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W F Haupt

Publications and source records attributed to W F Haupt.

At least 19 recordsLinked to original sources

Composition and biophysical properties of myelin lipid define the neurological defects in galactocerebroside- and sulfatide-deficient mice.

Oligodendrocytes and Schwann cell-specific proteins are assembled with a highly ordered membrane lipid bilayer to the myelin sheath of axons, which functions as an insulator and allows rapid saltatory conduction. We approached the question of the function of the CNS and PNS myelin-specific galactospingolipids cerebrosides and sulfatides by generating a ceramide galactosyltransferase null allelic mouse line (cgt-/-). Galactocerebroside- and sulfatide-deficient myelin loses its insulating properties and causes a severe dysmyelinosis that is incompatible with life. Here, we describe the biochemical and biophysical analysis of the myelin lipid bilayer of cgt-/- mice. The lipid composition of CNS and PNS myelin of cgt-/- mice is seriously perturbed and the sphingolipid biosynthetic pathway altered. Nonhydroxy and hydroxy fatty acid-substituted glycosylceramides (GlcC) are synthesized by oligodendrocytes and sulfated GlcC in addition in Schwann cells. The monogalactosyldiglyceride fraction is missing in the cgt-/- mouse. This new lipid composition can be correlated with the biophysical properties of the myelin sheath. The deficiency of galactocerebrosides and sulfatides leads to an increased fluidity, permeability, and impaired packing of the myelin lipid bilayer of the internodal membrane system. The loss of the two glycosphingolipid classes causes the breakdown of saltatory conductance of myelinated axons in the cgt-/- mouse.

Animals

[Brain death].

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Brain Death

[Clinically pure motor variant of Guillain-Barré polyneuritis. Report of 3 cases].

We report three patients with severe Guillain-Barré syndrome (GBS). On clinical examination, no sensory deficit was elicited. Electromyography (EMG) and motor nerve conduction velocity (NCV) studies were indicative of an axonal lesion. In two patients, we found total peripheral conduction block without volitional EMG activity. Sensory NCV and cortical median nerve evoked potentials remained normal. Sural nerve biopsy revealed unequivocal alterations of sensory nerve fibers, some in the form of primary demyelination. Therefore, these cases must bei classified as combined motor-sensory syndromes despite the clinical and electrophysiological findings. Although total denervation of the extremity muscles occurred, causing tetraparalysis, this manifestation appears to be attributable to the secondary axonal variant. In contrast to positive reports in literature, immunomodulatory treatment proved ineffective in all three cases.

Aged

Sequential treatment of Guillain-Barré syndrome with extracorporeal elimination and intravenous immunoglobulin.

Plasma exchange (PE) and administration of intravenous immunoglobulin (IgG) are established treatments for Guillain-Barré syndrome (GBS). Elimination of postulated pathogenetic factors by plasma exchange or similar methods, such as selective adsorption (SA) treatment using affinity-type adsorption columns and subsequent immunomodulation by intravenous IgG, may provide a more effective treatment. Forty-five patients with acute GBS were prospectively examined using a clinical score. We treated 11 patients by plasma exchange, 13 with selective adsorption using a tryptophan-linked polyvinyl alcohol gel adsorbent, and a group of 21 patients by selective adsorption followed by intravenous IgG. The patients treated sequentially by selective adsorption and intravenous IgG improved significantly better than the patients who received plasma treatment only. This pilot study suggests that sequential treatment of GBS may be superior to plasma treatment alone. The higher cost of this combined treatment might be offset by shorter hospital stays and lower overall expenditure. The preliminary results warrant further investigation in a multicenter trial.

Adult

Neurogenic lesions after posterolateral thoracotomy in young children.

