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Biomedical subjects

J Willeit

Publications and source records attributed to J Willeit.

At least 19 recordsLinked to original sources
PubMed1992

Superficial siderosis of the central nervous system: report of three cases and review of the literature.

We present 3 cases and a review of the literature to demonstrate the current state of clinical diagnosis and therapy of superficial siderosis of the central nervous system. Typical symptoms were progressive cerebellar ataxia, spasticity and hearing loss. Repeated subarachnoid hemorrhage was indicated by persistent xanthochromia of the cerebrospinal fluid and confirmed by the presence of erythrophages, siderophages and iron-containing pigments. Deposition of free iron and hemosiderin in pial and subpial structures leads to intoxication of the central nervous system and represents the pathophysiological mechanism of superficial siderosis. Hypointensity of the marginal zones of the central nervous system on T2 weighted MR images indicates an iron-induced susceptibility effect and seems pathognomonic for superficial siderosis. In 39 of the 43 previously described cases superficial siderosis was verified by biopsy or autopsy. Today magnetic resonance imaging enables diagnosis at an early stage of the disease. Therapeutic management requires the elimination of any potential source of bleeding. In patients with unknown etiology no proofed therapy is yet available.

Aged↗
PubMed1992

[Wilson's disease with primary CNS manifestation--current status in diagnosis and therapy].

Five cases studied at our clinic are discussed and the literature reviewed with the aim of assessing the diagnostic value of various examination methods used for Wilson's disease and a modified diagnostic approach is suggested. CT and MRI are compared with regard to sensitivity, specificity and prognostic value. Almost regularly, MRI showed bilateral lesions in the basal ganglia in combination with structural changes in the brain stem. A pattern consisting of symmetrical lesions of the red nuclei, the periaqueductal grey region and, facultatively, the substantia nigra and the dentate nuclei was discovered and appeared almost pathognomonic. Follow-up studies revealed excellent reversibility of MRI changes by both penicillamine and trientine. Auditory evoked potentials showed the highest sensitivity and the best correlation with structural findings. Kayser-Fleischer corneal rings--a diagnostic requirement in the literature--were not found in all patients. The current state of therapy and therapy management is discussed on the basis of pathophysiological considerations. As possible complications of penicillamine administration the deterioration of the clinical condition after initiating therapy, and the risks associated with an abrupt termination of therapy are discussed in detail.

Adolescent↗
PubMed1991

[Orthostatic tremor].

Two men, aged 57 and 77 years old, reported increasing unsteadiness on standing upright for the past 2 and 5 years, respectively. It had become impossible for them to remain standing for more than 10 seconds, as otherwise they might fall down. Walking, sitting and lying were possible without difficulty. Both patients were found to have a fine tremor in both legs on standing only. There were no other neurological signs or symptoms. Electromyography of the leg muscles demonstrated tremor activity at 14-16 Hz. Propranolol, 40 mg three times daily, in the 57-year-old patient brought no improvement. However, on taking primidone, in slowly increasing doses up to 250 and 500 mg daily, respectively, the condition lastingly improved in both patients.

Aged↗
PubMed1991

Cervical herpes zoster and delayed brainstem infarction.

Varicella-zoster (VZ) virus is a rare cause of CNS angiitis, which commonly presents as herpes zoster ophthalmicus with contralateral hemiplegia due to hemispheric infarction. We report the first case of VZ-angiitis with infarction in the ventral pons, following cervical herpes zoster.

Brain Stem↗
PubMed1991

Clinical and electrodiagnostic findings, nerve biopsy and blood group markers in a family with hereditary neuropathy with liability to pressure palsies.

Clinical, electrophysiologic and biopsy findings as well as studies of blood group markers in a family with hereditary neuropathy with liability to pressure palsies (HNPP) are reported. There was an autosomal dominant trait without genetic linkage between the HNPP gene and blood group markers controlled by chromosome 1. Reduced motor and sensory nerve conduction velocity was found in clinically affected and unaffected nerves. Characteristic morphological changes in sural nerve biopsy including tomaculous swelling were present.

Adolescent↗
PubMed1991

[Nemaline myopathy: an unusual course].

A girl presents immediately post partum with postures and movements typical for severe muscular hypotonia (floppy infant). Her sucking and swallowing abilities are reduced. There is marked drooling. Broad alveolar ridges give the impression of a high-arched palate. Floppy infant-screening (muscle enzymes, EMG, NCV) was within normal ranges apart from a slight elevation of aldolase. Muscle biopsy performed at the age of two years revealed the diagnosis of nemaline myopathy. An onset of the disease with severe muscular hypotonia during neonatal period usually is linked with rapidly progressing, mostly lethal outcome. Our patient--in contrast--seems to suffer from a mild form.

Biopsy↗
PubMed1990

[Reproducibility of ultrasound criteria for characterizing carotid artery stenoses].

Comparisons with intraoperative findings suggest that Duplex scanning may be of value in predicting the morphology of carotid artery stenoses. Such studies, however, are based on the results obtained by experienced investigators. To clarify whether sonographic criteria can be standardized, 6 investigators from different hospitals each documented 30 carotid artery stenoses of greater than or equal to 40% diameter reduction which could be imaged in at least 2 planes. Three sonographic criteria (plaque surface, echo density, echo structure), each with 8 categories, were assessed from the image documentations by the 6 as well as by 2 independent further investigators. Depending on the experience and on the device used 29-81% of all stenoses greater than or equal to 40% examined in the different laboratories could be included in the study. The +/- 1 category concordance between the different investigators averaged 50-60% for all sonographic criteria independent from the degree of stenosis and from the image quality. Because of lack of sufficient reproducibility the subjective assessment of sonographic criteria is found to be not suitable for use in multicentre studies.

