[Friedrich Christoph Pelizaeus--neurologist and balneology specialist].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A Ferbert.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
We report on a 68-year-old patient who was admitted with hemiparesis and disorientation. Computed tomography showed several old, mostly lacunar infarcts. One week after admission, the patient became comatose. A repeat computed tomography revealed several new hypodense lesions in both hemispheres compatible with acute cerebral infarctions. Autopsy confirmed the diagnosis of numerous acute encephalomalacias of the same age which were found in different territories of the anterior cerebral circulation. Histologic examination showed the extraordinary result of multiple cholesterol emboli with a maximum in the peripheral leptomeningeal vessels. The source of the emboli was severe atherosclerosis with an ulcerated and thrombotic surface of the aorta and both carotid arteries. This case is remarkable because of its severe and synchronous emboli of cholesterol in several different cerebral territories. One has to suspect a so far unknown physical or metabolic factor responsible for the shower of emboli.
Explore the source record for details and available documents.
OBJECTIVE: The excitability of human brainstem interneurons is measured by the blink reflex recovery cycle and is abnormal in blepharospasm. We wondered whether the results of this paradigm depend on the stimulus intensity. METHODS: We investigated the blink reflex recovery cycle in 13 healthy control subjects and in 13 patients with blepharospasm (7 of them treated with botulinum toxin) using 4 levels of stimulus intensity (5, 12, 19 and 26 mA) and two interstimulus intervals (ISIs, 150 or 250 ms). RESULTS: In all groups the inhibition of the second R2 response was reduced with strong stimulus intensities: In controls, the inhibition of the second R2 decreased significantly (e.g. young controls, ISI 150 ms, from 89.6+/-15.6% at 5 mA to 21.9+/-49.7% at 26 mA, mean+/-standard deviation). In patients the R2 inhibition found at 5 mA was converted in an R2 facilitation at 26 mA, irrespective of the status of treatment. In addition, the patients' results at 5 mA did not differ significantly from the controls' results at 26 mA. CONCLUSIONS: The R2 inhibition and its sensitivity to detect abnormal interneuronal excitability depend on the stimulus intensity, which may act by modifying the excitability of the R2 interneurons.
BACKGROUND AND OBJECTIVES: With the Health Reform 2000, the Australian Refined Diagnosis Related Groups (AR-DRG), Version 4.1 have been chosen as the basis for the future German costing system for hospitals. With regard to Stroke Severity (Barthel Index [BI]) we investigated to what extent the grouping according to AR-DRGs can reproduce healthcare expenditures for such patients. Options to adapt and optimize the system are discussed. PATIENTS AND METHODS: 632 patients who had suffered a cerebrovascular accident and were discharged from conservative acute care in 1999, were classified according to the AR-DRGs. For the grouping we alternatively used data from the current hospital information system and a stroke database for quality assurance. The results were also compared with the clinical profiles for the public hospital sector of the corresponding DRGs in Australia (1997-98). RESULTS: On average 0.99 additional diagnoses per case were documented in the hospital information system, compared to 3.65 in the stroke database. In the stroke database 177 cases (36.8%) were assigned to the DRG with the highest cost weight. 53.7% of these patients suffered a serious stroke (BI < 30). Grouping on the basis of hospital information system data led only to 14 cases (2.8%) assigned to the DRG with the highest cost weight. CONCLUSIONS: Type and extent of additional diagnoses are crucial for the grouping process. From a clinical and economic point of view, measures of disability and impairment should be assigned to the grouping process to improve homogeneity under both aspects. Scores can also serve for determining reliable outcome parameters. For the development of an outcome related reimbursement system, procedures must be included in the definition of medical DRGs. In future, DRGs, which cover overlapping healthcare sectors, should be developed for patients with poststroke rehabilitation.
The effect of botulinum toxin A (BTX) was studied on 12 patients with idiopathic craniofacial hyperhidrosis. After confirming the diagnosis by Minor's iodine starch test we first treated one-half of the forehead with an injection of 2.5-4 ng BTX (Dysport) equidistantly intracutaneously. After 4 weeks we assessed the efficacy by another Minor's iodine starch test and then treated the other half. Another 4 weeks later a standardized telephone interview was carried out. After 1-7 days the craniofacial sweating in the area injected had completely ceased in 11 patients and was mildly reduced in the remaining one. The efficacy was confirmed by repeated Minor's iodine starch tests. Mild weakness of frowning was the only side effect, lasting 1-12 weeks and completely resolving in all patients. Although sweating has not yet recurred in most patients at follow-up periods up to 27 months, one patient had a relapse 9 months after treatment. Following reports on palmar and axillary hyperhidrosis and gustatory sweating (Frey's syndrome) this is apparently the first report on the use of BTX in the treatment of idiopathic craniofacial hyperhidrosis. BTX seems a promising new treatment for localized hyperhidrosis.