In follow-up examinations of young patients we often noticed upward displacement of the left shoulder in cases where left-sided posterolateral thoracotomy had been carried out. Finding little discussion of this side-effect in the literature, we undertook the present study. The shoulder girdle of 69 patients who had undergone surgery for coarctation of the aorta between 1964 and 1984 was carefully examined to assess displacements and dysfunctions. In addition, in 24 of the patients electrodiagnostic examination was carried out of the associated muscles and nerves which were affected by the operation. We found disturbances of the muscular apparatus of the shoulder girdle in 80% of the cases: upward displacement of the shoulder (55%), downward displacement of the shoulder (8.7%), deviation of the scapula at rest (72.5%) and maximal elevated arms (40.6%), scapula alata (56.5%), and alterations of the posterior axillary line (39.1%). When considering only the cases of distinct upward or downward displacement of the shoulder, at least 25% of the operated children were affected. Children operated early (surgery during the first year of life) had more distinct alterations (47.6%) than those with later surgery (14.6%). Electromyographic examinations indicate that mainly disorders of the peripheral nerves caused by the operation led to these alterations of the shoulder girdle musculature. Although, except for one patient, no functional disorders were found, there were several cases in which the alteration caused quite severe cosmetic problems. One should pay more attention to positioning for surgery and do careful preparation to reduce these sequelae.

Age Factors

[The MR tomographic diagnosis of intra- and paraspinal abscesses].

PURPOSE: Analysis of the MRT signals and their extent from intra- and paraspinal abscesses with reference to predisposing factors, their causes and localisation. PATIENTS AND METHODS: The histories and MRT findings in 34 Patients with intra- and paraspinal abscesses were evaluated retrospectively. Most of the patients (24/34) were older than 50 years. A second peak was below 30 years. RESULTS: 27/34 patients had some underlying disease which predisposed to infection, e.g., diabetes mellitus. The most common causal organisms were Staph. aureus (53%) and streptococci (15%). In 23/34 cases (68%), the abscesses were in the thoraco-lumbar or lumbar region, while only 6/34 occurred in the upper two-thirds of the thoracic spine and only 5/34 in the cervical region. In only 8/34 was the abscess confined to two vertebral lengths; in the remaining patients it was much more extensive. Intraspinal abscesses were about twice as large as the vertebral components and 1.5 times greater than paravertebral abscesses. The age of the abscesses could be estimated approximately from the signals. CONCLUSIONS: Contrast enhanced MRT permits detailed analysis of the compartments and exact estimation of the extent of the lesions and permits accurate monitoring of treatment.

Abscess

[Damage to the lumbosacral plexus in psoas hitch operation].

The psoas hitch operation is used for the management of ureteral disease requiring replacement of varying lengths of the ureter. During this procedure the bladder is fixed to the psoas by 2 or 3 hitches of nonresorbable suture. Astonishingly, peripheral nerve injury to the lumbosacral plexus has not yet been described as a complication even though it has been seen when the psoas muscle was hitched unintentionally during abdominal surgery. Within 14 days we saw 2 patients with damage to parts of the lumbosacral plexus following a psoas hitch. In one case, hypesthesia and exercise-related pain in the areas supplied by the genitofemoral, the ilioinguinal and femoral nerve persisted, suggesting inclusion of at least parts of those nerves in the hitch. Initial paresis of hip flexion did not persist and the second patient recovered without sequelae. According to the literature this kind of complication does not occur if visible nerves are avoided and stitches an used that are no deeper than 3 mm into the psoas muscle.

Adult

[Monitoring somatosensory evoked potentials during carotid endarterectomy].

The tromboembolic and ischemic events during carotid endarterectomy can be avoided or detected with appropriate monitoring. Median nerve somatosensory evoked potentials recorded from the parietal cortex correlate with the blood flow in the middle cerebral artery. The good evoked responses after cross-clamping of the carotid artery indicate a sufficient collateral circulation, enabling a surgery without shunt, thus minimizing the risk of embolisation. Insufficient collateral circulation after cross-camping results in an amplitude reduction of the parietal N20-P25 complex of more than 50%. In this case an ischemic event can be prevented by shunting. As a total 83 carotid endarterectomies were done. In 65 cases (78.3%) the evoked potentials showed no significant alteration, and no postoperative neurological deficit occurred. Seven patients (8.45%) needed to be operated with a shunt, because of cross-clamping ischaemia. One of them presented a transient postoperative hemiparesis, which was predicted by the long-term loss of the SEP-s, and which resolved within 4 hours. Seven further patients (8.45%)--operated primarily with shunt, and 4 patients (4.8%)--monitored with transcranial Doppler sonography, showed no postoperative neurological deficit. We found that median nerve somatosensory evoked potential monitoring during carotid endarterectomy is a simple, sensitive and reliable method.

