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Biomedical subjects

F Schumm

Publications and source records attributed to F Schumm.

At least 37 records · Page 2Linked to original sources

[Antibody-controlled cytostatic therapy of malignant thymoma with concomitant myasthenia gravis].

In a 46-year-old female patient with malignant thymoma and concomitant myasthenia gravis relapse with gravitational metastases occurred 6 1/2 years after the first operation. Metastases could be removed surgically only partially and were subsequently irradiated with 50 Gy. After 3 1/2 years renewed metastatic growth occurred. Until then the concomitant myasthenia had been stable during treatment with pyridostigmine and azathioprin and intermittent prednisone; acetylcholine receptor antibody titres had remained largely stable. Combined cytostatic treatment with vincristine, cyclophosphamide, prednisone and doxorubicin or cisplatin led to regression of metastases during the observation period of 1 1/2 years and at the same time to stabilisation of the myasthenia. Acetylcholine receptor antibody titres decreased and this was roughly paralleled by clinical improvement. Whereas there is no obvious correlation of antibodies against acetylcholine-receptor protein and tendency of tumour growth there is good agreement with the course of the accompanying myasthenia.

Acetylcholine↗

Accessory nerve stimulation in the assessment of myasthenia gravis.

Repetitive nerve stimulation (5/second) was done at the median nerve at the wrist and at the accessory nerve just behind the sternocleidomastoid muscle before and 20 seconds to 5 minutes after tetanic nerve stimulation (1 minute). Since the degree of the neuromuscular block depends on the body temperature these investigations were done successively at skin temperatures of 32 degrees C and 36 degrees C. A comparison of the results obtained revealed the highest rate of pathologic decrement with posttetanic accessory nerve stimulation (32 degrees C = 77%, 36 degrees C = 87%), whereas with posttetanic median nerve stimulation pathological results were obtained in a significantly lower proportion (32 degrees C = 50%, 36 degrees C = 60%). The advantages of the stimulation of the accessory nerve for the detection of partial neuromuscular block are: 1. The superficially located accessory nerve allows for supramaximal stimulation with rather low stimulus intensities (6-20 mA). 2. Since the accessory nerve is mainly a motor nerve, the stimulation is less painful than the stimulation of a mixed nerve. 3. Stimulation of a proximal nerve is more sensitive for detecting a defect in neuromuscular transmission than stimulation of a distal nerve. 4. There is no risk of a pneumothorax and of a traumatic nerve lesion as there is with stimulation of the brachial plexus by needle electrodes.

Accessory Nerve↗

[Correlation of acetylcholine receptor antibodies and clinical severity of myasthenia gravis in combined immunosuppressive therapy].

The correlation between the severity of symptoms in myasthenia gravis (score) and the level of acetylcholine receptor-antibodies (ACHR-Ab) was studied in 24 patients treated with immunosuppressive therapy. This correlation was significant in every case regardless of whether or not thymectomy was performed in addition. It was demonstrated by means of a long-term follow up study, that a change in ACHR-Ab preceeds the corresponding change in score by 2-4 months in 80% of our patients. Measurements of ACHR-Ab ease the management of the therapy with immunosuppressants and make it safer. This is particularly true prior to and after termination of the treatment. A change in ACHR-Ab level by more than 20% indicates a change in score.

Adult↗

Myasthenia gravis: overlap with 'polyendocrine' autoimmunity.

81 patients with spontaneously acquired myasthenia gravis (MG) were investigated for the presence of autoimmune (AI) diseases and their sera were tested for a range of organ-specific autoantibodies. 77 of the patients were HLA-phenotyped. Antibody titres to acetylcholine receptors (AChR) were higher in non-thymomatous patients who possessed HLA-B8 (p less than 0.05) and/or -DR3 (p less than 0.05) as compared to patients lacking these HLA antigens. 3 out of 20 (15%) patients with ocular MG, 7/23 (30%) with generalized MG of early onset, 11/23 (48%) generalized MG of late onset and 5/14 (35%) patients with thymoma had either overt AI diseases or significant titres of organ-specific autoantibodies suggesting subclinical AI disease. In ocular MG, low titres and an infrequent finding of antibodies to AChR (32%) as well as the low prevalence of associated autoantibodies and AI diseases indicate that this subgroup of MG consists of patients with restricted AI reactivity. HLA-B8 and -DR3 were present in all the patients with associated AI disorders in the young onset group but in none of the patients with old age of onset. In the young group, 6 out of 7 patients with associated AI conditions were women whereas the sex ratio was about equal in the older cases in both, patients with and without associated AI diseases or autoantibodies. We conclude from these observations that ageing provides conditions that allow the breakdown of self tolerance. The simultaneous presence of HLA B8, DR3 and female sex provide important additional factors for early expression of MG.

Adult↗

[Unusual radiological and clinical findings in a calcified chronic extradural haematoma (author's transl)].

In three of 32 patients with epidural haematomas calcifications and ossifications of the bleeding were found. Quickly developing (3 to 7 weeks) ossifications could be demonstrated by repeated X-rays and CT of the skull in partial drained or untreated haematomas. No ossification could be observed in the remaining 29 cases, in which the haematomas were completely removed. The growth of the ossification from the parietal to the visceral side within the capsules of the haematomas could be monitored in its progress by CT and was histologically verified. The underlying pathological mechanisms are discussed.

Adult↗

[Myasthenia syndrome during chloroquine treatment (author's transl)].

