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Weitere diverse Neurologische Erkrankungen und Probleme:
Clin Neurophysiol 2001 Feb;112(2):359-68
Identification of the stimulated hemiretina in primary school children and
adults based on left and right hemifield pattern reversal visual evoked
potentials--a comparative study.
Hoffmann K, Leistritz L, Feucht M, Benninger F, Reiter D, Witte H.
OBJECTIVES: The analysis of left and right hemifield pattern-reversal visual
evoked potentials (PVEPs) in children and the identification of the stimulated
hemiretina testing different identification procedures previously applied to
adults. METHODS: Lateral hemifield PVEPs were recorded in 40 children (6-11
years) and 27 adults (25-40 years) from, at least, 19 standard electrodes. Two
procedures were tested for the determination of the stimulated hemifield:
firstly, the evaluation of the values of instantaneous frequency at the
occipital electrodes at P100 latency (determined by the global field power), and
secondly, the application of a generalised dynamic neural network (GDNN) using
the PVEP time course at selected electrode positions as the external input.
RESULTS: P100 latency as well as P100 amplitude over the contralateral occiput
in children were significantly greater than in adults. Contrary to the behaviour
in adults, instantaneous frequency is not a robust identifier of left and right
hemiretina stimulation in children. The best identification performances were
achieved when using group trained GDNNs with the bipolar difference signals of
electrodes P3/P4 or T5/T6 as the external input. CONCLUSIONS: The PVEPs at
electrodes P3/P4 and T5/T6 contain essential information for the determination
of the stimulated hemifield. This should be further considered during the
development of on-line procedures for automatic PVEP detection in future
Neuropediatrics 2001 Feb;32(1):28-37
Long-term MRI observations of childhood-onset relapsing-remitting multiple
Balassy C, Bernert G, Wober-Bingol C, Csapo B, Kornek B, Szeles J, Fleischmann
D, Prayer D.
PURPOSE: Long-term MRI follow-up of childhood-onset relapsing-remitting multiple
sclerosis (RRMS) was carried out in 4 cases. MRI findings were correlated with
clinical course and characteristic differences from adult-onset RRMS were
elaborated. METHODS: Two girls and one boy with true childhood-onset, and one
girl with juvenile-onset RRMS underwent 5-16 MRI examinations within 6-8 years.
The total number of lesions, the numbers of new, active, disappearing and
reappearing lesions, infratentorial and U-fibre lesions, "giant" plaques and
"black holes" were counted. Callosal atrophy and general brain atrophy were
assessed. The findings were related to the physical status according to the
Expanded Disability Status Scale (EDSS). RESULTS AND CONCLUSIONS: Results showed
that the primary differences in childhood-onset RRMS compared to adult-onset
RRMS lie in the lack of, or slower development of irreversible changes ("black
hole" formation, brain atrophy). Despite callosal atrophy and intensive U-fibre
region involvement, school performance was unchanged. Regarding the frequency of
"giant" lesions, an even more pronounced white matter involvement was found in
our children compared to adults. All children exhibited a rather "benign"
disease course. A more intensive remyelination, less severe neuronal loss, and
higher functional brain plasticity at younger ages may account for these
Brain Pathol 1995 Jul;5(3):201-11
The original Gerstmann-Straussler-Scheinker family of Austria: divergent
clinicopathological phenotypes but constant PrP genotype.
Hainfellner JA, Brantner-Inthaler S, Cervenakova L, Brown P, Kitamoto T,
Tateishi J, Diringer H, Liberski PP, Regele H, Feucht M, et al.
We present new data on the original Austrian kindred with
Gerstmann-Straussler-Scheinker disease (GSS) which encompasses currently 221
members in 9 generations. The mode of inheritance is autosomal dominant.