Arteriovenous Shunt, Surgical

[Neuralgic amyotrophy (Parsonage-Turner syndrome) following streptokinase thrombolytic therapy].

HISTORY AND CLINICAL FINDINGS: A 61-year-old man was given short-term thrombolytic treatment with streptokinase (1.5 million IU) 6 hours after the onset of anginal symptoms. Ten days later he complained of severe pain in the right upper arm and right shoulder, and also on the left 3 days later. Simultaneously there occurred severe atrophic pareses of the supra- and infraspinatus, deltoid and rhomboid muscles bilaterally, without sensory component. TESTS: When admitted to the neurological department 3 weeks later laboratory tests, immunological parameters, electrophoresis and cerebrospinal fluid were unremarkable. Radiological examinations of the cervical and thoracic vertebrae demonstrated moderate degenerative changes. Computed tomography and magnetic resonance imaging of the cervical vertebrae showed small disc protrusion and narrowing of the intervertebral foramina, but they could not explain the clinical picture. Electromyography demonstrated a bilateral peripheral neurogenic lesion without evidence of radicular distribution or any lesion of individual peripheral nerves. These findings led to the diagnosis of neuralgic amyotrophy (Parsonage-Turner syndrome). TREATMENT AND COURSE: With analgesics and physiotherapy the pareses slowly regressed over the following months. CONCLUSIONS: The infusion of streptokinase was the only probable cause found to explain the neuralgic amyotrophy, a connection that has never been reported until now.

Brachial Plexus Neuritis

Prognostic value of evoked potentials and clinical grading in primary subarachnoid haemorrhage.

In a retrospective study of 64 patients suffering from primary subarachnoid haemorrhage (SAH), the clinical grading according to Hunt and Hess as well as the initial findings of brainstem auditory evoked potentials (BAEP) and median-nerve somatosensory potentials (SEP) were correlated with each other and with disease outcome to determine the prognostic value of evoked potential testing in comparison to the initial clinical grading according to Hunt and Hess. All patients were treated in a neurological intensive care unit. Normal evoked potentials usually indicate a favourable course. Alterations of SEP and BAEP increase in parallel with the severity of clinical findings. Unilateral or bilateral loss of SEP or BAEP indicates a poor prognosis. Clinical and electrophysiological findings show a close correlation, but only BAEP provide prognostic information beyond Hunt/Hess grading. In SAH patients, clinical grading was well as evoked potentials correlate significantly with outcome. Use of both clinical and EP rating improves prognostic accuracy.

Adult

Pilocytic astrocytomas of the posterior fossa. A follow-up study in 33 patients.

The extent of resection in pilocytic astrocytoma of the posterior fossa often remains undefined and the indications for further treatment in incompletely resected tumours are a matter of debate. It has been also realized that the problem of hydrocephalus in patients with pilocytic astrocytoma of the posterior fossa has not yet been solved and the diagnostic impact of postoperative CT findings remains questionable. We retrospectively reviewed the data from 33 patients harbouring a pilocytic astrocytoma of the posterior fossa to evaluate the impact of surgical technique in terms of radicality and of postoperative imaging results upon prognosis and adjunctive treatment. In addition, the issue of hydrocephalus was considered and related to different treatment modalities. Thirty patients underwent surgical treatment whereas 3 had open biopsy of the tumour. Macroscopically gross total resection of the tumour was performed in 20 patients, whereas resection was partial in 10. Follow-up was obtained in 29 patients for a period which ranged between 2 and 184 months (85 months +/- 56 months). Outcome was good in 24 patients who had only slight neurological deficit and poor in 3 patients, who were severely disabled. Two patients died during the follow-up period. Recurrent tumour growth occurred in 2 cases with incompletely resected tumours. From the series presented, it was concluded that long-term follow-up with CT seems mandatory in cases with contrast-enhancing residual tumour. Recurrent tumour growth should be assumed in postsurgical patients with an enlarging area of enhancement shown in follow-up CT studies. Permanent ventriculoperitoneal shunting is required in certain patients with pre- or postoperative hydrocephalus.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Intraoperative recording of parietal SEP can miss hemodynamic infarction during carotid endarterectomy: a case study.