Brain Ischemia↗
PubMed1989

Doppler and duplex sonography of the cervical arteries and correlations with other examinations. A field study in a population over forty years.

Of the population of a small Tyrolean village, 185 (56%) of the 329 inhabitants over 40 years were investigated by means of Doppler and duplex sonography, electroencephalography (EEG), electrocardiography (ECG), and neurological examination. Four subjects (2%) previously had a transitory ischemic attack (TIA) or stroke in the carotid territory. Sonographically detectable abnormalities in one or more extracranial arteries were present in 42 (23%) persons. Of the 14 subjects with more than slight abnormalities in the common or internal carotid artery two were symptomatic. Two additional cases with TIA or stroke did not show relevant lesions on sonographic examination. The presence of narrowing in the extracranial artery was not related to risk factors (hypertension, smoking, obesity) or abnormalities on ECG and EEG. This study shows 1) that the clinical relevance of ultrasound screening of the carotid arteries in an average population is 15%; 2) that significantly more patients with TIAs or strokes are found in the group with more severe sonographic findings (p = 0.001) than in the group with normal ultrasound results.

Carotid Arteries↗
PubMed1988

Primary CNS lymphoma presenting as a choreic movement disorder followed by segmental dystonia.

Clinical presentation of primary CNS lymphoma with an extrapyramidal movement disorder has not been recorded. A 66-year-old woman presented with chorea involving her left arm and subsequently developed right-sided segmental dystonia with prominent hemifacial dystonic spasms, milder torticollis and dystonia of the right arm. Investigations revealed primary CNS lymphoma with extensive involvement of the right-sided basal ganglia as well as lesions confined to the head of the left caudate nucleus and the corpus callosum. Chorea of her left arm subsided with progressing disease while remission of right-sided segmental dystonia was observed following radiotherapy of the brain. This patient's findings and a review of the literature suggest a possible relation between cranio-cervical dystonia and pathology affecting the head of the caudate nucleus.

Aged↗
PubMed1988

[Hypophyseal abscess and cerebral arteritis in a fatal course of pneumococcal meningitis].

Even today uncomplicated courses of pneumococcal meningitis show relatively high fatality rates. Abscess formation leads to a drastic deterioration of prognosis; the same applies to the rarely observed occurrence of a cerebral arteritis. Up to 1985 only 50 cases of pituitary abscess had been described in the literature. This case report describes the signs and symptoms as well as the neuroradiological and post mortem findings on a patient who succumbed to a pneumococcal meningitis, complicated by formation of a pituitary abscess and cerebral arteritis.

Abscess↗
PubMed1987

[Differential diagnosis of spinal processes using modern diagnostic procedures].

Modern diagnostic management of diseases of the spinal cord is discussed, illustrated by some exemplary cases and reviewed by the literature. Special attention is directed to high resolution tomography, magnetic resonance imaging, analysis of the cerebrospinal fluid and electrophysiological methods. It seems conceivable that a shift from invasive methods to noninvasive procedures (e.g. magnetic resonance imaging) may occur.

Angiography↗
PubMed1987

[Quantification of cerebral circulation using duplex sonography].

An attempt was made to measure quantitatively the total cerebral blood flow by means of Duplex sonography. In a group of healthy young subjects a median value for total cerebral blood flow was obtained amounting to 469 ml/min +/- 30%; repeat measurements yielded a maximum deviation of +/- 11%. In three patients the values obtained after severe apoplectic insult due to occlusion of the internal carotid artery were definitely below the value of the group of healthy subjects, whereas the value for the total blood flow was in the upper range of normal values in a patient with occlusion of the a. cerebri media. Comparative measurements of the regional cerebral blood flow with xenon 133 yielded in those patients with occlusion of the internal carotid artery a markedly reduced mean flow and in the patient with occlusion of the a, cerebri media a less markedly reduced mean flow. Regionally reduced perfusion was seen in all the four patients in the range of the clinically and computer tomographically well-known ischaemia zone. Thanks to the simplicity of this sonographic examination method it could be a useful decision parameter in determining the indication for a reconstruction of the carotid artery, especially in asymptomatic patients.

Carotid Artery Diseases↗
PubMed1986

Meningopolyneuritis Bannwarth with focal nodular myositis. A new aspect in Lyme borreliosis.

A patient with serologically confirmed infection by Borrelia burgdorferi presenting with painful paresis and atrophy of the proximal muscles of both upper extremities and bilateral facial paresis is described. Electromyography showed a neurogenic and myopathic pattern, and creatine kinase was raised. Muscle biopsy revealed the typical signs of focal myositis. Treatment with i.v. penicillin led to dramatic clinical and serological improvement. Muscle biopsy was repeated 2 months later; neurogenic changes were still present, but no inflammatory signs could be seen anymore. Thus, the presented case may be the first reported of meningopolyneuritis accompanied by focal nodular myositis, in the second stage of Lyme borreliosis.

Biopsy↗