We studied the diagnostic value of computed tomographic angiography (CTA) in the posterior circulation as a noninvasive substitute for intra-arterial digital subtraction angiography (DSA). We prospectively investigated 103 patients with acute stroke in the posterior circulation. All patients underwent CTA and Doppler ultrasound, and DSA was performed in 22 patients. Vascular findings were classified in categories according to the type of lesion and the location in the vertebral, basilar, or posterior cerebral artery. In the first part of the analysis we examined the correlation between CTA and intra-arterial DSA (n=22), and in the second part that between CTA and Doppler ultrasound (n=103). Intra-arterial DSA identified 11 stenoses, 13 occlusions, and 3 hypoplasias. The correlation between DSA and CTA was best for the basilar artery (all lesions were identified with CTA). Of the 14 lesions detected in the vertebral artery by DSA 13 were also detected by CTA, but the specific type of lesion was identified in only 7 cases. The correlation between CTA and Doppler ultrasound was lower. In conclusion, CTA is thus a reliable method for detecting lesions in the posterior circulation and may replace DSA in many cases. However, particularly in the vertebral artery DSA remains the superior technique.
In medical literature peripheral neurological disorders are associated with Crohn's disease only in particular cases. We report on two patients who suffering from Crohn's disease developed an acute Guillain-Barré syndrome or a polyneuropathy associated with myositis. Two weeks after a 65-year old female patient developed a serious Guillain-Barré syndrome, a haemorrhagical Crohn's colitis was diagnosed. Within a few weeks the specific therapy led to a satisfying recovery. A 45-year old female had suffered from Crohn's disease since 12 years. Within 8 weeks she developed a serious sensorimotor mixed axonal and demyelinating neuropathy and a granulomatous myositis. After treatment with steroids her signs constantly decreased. These observations as well as a literature review show that polyneuropathy and Guillain-Barré syndrome can be associated with chronic inflammatory bowel diseases. Our results suggest that peripheral neurological signs could be regarded as a possible extraintestinal manifestation of inflammatory bowel disease.
Primary dystonia is a clinically and genetically heterogeneous movement disorder characterized by sustained involuntary muscle contractions causing repetitive movements and/or abnormal postures. Recently, the gene locus (DYT1) and mutation responsible for a substantial number of cases suffering from early-onset primary dystonia was described. Here we report 2 German families and 1 sporadic patient with early-onset dystonia due to the DYT1 mutation in order to illustrate the variability of clinical manifestation within this molecularly defined entity. We demonstrate that writer's cramp or focal cervical dystonia is a clinical presentation of DYT1 as well as generalized dystonia.
OBJECTIVES: Orthostatic tremor was first described by Heilman in 1984. It usually occurs in the legs during stance and decreases markedly during sitting or walking. The aim of this study was to determine if orthostatic tremor is invariably associated with the orthostatic and weight bearing conditions in the arms and legs, and to investigate the features of orthostatic tremor under different levels of peripheral loading. METHODS: Multichannel surface EMG recordings were obtained under different conditions (body posture and peripheral loading) from the proximal arm and leg muscles of seven patients fulfilling the clinical and electrophysiological criteria of orthostatic tremor. RESULTS: In weight bearing positions (stance; weight bearing on the hands on all fours), all patients showed 13 Hz-16 Hz tremor activity, predominantly in the active limb. No tremor activity could be found in a supine position with muscles at rest. Isometric contraction of the limbs in the supine position led to synchronous 13 Hz-16 Hz rhythmic activity in five patients. No tremor was seen when the subjects were suspended in a harness with relaxed legs. Isometric contraction of the legs in this position produced tremor in two patients. A stepwise reduction of the body weight by a harness reduced the tremor activity. Additional loading (10 kg-20 kg) during stance led to an increase in tremor amplitude, but tremor frequency remained unchanged. CONCLUSIONS: Orthostatic tremor is invariably present during stance or other weight bearing positions. It is not, however, always associated with orthostasis. In at least some patients it can be classified as an orthostasis independent action tremor. The failure of peripheral loading to modify tremor frequency indicates that orthostatic tremor may have a central, rather than a peripheral, origin.