Serial recording of median nerve somatosensory evoked potentials (SEPs) provides reliable intraoperative information on critical lowering of brain perfusion which compares favorably with other monitoring methods. However, single-channel parietal SEP recording may not detect hemodynamic cerebral infarctions affecting the pre-rolandic area. Following left carotid endarterectomy for high-grade symptomatic stenosis in a 66-year-old male, predominantly motor hemiparesis and aphasia were detected which were largely reversible. Pre- and intraoperative SEPs were normal. Postoperative SEPs showed significant interhemispheric amplitude differences. Postoperative CT examinations demonstrated hemodynamic watershed-type infarctions. This rare complication of carotid endarterectomy was not detected by intraoperative single-channel parietal SEP monitoring.

Aged

Visual evoked potentials in Alzheimer's disease: investigations in a PET-defined collective.

Intravital diagnosis of Alzheimer's disease (AD) based clinical and neuropsychological findings alone does not allow sufficient differentiation from other disorders such as vascular dementia. Positron emission tomography (PET) secures intravital diagnosis with high accuracy by determination of a typical metabolic pattern. Numerous publications have proposed visual evoked potentials (VEP) for the detection of AD. In a collective of 15 patients with probable AD according to NINCDS/ADRDA criteria and the typical metabolic pattern of AD, we examined pattern reversal and flash VEPs. The results were compared to those of an age-matched collective of patients without impairment of memory. Neither pattern reversal nor flash VEP were correlated to severity of dementia as determined by the mini-mental status examination (MMSE). The positive results of other investigators are probably due to different patient selection criteria which do not include findings of cerebral glucose metabolism in the definition of AD.

Aged

Long-term results of carpal tunnel decompression. Assessment of 60 cases.

The long-term prognosis of 60 patients operated on for carpal tunnel syndrome has been assessed in a prospective study with a median follow-up period of 5.5 years (range 2-11 years). Analysis of motor, sensory, trophic, and electrodiagnostic findings and assessment of pain were performed pre- and post-operatively using a standardized grading system. The results were generally favourable with a variable degree of improvement in 86% of cases. Statistical evaluation using multiple Dunn-Rankin tests revealed pain to be the most prominent pre-operative finding. On post-operative re-examination, pain was found to be improved to a significantly greater extent than any other variable. Analysis of several potential prognostic factors showed that pain lasting for more than 5 years prior to surgery indicates a poor prognosis. Only patients with diabetes mellitus exhibited a trend toward less pain relief.

Carpal Tunnel Syndrome

[The prognostic value of AEP and SEP values in subarachnoid hemorrhage. An analysis of 64 patients].

In 64 patients with confirmed subarachnoid haemorrhage the clinical grading according to Hunt and Hess was compared to the initial findings of BAEP und SEP to elicit possible prognostic statements. Clinical and electrodiagnostic findings showed a high correlation. Patients without or with only slight alterations of amplitude and/or latency generally showed a favourable course. Recurrent haemorrhages or internal complications which can be decisive for outcome cannot be predicted by clinical or electrodiagnostic findings. The alterations of BAEP and SEP increase parallel to severity of clinical findings. Bilateral loss of BAEP or SEP indicates poor prognosis. Prognostic statements can be made with certainty only in cases with primary poor clinical condition (Hunt-Hess Grade IV-V) and marked alterations of evoked potentials.

Adult