Predominant clinical features are slowly progressive ataxia and late impairment
of higher cerebral functions. In contrast, a recent case with proven P102L
mutation of the PRNP gene had rapidly developing dementia and severe cortical
damage indistinguishable from the clinicopathological phenotype of
Creutzfeldt-Jakob disease (CJD). PRNP codon 129 was homozygous for methionine in
both the historic and recent cases. Neuropathology confirms spongiosis of
variable degree and numerous protease resistant/prion protein (PrP) amyloid
plaques scattered throughout most of the brain as constant features in this
family. Some amyloid deposits are surrounded by dystrophic neurites with
accumulation of phosphorylated neurofilaments and abnormal organelles,
reminiscent of Alzheimer-type plaques. Severe telencephalic damage and a
synaptic-type fine granular immunoreactivity in laminar distribution in the
cortex with anti-PrP after hydrated autoclaving of sections were seen only in
the recent patient. In conclusion, factors in addition to the PRNP genotype at
codons 102 and 129 must play a role in determining clinicopathological
characteristics of this inherited brain amyloidosis.
J Cereb Blood Flow Metab 1995 May;15(3):513-8
D2 receptor blockade by flunarizine and cinnarizine explains extrapyramidal side
effects. A SPECT study.
Brucke T, Wober C, Podreka I, Wober-Bingol C, Asenbaum S, Aull S, Wenger S,
Ilieva D, Harasko-van der Meer C, Wessely P, et al.
Twenty-six patients under treatment with the calcium channel blockers
flunarizine (Fz) or cinnarizine (Cz) were examined-with single-photon emission
computed tomography using [123I]iodobenzamide as a ligand. The striatal dopamine
D2 receptor-binding potential was determined and found to be reduced by 14 to
63% (39.5 +/- 15.0%; p < 0.0001) in patients compared with age-matched control
values. This reduction was larger in 12 patients with extrapyramidal symptoms
and was only slowly reversible after discontinuation of treatment. Patients
treated for > 6 months had significantly larger reductions than patients treated
for a shorter period. Parkinsonian symptoms were only seen in patients older
than 50 years. Our findings prove a neuroleptic-like action of Fz and Cz, which
seems to be the major reason for their extrapyramidal side effects. Older age
and long-term treatment are predisposing factors for these effects.
Lancet 1995 Feb 4;345(8945):331
Interferon-alfa-2b for meningioma.
Wober-Bingol C, Wober C, Marosi C, Prayer D.
Wien Klin Wochenschr 1994;106(21):671-2
Huntington chorea: (CAG)n repeats on gene IT 15 in Austria
Meszaros K, Willinger U, Heiden AM, Fuchs K, Baumhackl U, Brucke T, Feucht M,
Fathi N, Lenzinger E, Miller E, et al.
In March 1993 the gene IT 15 was identified on chromosome 4p and it was
demonstrated that it contained an unstable (CAG)n trinucleotide repeat that is
elongated in patients with Huntington's chorea (HC). Persons with more than 37
(CAG)n repeats tend to have a higher risk of developing the disease. Testing the
(CAG)n repeats in Austrian HC patients with PCR techniques shows correspondence
between the clinical diagnosis of HC and genotypes [more than 42 (CAG)n
repeats]. There was a weak correlation between the number of (CAG)n repeats and
age of onset, however, this finding is without diagnostic value due to the
scatter of the values.
Arch Phys Med Rehabil 1993 Nov;74(11):1151-6
Posturographic measurement of body sway in survivors of severe closed head
Wober C, Oder W, Kollegger H, Prayer L, Baumgartner C, Wober-Bingol C, Wimberger
D, Binder H, Deecke L.
Postural imbalance was measured in 39 survivors of severe closed head injury 7
to 66 months after head injury using a posturographic platform. The results were
compared with those derived from age-matched healthy control subjects. Severe
postural imbalance, particularly in an anteroposterior direction, was found in
16 patients, whereas 9 patients showed moderate imbalance and 14 patients showed
normal results in the posturographic investigation. Low initial Glasgow Coma
Scale scores and deep parenchymal brain lesions demonstrated by magnetic
resonance imaging were shown to be significant indicators of subsequent severe
postural imbalance. The duration of posttraumatic amnesia, the localization and
size of cortical contusions and subcortical white matter lesions, on the
contrary, were not associated with postural imbalance in the long-term outcome.
Dev Med Child Neurol 1993 Feb;35(2):163-6
Transient bilateral internuclear ophthalmoplegia after minor head-trauma.
Mueller C, Koch S, Toifl K.