BACKGROUND AND PURPOSE: To our knowledge, no large-scale studies comparing the accuracy of CT angiography (CTA) to intraarterial digital subtraction angiography (DSA) of intracranial stenosis have been reported. We attempted to determine the diagnostic value of intracranial CT angiography (CTA) of normal vasculature and variants as well as of stenoocclusive disease. METHODS: One-hundred and twelve patients underwent CTA and intraarterial angiography, and 2205 vascular segments were examined to ascertain presence, visibility, and degree of arterial stenoses (n = 105) as well as anatomic variants. Source, maximum intensity projection (MIP), and MIP-generated multiplanar reformatted (MPR) images were evaluated. RESULTS: All 55 anatomic variants were identified correctly. Visibility of small-vessel segments was increased from 75% to 83% by using source images. MPR was helpful in differentiating distal vertebral hypoplasia from stenosis and in overcoming artifacts. All 43 occlusive segments were graded correctly (sensitivity = 100%, predictive value = 93.4%) as follows: severely stenotic ([n = 23], sensitivity = 78%, predictive value = 81.8%); moderately stenotic ([n = 36], sensitivity = 61%, predictive value = 84.6%); and mildly stenotic ([n = 3], sensitivity = 66%, predictive value = 28%). Normal segments (n = 2100) had a sensitivity of 99.5%, and CTA evinced a specificity of 99% for detecting stenoocclusive disease. Approximately one-third of wrong assessments were related to the petrous segment of the carotid artery. CONCLUSION: CTA with double-detector technology and advanced postprocessing algorithms, including MPR, is about as reliable as MRA in depicting the vasculature of the anterior and posterior circulation and in grading intracranial stenoocclusive lesions, with the exception of the petrous segment of the carotid artery. CTA might be superior to MRA in the evaluation of poststenotic low-flow segments.
Explore the source record for details and available documents.
In most patients with basal ganglia calcification no disturbance of calcium metabolism is present. We present four patients with basal ganglia calcification. Two suffered from a secondary hypoparathyroidism following a complicated strumectomy years ago, one had an Alport-Syndrome and hypoparathyroidism. Her mother showed basal ganglia calcification and an abortive Alport-Syndrome as well, but no hypoparathyroidism.
We present a patient with Bickerstaff's brainstem encephalitis in whom MR imaging correlated with the clinical findings during the course of the disease. T2-weighted MR imaging showed increased signal intensity in the medulla oblongata, upper pons, pendunculi cerebelli and the cerebellum. In the course of the disease the lesions on MRI tended to descend more caudally. No anti-GQ1b antibody titers could be detected in the acute phase of the illness. Our findings suggest that MRI is the most valuable diagnostic tool in supporting the clinical diagnosis of Bickerstaff's brainstem encephalitis and may be helpful in the distinction between Miller Fisher syndrome and Bickerstaff's brainstem encephalitis.
In order to determine the extent to which the recovery cycle of the blink reflex is modified by voluntary contraction of the eyelids we investigated the electrically elicited blink reflex with paired stimuli of identical intensity in 9 healthy subjects. We pseudorandomly administered two interstimulus intervals (150 ms and 250 ms) in three different conditions of voluntary contraction of the orbicularis oculi muscle (relaxed, mild lid closure and strong lid closure). Our results show that inhibition of the R2 response following the second stimulus is significantly reduced with voluntary contraction. In addition, we found significantly larger amplitudes and shorter latencies of R1 and R2 with voluntary contraction. We conclude that in healthy subjects, voluntary eyelid contraction causes facilitation or disinhibition not only in the nuclei of the seventh cranial nerve, but also in the polysynaptic pathway of the R2. The possible implications on the interpretation of clinical data are discussed.
OBJECTIVES: To investigate the effects of injection of botulinum toxin type A (BTX A) into the orbicularis oculi muscle and lacrimal gland in patients with aberrant regeneration after facial palsy (facial synkinesias and hyperlacrimation). METHODS: The effect of the toxin injection (on average 75 mouse units of BTX A) into the orbicularis oculi muscle on facial synkinesias was assessed on a five point (0 to 4) scale in 10 patients with aberrant regeneration of facial nerve fibres after a peripheral facial nerve palsy. Six patients underwent a videographic control, which was assessed by a blinded independent investigator. In two patients with hyperlacrimation an extra dose of botulinum toxin (on average 20 mouse units BTX A) was injected into the lacrimal gland and the effect was assessed using the Schirmer test and on a three point scale. RESULTS: Botulinum toxin type A had a good to excellent (grades 3 and 4) effect over an average of six months after 91% of injections. In 9% the injections had a moderate (grade 2) effect. Patients with hyperlacrimation showed a nearly complete recovery. There were no systemic side effects but focal side effects due to a temporary weakness of the orbicularis oculi muscle were not uncommon. CONCLUSIONS: Botulinum toxin type A is the treatment of choice in motor and autonomic effects of aberrant regeneration of facial nerve after a peripheral palsy. The required dose is similar to or slightly lower than the dose usually recommended for hemifacial spasm.
Explore the source record for details and available documents.