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Biomedical subjects

W F Haupt

Publications and source records attributed to W F Haupt.

At least 19 recordsLinked to original sources
PubMed2006

[Glycogenesis Type II (M. Pompe). Selective failure of the respiratory musculature--a rare first symptom].

Late-onset Pompe's disease, a generalized lysosomal glycogen storage disease caused by acid maltase deficiency, usually presents as a slowly progressive muscular weakness of proximal muscles in lower limbs, followed by involvement of respiratory muscles. In the case presented here, however, respiratory failure was the first and selective symptom which caused the uncommon appearance of a patient entering our outpatient clinic on foot carrying his own artificial respirator. Intercostal muscle biopsy eventually led to the diagnosis.

Adult↗
PubMed2006

[Measuring fatigue in patients with multiple sclerosis with standardized methods in German speaking areas].

Fatigue describes the presence of a pronounced and advanced state of weariness. People with fatigue need more energy and it takes more effort to perform different activities than expected when compared to the patients disability. Fatigue can be observed in up to 92 % of patients suffering from multiple sclerosis. In the presented study, the German fatigue severity scale (dFSS) was established following the English "Fatigue Severity Scale". We enrolled 20 patients suffering from a primary relapsing multiple sclerosis and compared them to 20 healthy controls. Fatigue was detected if at least 4 points were reached in the dFSS. The dFSS demonstrated high validity and reliability. The dFSS is able to differentiate patients with fatigue from healthy controls. As consequence, the dFSS can be used to evaluate fatigue in German speaking individuals. The presented data demonstrated a good internal consistence. The scale is able to measure fatigue in an economic and rapid fashion. Therefore, it can be used in clinical situations for measuring fatigue in German speaking individuals.

Adult↗
PubMed2006

Gender change and its impact on the course of multiple sclerosis.

We report the case of a 22-years old genotypic women suffering from a relapsing-remitting multiple sclerosis (MS) according to the Poser criteria. In this patient, a gender change had been performed by androgen-supplementation and surgical intervention. During gender change, the patient experienced further relapses. Different immunomodulatory and immunosuppressive treatment strategies did not stabilise the course of MS in this patient. Actually, an escalating therapy with mitoxantrone has been initiated. During the observation period the patient received long-term testosterone-supplementation. Testosterone levels were elevated in the serum of this genotypic female MS patient under such a hormonal treatment compared to normal ranges before. The clinical course of the patient is presented in this case. As there are several studies investigating an immunomodulatory impact of hormones on the course of MS or experimental allergic encephalomyelitis, we discuss the presented case and a possible influence of androgens in this patient.

Adult↗
PubMed2005

[Complications and diagnostic limitations of chronic meningitis].

HISTORY AND ADMISSION FINDINGS: A 37-year-old woman was admitted with total loss of vision of the left eye within 24 hours. Additionally, she complained about fatigue, headache, chills, fever, muscle pain and neck stiffness since 4 days. At admission, the body temperature was 38.7 degrees C. Neurological examination revealed papilledema and meningism. INVESTIGATIONS: Ophthalmologic findings were consistent with a papillitis. The vision was lost, the pattern-shift checkerboard visual evoked potentials were not measurable. MRI of the brain and the optical nerve was without pathological findings, meningeal or cerebral Gadolinium enhancement was not present. The CSF analysis yielded a lymphocytic meningitis with 249 cells/mm (3), the glucose ratio of cerebrospinal fluid and serum was normal. DIAGNOSIS, TREATMENT AND COURSE: The papillitis was treated unsuccessfully with high-dose methylprednisolone, the left eye remained blind. Persistence of the pleocytosis under initial treatment with aciclovir and ceftriaxone, reduction of the glucose ratio of cerebrospinal fluid and serum and intrathecal immunoglobuline A -- synthesis required a change of the diagnostic and therapeutic regimen. Various common and rare differential diagnoses were considered and ruled out, a chronic meningitis of unclear aetiology with the complication of amaurosis was diagnosed. In consideration of the most probable diagnosis, a tuberculostatic therapy was initiated. A prolonged reduction of the pleocytosis and normalization of cerebrospinal fluid parameters could be observed. CONCLUSIONS: A large number of aetiologies can cause chronic meningitis; this case report reviews the most important differential diagnoses and highlights the limitations of the diagnostic work-up although various methods are available. Clinical course and symptoms of chronic meningitis are mild to moderate and may even be absent, but it can cause severe complications.