Myasthenic reaction with partial neuromuscular block in the electromyogram and increased antibodies against acetylcholine-receptor protein developed during chloroquine administration over two months in a 52-year-old man known for eight years ot have rheumatoid arthritis. When the drug was discontinued and pyridostigmine administration begun, myasthenia improved within six weeks and had completely disappeared after three months. During the same period abnormal neuromuscular transmission regressed. Also, the significantly increased antibodies against acetylcholine-receptor protein became normal. It remains undecided whether this was a drug-induced myasthenia gravis or only a latent myasthenia manifested by the drug.

Arthritis, Rheumatoid↗

[Morbus Gerstmann-Sträussler-Scheinker. The Sch. family-a report of three cases (author's transl)].

The clinical symptoms from three cases and histological findings from two patients suffering from morbus Gerstmann-Sträussler-Scheinker (M-GSS) are reported. This disease belongs to the group of subacute spongiform encephalopathies. It is extremely rare and so far has only been observed in 52 members of four large families, in which the symptoms begin between the age of 33 and 50 and lead to death in 4-5 years. In the family reported here, cerebellar symptoms including myoclonia and later dementia, bulbar, and pyramidal symptoms were typical; two patients also had deterioration of vision and hearing. CSF and other biochemical data were normal. The EEGs showed progressive general slowing without periodic dysrhythmia. Evoked potential gave no evidence of demyelinization. The disease may safely be distinguished from morbus Creutzfeldt-Jakob (M-CJ) and Alzheimer's disease by histology, which reveals kuru plaques in most cases and invariably multicentric plaques as well as cortical spongiform changes of varying degree with loss of nerve cells and glial proliferation; however, only minor degenerative alterations in the cortical vessels are seen. The transmission to monkeys and histological similarities to M-CJ and kuru suggest a slow virus related to that causing scrapie. Alternatively, the genetically determined susceptibility of the patient may decide the type of reaction to the slow virus. The disposition to M-GSS is autosomally dominant.

Adult↗

[Spontaneous oral dyskinesia. Successful treatment with tetrabenazine (author's transl)].

Tetrabenazine (50-150 mg/day) was a moderate to excellent efficacy in each of six patients with severe spontaneous oral dyskinesia. Slight akinesia, rigidity, and transient disorientation were occasional side effects that could easily be controlled and were tolerated by the patient in view of the lasting amelioration of this debilitating symptom. When tetrabenazine was administered for blepharospasm, only two of six cases showed partial improvement with more severe side effects.

Aged↗

Gerstmann-Sträussler-Scheinker's disease. Electron-microscopic observations on a brain biopsy.

Light and electron microscopic observations are reported on a brain biopsy of a man of 59 with a rare familial disease of the CNS and a 5-year clinical course. Electron micrographs of the frontal biopsy reveal plaque-like deposits composed of amyloid cores, often multicentric in the cortex and subcortical white matter. They are localized between enlarged astrocytic processes. In the neuropil they are sometimes associated with abnormal neuritic processes, in the white matter with processes of fibrous astroglia and basement membranes. There are no signs of primary neuritic or synaptic involvement in the plaque formation which is more obviously associated with altered astrocytic processes. Moreover, degenerative alterations in the cortical vessels and slight astroglial spongiform changes as well as oligodendroglial proliferation can be found. Plaques are considered to be mainly of the kuru type; the relationship with transmissible spongiform encephalopathies is discussed.

Astrocytes↗

[Myasthenic syndrome during penicillamine treatment (author's transl)].

The clinical and electrophysiological findings in 2 men who had developed a myasthenic syndrome after taking penicillamine for rheumatoid arthritis will be described. The symptoms began with dysfunction of the eye muscles following a generalised muscle weakness. Course of illness after withdrawal of penicillamine was not uniform. In one of the patients a complete remission occurred within a year. The other became steadily worse and required continuous treatment with cholinesterase inhibitors. Electrophysiological examinations showed neuromuscular blockade, posttetanic exhaustion, posttetanic potentiation was found in one patient only. An immunopharmacological block of acetylcholine receptors induced by penicillamine is discussed from a pathogenetical point of view.

Adult↗

Normal sensory conduction in the saphenous nerve in man.

Measurements of the sensory conduction velocity and the parameters of nerve action potentials in the saphenous nerve offer many advantages, compared with measurements in other nerves of the leg, regarding accuracy and the time required to complete the investigations. Therefore, the electroneurographical examination of the saphenous nerve is recommended in the early diagnosis of polyneuropathies and is indispensable in the exact diagnosis of proximal neuropathies and lesions of the femoral nerve. In the present study we indicate the proximal and distal segments of the saphenous nerve and illustrate the nerve action potentials from 70 normal subjects.

Adult↗

Retrograde changes in motor and sensory conduction velocity after nerve injury.

Nerve section is followed by a reduction of motor and sensory conduction velocity in the proximal segment of the injured nerve. This reduction of velocity is associated with retrograde changes in fiber size. If reinnervation does not occur within the next 1 1/2--2 years, retrograde degeneration of nerve fibers results, and the amplitude of the evoked nerve potential in the proximal segment of the injured nerve decreases. This retrograde degeneration is probably significant in view of the poor results frequently obtained after nerve transplantation which is carried out too late.

Humans↗

[The ultimate electromyographic patterns in reinnervated muscle (author's transl)].

We investigated 59 patients in later stages after section and suture (n =23) or transplantation (n = 36) of the ulnar or median nerve. The most typical change in electromyographic pattern consists in a markedly increase of mean amplitude, due to an increased number and concentration of muslce fibers in the reconstituted motor-units. The mean duration and the incidence of polyphasic potentials are only slightly increased, indicating a nearly synchronous excitement of the different muscle fibers of the various motor-units. In no case there is a complete recovery; the mean amplitude of evoked potentials in reinnervated muscles varied from 32% in poor-, to 62% in satisfactory reinnervation, compared with the corresponding normal muscle in the opposite hand.

Electromyography↗