Following minor occipital head-trauma, a six-year-old boy developed bilateral
internuclear ophthalmoplegia (INO) as the only neurological sign. After having
excluded all other possible aetiological causes by careful examination,
including magnetic resonance imaging, the symptoms eventually were attributed to
the trauma. The course of the INO was documented by electro-oculography. A
saccades-training programme specially adapted to the boy's age was performed,
and complete remission of the oculomotor symptoms occurred after one year.
Wien Klin Wochenschr 1993;105(2):61-3
Mefloquine and sulfadoxine/pyrimethamine overdose in malaria tropica
Burgmann H, Winkler S, Uhl F, Feucht M, Hellgren U, Bergqvist Y, Feistauer S,
Breyer S, Graninger W.
A 39 year-old man with malaria due to Plasmodium falciparum received 3500 mg
mefloquine over 3 days, in addition to 3250 mg chloroquine and 175/3500 mg
sulfadoxine/pyrimethamine. He developed severe neuropsychiatric symptoms and had
to be hospitalized. Treatment with diazepam, haloperidol and thioridazine
achieved relief of the severe symptoms after 4 days. The patient was still
suffering from discrete neuropsychiatric symptoms 8 months after treatment.
Lancet 1991 May 11;337(8750):1160
Prion protein mutation in family first reported by Gerstmann, Straussler, and
Kretzschmar HA, Honold G, Seitelberger F, Feucht M, Wessely P, Mehraein P, Budka
Wien Klin Wochenschr 1990 Feb 2;102(3):90-4
24-hour blood pressure determination in Shy-Drager syndrome. A case report
Wober-Bingol C, Wessely P, Gradner W, Deecke L.
The case history is presented of a 57 year-old male patient suffering from the
Shy-Drager syndrome. This syndrome was first described by G. M. Shy and G. A.
Drager in 1960. The clinical manifestations are extrapyramidal, with
Parkinsonian features, combined with severe orthostatic hypotension and failure
of the autonomic nervous system. Apart from the standard clinical investigations
in this condition, namely the orthostasis test and polygraphic recording of the
EEG, ECG, breathing and blood pressure on the tilting table, additional
non-invasive long-term determination of the blood pressure was carried out. This
method allows simultaneous measurement of blood pressure and heart rate without
appreciably interfering with the patient's daily activities.
Padiatr Padol 1988;23(3):195-207
The prognosis of juvenile myasthenia gravis
Toifl K, Zeitlhofer J, Vass B.
In 18 patients with juvenile myasthenia gravis (MG) the mean age of
disease-onset was 12.1 years. The mean observation time was 6.8 years. After
classification with regard to the clinical severity of the disease (Ossermann,
1958) 4 patients (22%) could be assigned to type I, 10 patients (56%) to type II
A, 2 patients (11%) to type II B, and 2 patients (11%) to type III. 14 patients
(77%) could be assigned to type I and type II A, both of them rather benign
types. All 4 male patients belonged to this group. The tensilon test,
investigated in 15 patients, showed a positive result in 13 cases (87%). The
repetitive stimulation was done in 13 patients and was positive only in 6 cases
(46%). Investigations in MG-patients without limitation of age showed positive
results in about 70%. Increased levels of acetylcholin-receptor-antibody were
found in 10 of 11 patients (91%). In 8 patients with the diagnosis MG, type II A
and III, a thymectomy was done, 16 patients received cholinesterase-blockers, 1
patient with type II A, and both patients in group III received additionally
azathioprin. In 1 patient with type III plasmapheresis was done. 75% of all
thymectic patients showed a remission or improvement. Related to all 18 patients
we found in 16 cases (89%) a remission or correction of the symptoms. In summary
the prognosis in the group of our patients with juvenile myasthenia gravis was
Klin Padiatr 1984 Jan-Feb;196(1):14-7
Hormonal findings in children with central manifestations of Recklinghausen's
Zeitlhuber U, Frisch H, Toifl K, Wimmer S.