Adult↗
PubMed2004

Long-term persisting interferon beta-1b neutralizing antibodies after discontinuation of treatment.

OBJECTIVES: Neutralizing antibodies (NAB) against interferon beta (IFNB) with presumably negative impact on treatment outcome have been described in up to 42% of patients undergoing IFNB treatment. However, in most cases NAB decrease despite continuation of IFNB therapy. Observations on NAB after discontinuation of IFNB therapy are lacking. Here, we report for the first time on NAB which now persist for several years following discontinuation of IFNB treatment. MATERIALS AND METHODS: We present two multiple sclerosis patients followed over 8 and 10 years. NAB have been measured repeatedly and are presented together with clinical data. NAB developed after 2 years of treatment and persisted despite discontinuation of treatment at high titers for more than 4 years. CONCLUSION: Our data indicate that IFNB therapy may induce long-term NAB production which persists even after discontinuating IFNB treatment. Possible immunological mechanisms are discussed.

Adjuvants, Immunologic↗
PubMed2003

Early antithrombotic prophylaxis with low molecular weight heparin in neurosurgery.

BACKGROUND: Despite the high risk of venous thromboembolic events (VTE) in neuro-surgical patients, heparin prophylaxis has not been routinely established due to concern about bleeding complications. After initiating early low molecular weight heparin (LMWH) prophylaxis, we reviewed our patients in order to examine the viability of this practice. METHOD: Over a 3 year period, the records of patients admitted for elective neuro-surgery (ES), head injury (HI) or spontaneous intracranial haemorrhage (ICH) were analysed. Prophylaxis was performed with certoparin (3000 U anti-factor Xa s.c.) on the evening before ES and within 24 hours after surgery or admission whenever a CT did not show a progressive haematoma. Contraindications for LMWH were prothrombin time <70%, partial thrombo-plastin time >40 s, platelet count <100.000/ml, and platelet aggregation test sum <60%. The incidence of bleeding complications, VTE, and resulting morbidity/mortality was assessed. FINDINGS: 294 patients were admitted for ES, 344 for HI, and 302 for ICH. 155 of these were excluded because of contraindications. Intracranial bleeding was recorded in 1.5% (ES 1.1%, HI 3.5%, ICH 0%) and operative revision was performed in 1.1% (ES 0.7%, HI 2.8%) of patients. One case of moderate disability and no mortality occurred. The incidence of VTE and pulmonary embolism was documented in 0.2% and 0.1% of patients, with no associated mortality. No heparin induced thrombocytopenia was observed. INTERPRETATION: In neurosurgical patients, antithrombotic prophylaxis with certoparin was determined to be safe and efficacious when contraindications are carefully considered and a 12-hour time interval before and after surgery was guaranteed. This retrospective analysis should encourage a prospective trial of early LMWH prophylaxis.

Adult↗
PubMed2002

[Transient neonatal myasthenia gravis].

Ten to twenty percent of the offspring of mothers suffering from myasthenia gravis (MG) also develop transient neonatal MG, since maternal antibodies are able to cross the placenta. We report the course of two newborns of a mother with MG and a healthy father. The first pregnancy was complicated during the 3rd trimester by a hydramnion. The newborn presented with generalized muscle weakness, respiratory distress, weak sounding, anaemia, and poor sucking. Mechanical ventilation was necessary. Confirmation of the diagnosis was achieved by the result of repetitive muscle stimulation, showing a typical decrement in the EMG, and measurement of serum antiacetylcholin receptor antibodies. For 3 months, the infant was treated with neostigmin (cholinesterase inhibitor). After 26 days of hospitalization, the patient was released and followed up regularly. Myasthenic symptoms completely resolved. Side effects of the treatment were not observed. The course of the second pregnancy was normal. This second newborn was healthy. Our case report is remarkable for the very different presentation of two children of the same mother with MG during pregnancy and after delivery, with one child developing severe transient neonatal MG, initially requiring intensive care unit (ICU) treatment followed by quick recovery, and one child being healthy. We also present a score for monitoring the clinical course and adjusting anticholinesterase therapy accordingly.

Adult↗
PubMed2002

[The diagnosis of brain death: medical and legal aspects with special reference to the German Transplantation Law (TPG)].

