3 and 4 supratentorial gliomas with maximum feasible tumour resection, postoperative Karnofsky performance over 50 and minimum survival of 8 weeks compares the results of supportive care (45 cases), high-dose irradiation of 40 to 66 Gy (59 cases), COMP protocol (CCNU, procarbazine, vincristine, methotrexate, prednisone in 15 day cycles-42 cases) and simultaneous irradiation and COMP chemotherapy (80 cases including 30 survivors). Median recurrent-free intervals in the treatment groups (7 to 11.7 months) were significantly longer than after supportive care (4.4 months). Median survival with supportive care (6.7 months) was significantly shorter than after radiation or COMP treatment (11.7 and 12.3 months) and 14.9 to over 19.9 months with combined treatment, where the two-year survival rates were 33 and 67% (for survivors), and the 3-year survival rates 13 to 30%. Toxic side effects of multimodality treatment were more frequent than after chemotherapy. In addition to space-occupying intracranial cysts often simulating tumour recurrence (12%) and rare radiation necrosis, about 15% of long-term survivors developed progressive intellectual dysfunction with brain atrophy, in the absence of tumour regrowth. Despite some promising results of multimodality approaches towards the management of malignant supratentorial gliomas, the overall results are unsatisfactory and need further optimization.

Adult↗

Correlation between EEG changes indicative of sedation and subjective responses.

The central activity of ketotifen ( Zaditen ), a benzocycloheptathiophene derivative for use in the prophylaxis of asthma, was determined by quantitative pharmaco-EEG in 7 healthy volunteers in a single-blind trial. During the 1st week of the trial, placebo was given twice daily followed by ketotifen 1 mg twice daily for 3 weeks. Placebo was again given for a further week. 15-min resting EEGs were taken immediately before and 3 and 6 h after medication on 8 defined days during the study, and the subjects were asked for side effects. Lead O2-Cz was analyzed by spectral analysis, and the relative power of the delta, theta, and fast and slow alpha bands as well as the dominant alpha frequency were calculated. The mean of each of these parameters was calculated per subject for each of the three measurements on each study day and compared with the baseline by means of one-way analysis of variance. A statistically significant slowing of the dominant alpha frequency, a decrease of the relative power of the fast alpha activity, and an increase of the relative power of the theta rhythm were found. These effects, indicative of a mild sedation, were highest during the 1st week of treatment with ketotifen, with a peak at the 3rd day, and gradually decreased thereafter. In contrast to the sensitive pharmaco-EEG method, none of the subjects complained of sedation or tiredness while taking ketotifen.

Adult↗

Cerebral atrophy in Parkinson syndrome.

Review of 100 autopsy cases of Parkinson disease (PD), 100 age and sex matched controls, 16 cases of senile Parkinson (sP), 85 cases of senile dementia of Alzheimer type (SDAT) and 55 cases of Alzheimer disease (AD) showed that the average brain weight in PD was not significantly less than in controls but was much higher than in sP and SDAT. PD shows only slight but insignificant increase of cortical neuronal loss and Alzheimer lesions (AL) as compared to controls, while in PD the AL are significantly less severe than in sP, SDAT and AD. There is no increased simultaneous occurrence of PD and AD. Several types of PD are separated: PD with dementia with a) subcortical PD lesions combined with AD or severe AL, b) severe AL with little nigral damage; c) severe AL with cortical Lewy bodies; PD without dementia with no or little AL. Demented PD patients with severe AL are older than non-demented ones without AL. In general, there is good correlation between the degree of dementia and the severity of AL in both PD and controls.

Age Factors↗

Chronic encephalitis in X-linked agammaglobulinaemia.

Clinicopathological and immunological findings are reported in a boy with X linked agammaglobulinaemia (XLA), who after multiple episodes of purulent infections in various organs developed encephalitis associated with excemata and non suppurative arthritis. Inspite of gammaglobulin administration he developed progressive spastic tetraparesis, convulsions, mental deterioration and blindness and died in a decerebrate state at the age of 8 years. Necropsy revealed a general diminuition of the lymphoid tissue. The atrophic brain showed multiple cystic destructions and chronic, but still active polioencephalitis with prominent inflammatory involvement of basal ganglia and brain stem. Symmetric Wernicke-like lesions were seen in inferior corpora quadrigemina. The aetiology of this particular type of chronic progressive encephalitis in XLA is unknown but some kind of viral infection is to be considered.

Agammaglobulinemia↗

Giant-cell glioblastoma of the thoracic cord.

A case of giant-cell glioblastoma occurring in the upper thoracic cord of a male aged 76 without evidence of intracranial lesion is reported. Partial tumour resection with decompression and radiotherapy did not improve the patient's neurological deficit.

Aged↗

Necrotizing myelopathy associated with acute lymphoblastic leukemia. Case report and review of literature.