Hormone parameters were evaluated in 14 children with cerebral manifestation of
N.R. to find a possible involvement of the hypothalamo-pituitary axis. In
addition to growth hormone and prolactin stimulation, tests of gonadotropic,
thyreotropic and adrenocorticotropic function were performed. 6 patients had
insufficient growth hormone secretory capacity, and 6 times an increased
prolactin-stimulation was found; in one patient ACTH-reserve was diminished. A
brain tumour was found in 5 of these 8 patients with impaired hormone results;
however, in 3 cases pathologic results were found only after operation.
Padiatr Padol 1983;18(1):29-37
Clinical and neuroradiological aspects in the infantile form of Moyamoya
Dal-Bianco P, Reisner T, Friedrich MH, Spiel W.
Eur Neurol 1983;22(1):65-9
Adenylate kinase activity of cerebrospinal fluid in central nervous system
Unger W, Toifl K, Bohm MP, Bayer PM.
Adenylate kinase activity was measured in 41 samples of cerebrospinal fluid in
34 patients with various neurological disorders or psychiatric symptomatologies.
Activities of the enzyme showed to be linked to clinically estimated acuteness
or progression of the changes in the central nervous system at the time of
specimen collection. The findings suggest the conclusion that determination of
adenylate kinase activity in cerebrospinal fluid is a meaningful tool for the
evaluation of progression and/or acuteness of central nervous system disorders.
Clin Chim Acta 1982 Nov 10;125(3):299-305
Detection of adrenoleukodystrophy by increased C26:0 fatty acid levels in
Molzer B, Bernheimer H, Heller R, Toifl K, Vetterlein M.
Very long chain fatty acids of peripheral blood leukocytes were analyzed by gas
chromatography in nine members of a family including two hemizygotes and one
obligate heterozygote for adrenoleukodystrophy (ALD), as well as in twelve
controls. Comparative investigations were done in cultured fibroblasts. Elevated
C26:0 levels were observed in leukocytes and fibroblasts of ALD hemizygotes. The
obligate heterozygote displayed a clear-cut increase of C26:0 concentration in
leukocytes but not in fibroblasts. Determination of C26:0 in leukocytes may
serve as diagnostic tool in the detection of ALD gene carriers.
Nervenarzt 1982 Aug;53(8):471-4
Case report on neurologic concomitant symptoms in angioneurotic edema
Toifl K, Wessely P.
J Neurol Sci 1981 Aug;51(2):301-10
Accumulation of very long chain fatty acids is common to 3 variants of
adrenoleukodystrophy (ALD). "Classical" ALD, atypical ALD (female patient) and
Molzer B, Bernheimer H, Budka H, Pilz P, Toifl K.
Fatty acids of cholesterol esters were analyzed by gas chromatography in
affected CNS white matter of 3 variants of ALD ("classical" ALD, atypical ALD
(adult female) and AMN) and of 10 controls with myelin breakdown of an etiology
other than ALD. In all 3 ALD variants a marked accumulation of very long chain
fatty acids (VLFA) as compared to control material was observed. This was due to
the accumulation mainly of saturated C24-C32 fatty acids, particularly of C26:0,
C25:0 and, to a lesser extent, C24:0 and C27:0 fatty acids. Our results
demonstrate for the first time an accumulation of VLFA in an adult female
patient (atypical ALD), who probably is an ALD heterozygote rather than a
variant of AMN, and confirm and extend earlier findings in classical ALD and
AMN, respectively. It appears that ALD may be a single nosological entity with
clinically and morphologically different variants sharing specific
ultrastructural (accumulation of paired leaflets) and neuro-biochemical
(accumulation of VLFA) diagnostic markers.
Nervenarzt 1981 Apr;52(4):211-8
EEG- and biochemical findings in Kleine-Levin-syndrome. A case report (author's
Vollmer R, Toifl K, Kothbauer P, Riederer P.
Neurochirurgia (Stuttg) 1981 Jan;24(1):35-7
Primary diffuse leptomeningeal melanoblastosis and its CT appearances (author's
Auff E, Reisner T, Toifl K, Pernecky A.
We report the case of a twelve-year-old boy who died, after a six months
history, from primary diffuse leptomeningeal melanoblastosis, CT investigations
showed a saw-tooth contrast enhancement at the brain surface. CT investigations
may therefore be very helpful in meningeal lesions - apart from the importance
of CSF investigations.