The diagnosis of brain death following total and irreversible cessation of all cerebral functions is based on anthropological assumptions and conventions as well as on the exact medical diagnosis of total loss of brain function. The question whether individual life ends after cerebral function is irreversibly lost cannot be answered by medical definition alone. Clear and unrefutable legal definitions of death and the cessation of the rights of the individual must be provided before organs may be harvested from brain dead individuals. Acceptance of these definitions by the general population is of paramount importance for the practice of organ donation. In the first part of this article, the legal definition of death and the provisions of the German transplantation law are critically reviewed. The legal statements deal with the question of the definition of death and how death can be detected. The provisions of the German transplantation law are referenced with special attention to the provision of prior consent to the removal of organs following after the diagnosis of brain death. The provisions of the German constitution with respect to the preservation of the personal rights of the individual are discussed in the light of organ harvesting. The second part deals with the medical procedure of determining brain death in adults. The medical statements pertain to the diagnostic steps to be taken in the diagnosis and determination of brain death. The prerequisites for entering the diagnostic procedure to determine brain death are described. The clinical signs of total and irreversible cessation of brain function are listed, and the technical examinations to corroborate the clinical signs of brain death as accepted in Germany are delineated. In the perspective of the authors, individuals having suffered brain death still possess the protection of their personal human rights according to the German constitution since it cannot be conclusively demonstrated that total loss of brain function alone constitutes the cessation of life in the sense of the German constitution.

Brain Death↗
PubMed2001

Bell's palsy: electrodiagnostics are not indicative of cerebrospinal fluid abnormalities.

Electrodiagnostic testing (electromyography, electroneuronography, and blink reflex) and cerebrospinal fluid (CSF) examination (cell count, immunoglobulins, and antigen-specific intrathecal immunoglobulin G synthesis against herpes simplex virus, varicella zoster virus, cytomegalovirus, and Borrelia burgdorferi sensu latu) were performed in 56 patients with Bell's palsy. The CSF was normal in 45 patients and abnormal in 11 patients. Acute borreliosis was the most common specific pathological CSF finding (4 of 11). Electromyography revealed abolished volitional activity in 22% of patients with normal CSF and in 36% with pathological CSF. Electroneuronographic tests with an amplitude decrease of more than 90% on the affected side or abolished responses were found in 20% of patients with normal CSF and in 18% with pathological CSF. Abolished orbicularis oculi reflexes were seen in 67% of patients with normal CSF and in 82% with pathological CSF Concerning electrodiagnostic testing, no statistically significant difference between patients with normal and abnormal CSF was found, so we conclude that electrodiagnostic testing has no indicative value for abnormal CSF in Bell's palsy.

Action Potentials↗
PubMed2000

Apheresis and selective adsorption plus immunoglobulin treatment in Guillain-Barré syndrome.

Plasma exchange (PE) and administration of intravenous immunoglobulin (i.v. IgG) are established treatments for Guillain-Barré syndrome (GBS). Combination treatment with elimination of postulated pathogenetic factors by PE or selective adsorption (SA) treatment and subsequent immunomodulation by intravenous IgG may provide a more effective treatment. In a single-center randomized study, 45 patients with acute GBS were prospectively examined using a clinical score. We treated 11 patients by PE, 13 with SA using a tryptophan-linked polyvinyl alcohol gel adsorbent, and 21 with SA 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. The results suggest that combination treatment of GBS may be superior to plasma treatment alone.

Acute Disease↗
PubMed2000

Immunoadsorption in Guillain-Barré syndrome and myasthenia gravis.

Elimination of circulating antibodies by hemapheresis is an empirical treatment concept in various neuroimmunological diseases. Plasma exchange (PE) has been shown to be superior to symptomatic treatment in Guillain-Barré syndrome (GBS) in two large multicenter studies. It is also effective in myasthenia gravis (MG), although no comparative studies have been performed. Immunoadsorption (IA) using polyvinyl alcohol gel columns to which phenylalanin (IM-PH) or tryptophan (IM-TR) are covalently bound is an alternative to PE, and seems to have equal efficacy and comparable side effects. This method also obviates the need for replacement of plasma with human albumin or plasma. We compared the treatment results of 11 patients with GBS treated by PE to those of 13 patients treated by IA using an IM-TR column. Here, we found no statistically significant differences with regard to efficacy and clinical or procedural complications. From these data we conclude that immunoadsorption can be used as an equal alternative to PE. A large multicenter study comparing PE, intravenous immunoglobulins (IVIG), and the combination of both in the treatment of GBS revealed no significant difference between the 3 treatment groups. In MG, only 2 small studies have been performed using IA, and no studies comparing PE or other treatments to IA have been conducted. Both investigations of IA therapy demonstrated a marked reduction in the acetylcholine receptor (AchR) antibodies and a sustained improvement of the clinical signs. These results therefore show that IA is an effective treatment for myasthenia gravis.

