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Biomedical subjects

F Engler

Publications and source records attributed to F Engler.

13 recordsLinked to original sources

Treatment with Sulthiame (Ospolot) in benign partial epilepsy of childhood and related syndromes: an open clinical and EEG study.

The effect of Sulthiame on the EEG and on clinical seizures was evaluated in an open uncontrolled study in 25 children with focal sharp waves on the EEG (FSW). 16 children had typical benign partial epilepsy with rolandic spikes (BPERS), 5 children with atypical forms and 4 children with no clinical seizures but cognitive disturbances possibly related to the FSW. The effect of Sulthiame in suppressing the EEG discharges was evaluated on the waking and sleep EEG before introduction of the drug, and at 3 - 6 months, 6 to 12 months and beyond while under therapy. The children were followed clinically for one to several years. The EEG discharges disappeared or decreased under Sulthiame in 13/21 cases at 3 to 6 months but reappeared in 3/13 cases beyond this period. No case had a worsening of the EEG or of clinical seizures under Sulthiame, and no cognitive stagnation was noted. Our data confirm the good tolerance and positive effects on the EEG and justify systematic trials of this drug in the partial "functional" epilepsies, especially when negative cognitive consequences of the epileptic discharges are suspected.

Adolescent↗

Analysis of immunoglobulins in sarcoidosis.

BACKGROUND: Assessment of local immunoglobulin(Ig)-production in sarcoidosis may be indicative of disease activity. However, in interstitial lung disease an increase in protein leakage across the alveolar-capillary membrane complicates determination of local Ig-production. In order to overcome this problem, techniques successfully used for the evaluation of local Ig-production in cerebrospinal fluid were applied to bronchoalveolar lavage (BAL)-analysis. METHODS: Ten patients with biopsy-proven sarcoidosis, seven patients with respiratory infections and ten patients as controls without any sign of interstitial lung disease or infection underwent BAL. Equal amounts of total protein (2 micrograms/lane) from BAL and serum samples were run on SDS-PAGE gradient-gel and blotted to a nitro-cellulose membrane. The blots were stained for total protein, IgA, IgM, IgG and IgG1-4-subgroups. RESULTS: Densitometric analysis revealed a significant increase of the IgG/albumin-ratio in BAL of sarcoidosis patients compared to the control group. In all control patients a single IgG band of identical molecular weight was detected both in serum and BAL. In sarcoidosis and pneumonia the serum showed multiple bands distinct from the BAL-band in regard to molecular weight. Subclass analysis of this group revealed an increased band intensity and different molecular weight of IgG1, IgG2 and IgG4-bands in BAL compared to serum indicative of local production. IgA and IgM were detected in all samples without any significant differences between the three groups. CONCLUSIONS: Molecular weight analysis of IgG-subgroups revealed local production of IgG1,2+4 in sarcoidosis and respiratory infection. This technique may prove useful with regard to the assessment of disease activity in sarcoidosis.

Adult↗

Postganglionic cholinergic dysautonomia with incomplete recovery: a clinical, neurophysiological and immunological case study.

A 26-year-old man presented with signs and symptoms of marked postganglionic cholinergic autonomic dysfunction manifested by non-reacting dilated pupils, paresis of accommodation, decreased salivation, dry skin, atony of the bladder, erectile impotence and complete gastrointestinal paresis. Standard neurophysiological tests for myelinated sensory and motor fibre function and quantitative methods to examine unmyelinated parasympathetic, sympathetic and afferent fibres were performed: parasympathetic function was measured by heart rate variation tests. Sympathetic cutaneous vasoconstrictor responses induced by deep inspiration were examined with laser Doppler flowmetry. Cutaneous nociceptive C-fibre function was assessed by measurement of axon reflex vasodilatation and flare size induced by histamine iontophoresis. The findings confirmed that the abnormalities were restricted exclusively to the cholinergic postganglionic autonomic systems. All other functions were completely preserved. Modern neurophysiological methods of testing sympathetic and afferent small fibre function might help in the diagnosis of cholinergic postganglionic dysautonomia in the early stages. The specificity of the dysfunction argues in favour of an immunological pathogenesis. However, antibody screening including acetylcholine receptor antibodies and voltage-gated calcium channel antibodies gave negative results. Whatever autoimmunological mechanism might be involved, the postulated antibodies act highly specifically on unknown structures of the cholinergic postganglionic autonomic neurons.

Adult↗

[Affective and schizophrenic syndromes in multiple sclerosis. Review of the literature and case reports].

Based on reports of three cases, the manic, depressive, schizophrenic and organic mental symptoms coincident to multiple sclerosis (MS) are described. The literature indicates an increased prevalence of psychiatric disturbances in MS. However, the relation between the psychiatric and the neurological disorders remains speculative. The temporal covariance and responsiveness to pharmacotherapy of the psychiatric and neurological symptoms of MS as well as the localisation of MS foci in diagnostic procedures (CT, MRI) are discussed in the light of the literature. The present authors hypothesize that every disturbance of the central nervous system (CNS), especially if chronic-inflammatory and multilocal, increases the probability that psychiatric symptoms will arise (as measured on a continuum ranging from "psychically conspicuous" to "psychically inconspicuous"). To this extent, then, MS would be comparable to other chronic-inflammatory CNS disorders such as AIDS, neuroborrelioses, syphilis and certain forms of viral encephalitis.

Adult↗

[Lambert-Eaton myasthenia syndrome--review with case report].

The pathogenetic and therapeutic concepts in Lambert-Eaton Myasthenic Syndrome (=LEMS) are changing. The disease has been proved to be an autoimmune reaction to voltage-dependent calcium channels of the presynaptic membrane at the neuromuscular junction. As pointed out in a case report, autonomic dysfunction can be an early sign and symptom of LEMS. The update review of the literature deals with the diagnosis, aetiology and therapy of the often but not exclusively paraneoplastic induced disease.

Autonomic Nervous System Diseases↗