Acta Neuropathol Suppl (Berl) 1981;7:211-4
Fatty acid patterns in brain, fibroblast, leukocyte and body fluid lipids in
Molzer B, Bernheimer H, Toifl K.
Fatty acid patterns of certain tissue and body fluid lipids of ALD and control
cases were investigated by thin layer and gas chromatography. A high percentage
(55.2%) of VLFA was found in the ALD brain white matter cholesterol esters; this
was mainly due to increased proportions of 26:0, 25:0, 26:1 and greater than 26
C fatty acids. The percentage of VLFA in the white matter cholesterol esters of
pathological controls with myelin breakdown of different etiology was low
(0.4-7.1%). In the fibroblast total lipids of an ALD patient an increase of 26:0
and 25:0 fatty acids was found. Moderately increased proportions of hexacosanoic
acid were observed in the leukocyte total lipids of 2 severely affected ALD
patients, but not in a slightly as well as in a possible affected case. No
accumulation of VLFA was detected in cholesterol ester, triglyceride and free
fatty acid fractions in the CSF of an ALD patient and in ALD serum
sphingomyelin. Our results substantiate the specificity of VLFA accumulation in
the white matter cholesterol esters of ALD brain and lend some further evidence
to the view that a generalized impairment of VLFA metabolism seems to be present
in this disease.
J Neurol 1981;225(1):47-55
A combination of spastic paraparesis, polyneuropathy and adrenocortical
insufficiency-a childhood form of adrenomyeloneuropathy.
Toifl K, Mamoli B, Waldhauser F.
This report describes a combination of spastic paraparesis and symmetrical
sensory motor polyneuropathy with a pathological response to the ACTH test in
the case of a 16 year-old boy and a borderline response to the ACTH test in the
case of his 8-year-old sister. Another sister, aged 14, showed only a
pathological response to ACTH testing, the neurological status being
unremarkable. The EEG was normal in all three children examined. Visually evoked
potentials were borderline in the case of the boy and normal in the case of the
clinically involved sister. Although on examination by light microscopy the
sural nerve proved to be normal, the clinical diagnosis of adrenomyeloneuropathy
(AMN) in its juvenile form may be assumed, in view of the clinical symptoms and
the evidence of adrenocortical insufficiency revealed by the ACTH test.
Wien Klin Wochenschr 1980 Dec 19;92(24):851-4
Psychopathological manifestations in the early diagnosis of cerebral tumours in
Friedrich MH, Leixnering W, Bogyi T.
The psychopathology of cerebral tumours in childhood is divided into syndromes
of diminished consciousness, diffuse organic psychosyndromes, regression or
retardation and local cerebral psychosyndromes. 15 unselected paediatric
patients suspected of having a cerebral tumour owing to psychiatric symptoms
were admitted for psychopathological and psychopathometric investigations. It is
not possible to detect connections between symptoms and localisation by
psychopathological examinations, but skill disorders serve as early
unmistakeable pointers to local cerebral lesions. Specific differentiation of
psychopathometric investigations give an indication of possible future
improvements in tumour localisation.
Electroencephalogr Clin Neurophysiol 1979 Oct;47(4):411-9
EEG, pattern-evoked potentials and nerve conduction velocity in a family with
Mamoli B, Graf M, Toifl K.
In a family with 8 children one case of adrenoleucodystrophy (ALD), verified by
autopsy, 2 cases with clinical signs of ALD and four other clinically healthy
subjects with pathological ACTH tests were found. As an initial sign in the EEG,
temporo-occipital slowing occurred. Further EEG studies showed spread of the
primary local abnormalities, indicating diffuse brain dysfunction during
progression of the disease. Terminal EEG flattening may be characteristic for
late stages of the disease. No correlation could be established between
lateralization, paroxysms and actual stage of the disease. Abnormal EEGs
occurred not only in clinically and subclinically affected male patients, but
also in a girl, who might be a carrier of ALD. Visual evoked potentials showed
an increased latency of the P2 wave in the clinically, and in one subclinically,
affected subjects. Motor nerve conduction velocity was reduced in only 2
patients. Antidromic sensory nerve conduction velocity of the median nerve was
normal in all patients.