The clinico-pathological findings are reported of a 16-year-old boy with acute lymphoblastic leukemia who during chemotherapy which included intrathecal Methotrexate developed a transverse cord lesion 5 months before death. Autopsy revealed necrotizing myelopathy of the T8--T10 segments and moderate lymphoblastic infiltration of the cerebrospinal meninges and spinal nerve roots. Neither parenchymal infiltration nor vascular lesions were found. The pathogenesis of this rare lesion associated with acute lymphoblastic leukemia remains obscure.

Adolescent↗

[Articulation disorder as the initial manifestation of facioscapulohumeral muscular dystrophy in childhood].

A five year old boy who had received logopedic treatment for more than two years was seen as an outpatient because of speech retardation. He presented with myopathic face, incomplete closure of both lids and severe weakness of facial muscles, bilateral winging of scapulae and hyperlordosis. Extraocular and pharyngeal muscles were not affected. Motor and sensory nerve fibre conductions and electromyography were within normal limits. CPK was moderately elevated (320 U/I). Muscle biopsy of right deltoid muscle revealed unspecific myopathic changes. The patients brother aged 7 also presented with facial weakness, elevated CPK and neurogenic changes in EMG of deltoid muscle. Both parents were clinically and electrophysiologically unremarkable. Although problems to speak distinctly are usually not the first manifestation, we found in this family facio-scapulo-humeral muscular dystrophy.

Arm↗

[Comparison of the acetylcholine receptor antibody titer and clinical course in neonatal myasthenia].

Ten to fifteen percent of infants of myasthenic mothers develop neonatal myasthenia independent of the severity of the maternal disease. The antiacetylcholin receptor-protein antibodytiter (AChR-AK) was followed through the first twelve months in a boy who's mother suffered from myasthenia gravis (M.G.) since twelve years. Only unspecific symptoms for an impaired neuromuscular transmission were found, the diagnosis however was confirmed by determination of AChR-AK (alpha-Bungarotoxin binding assay, Lindström 1979). Maternal AChR-AK did not change during the observation period: values were between 120 X 10(-9)mol/l and 140 X 10(-9)mol/l. The neonatal titer on day one was 100 X 10(-9) mol/l, somewhat below the corresponding maternal titer (120 X 10(-9)mol/l). By 7 months the AchR-AK had decreased to the reference value according to the half time for IgG (t/2 = 17 days). In contrast to Keesey and Donaldson we found no relationship between AChR-AK levels and clinical course.

Adult↗

New phenotype of adult alpha-L-iduronidase deficiency (mucopolysaccharidosis I) masquerading as Friedreich's ataxia with cardiopathy.

Clinical, ultrastructural and biochemical studies are reported in a 42-year-old woman presenting with congenital pes cavus who, at the age of 23 years, developed slowly progressive distal amyotrophies, hypesthesia, bilateral hearing loss and severe cardiopathy leading to death. There were skeletal anomalies, mild reduction of motor NCVs, but no corneal opacity, retinitis pigmentosa, organomegaly or vacuolated lymphocytes. Autopsy disclosed severe thickening of fibrous tissues (endocardium, cerebrospinal dura) with accumulation of vacuolated cells containing glycosaminoglycans in numerous membrane-bound cytoplasmic vacuoles, and/or compound multilamellar or zebra-body-like structures. The CNS, in addition to enlarged perivascular lacunes in cerebral white matter with lipid-containing macrophages, showed neuronal lipid storage in thalamus, hypothalamus, hippocampus, brain stem nuclei, spinal motor neurons and Purkinje cell dendrites. Ultrastructurally, lamellated inclusions containing gangliosides were seen in mesenchymal cells, oligodendrocytes, pericytes and Schwann cells. Neurons contained abundant ceroid but no lamellated inclusions. Neurochemistry revealed decrease of alpha-L-iduronidase activity in brain tissue to 4% of normal, normal activities of other lysosomal enzymes, and normal lipid and ganglioside patterns. While the morphology and neurochemistry data are characteristic of mucopolysaccharidosis I, the phenotype of adult alpha-L-iduronidase deficiency mimicking Friedreich's disease has not been described so far.

Adult↗

Human neurolymphomatosis in a patient with chronic lymphatic leukemia.

A 62-year-old woman with chronic lymphatic leukemia (CLL) (RAI stage IV) with multiple organ involvement and diabetes mellitus, three months prior to death presented with a symmetrical sensory neuropathy of the upper extremities with little motor impairment and, two months later, sensory atactic neuropathy of the lower limbs. No cranial nerve or CNS impairment was noted. Clinical diagnosis was predominantly sensory neuropathy, but nerve conduction velocities were normal on upper limbs and moderately abnormal on lower limbs, the latter attributing to long lasting diabetes mellitus. The women died from acute subarachnoid hemorrhage. Autopsy revealed CLL of B-cell type with generalized organ involvement and acute craniospinal subarachnoid hemorrhage from ruptured cerebral aneurysm. There was selective neoplastic infiltration of the dorsal root ganglia and peripheral nerves, particularly the median nerve. Although selective infiltration of peripheral nerves by B-cell lymphoma cells was not associated with myelo-axonal degeneration, the relationship of this case to human neurolymphomatosis is discussed.

Axons↗