Guillain-Barre Syndrome↗
PubMed2000

Recent advances of therapeutic apheresis in Guillain-Barré syndrome.

Acute idiopathic demyelinating polyneuritis (AIDP), commonly known as the Guillain-Barré syndrome (GBS), is an immune-mediated demyelinating disease of the peripheral nerve and nerve roots. A number of immunological mechanisms were described, but the exact pathomechanism has not been explained fully. Presumably, a variety of immunological processes lead to a relatively uniform clinical phenotype. Two large multicenter studies showed that plasma exchange (PE) was significantly superior to supportive treatment only. Selective adsorption (SA) also was employed as a method of therapeutic apheresis, and various smaller studies established that both PE and SA are equally effective treatments for GBS. Recently, it was demonstrated that the number of apheresis treatments should be adapted to the severity of disease. A large multicenter controlled study established equal efficacy of PE and intravenous immunoglobulin treatment (IVIg) as well as the combination of PE and IVIg. Since that time, the use of apheresis for the treatment of GBS declined in many countries due to the easier application of IVIg. The number of patients treated in larger hospitals with long-standing experience in the treatment of GBS also has declined.

Blood Component Removal↗
PubMed2000

Serial evoked potentials and outcome in cerebrovascular critical care patients.

Median-nerve evoked somatosensory evoked potentials (SEPs) and brainstem auditory evoked potentials (BAEPs), examined early in the course of patients suffering from cerebrovascular disease, correlate statistically significantly with outcome. Little is known about the changes of evoked potentials in the course of disease and their correlation to outcome. In a series of 215 patients (75 supratentorial infarctions, 36 infratentorial infarctions, 58 supratentorial hemorrhages, 18 infratentorial hemorrhages, and 28 aneurysmatic subarachnoid hemorrhages) requiring neurologic intensive care treatment, we prospectively examined the correlation between the findings of serial SEPs and BAEPs and outcome at 4 weeks. Evoked potentials were examined after admission, after 1 week, and after 2 weeks. The findings were classified in 4 categories (normal, unilateral or bilateral pathologic findings, unilaterally attenuated, and bilaterally attenuated). Clinical outcome was determined by classification according to the Glasgow Outcome Scale (death, persistent vegetative state, severely incapacitated, mildly incapacitated, and recovery). Statistical evaluation was performed using Fisher's exact test for all variables. In all subgroups, SEPs correlated statistically significantly with outcome at all three examinations. No correlation was found for BAEPs at first examination in infratentorial disease, nor at second examination in subarachnoid hemorrhages. In all other cases, SEPs and BAEPs were correlated statistically significantly with outcome at all three examination timepoints.

Adult↗
PubMed1999

[Hypokalemic paralysis in furosemide therapy and simultaneous laxative abuse].

BACKGROUND: Clinical signs of hypokalemia are not directly related to the extent of the electrolyte imbalance, and therefore monosymptomatic cases may be observed. CASE REPORT: Following an acute gastroenteritis with considerable diarrhea, a 47-year-old male patient was admitted to hospital for progressive painful paraparesis. Upon admission, the patient complained of painful paresthesias in both legs, and a moderate flaccid paraparesis with widespread fasciculations and loss of leg tendon reflexes was found. Serum potassium level on admission was 1.7 mmol/l. Other signs of hypokalemia were absent, and the ECGs showed a slow sinus rhythm without disturbances of de- or repolarisation or cardiac arrhythmias. Hypokalemic paralysis was diagnosed and was considered to be primarily drug-induced, as the patient had a history of laxative abuse and was on a continuous medication with furosemide (80 mg/d) without regular assessment of serum electrolytes. The additional electrolyte loss following the gastroenteritis precipitated the development of clinical signs of hypokalemia. In parallel to the rise in serum potassium levels, both painful paresthesias and muscle weakness disappeared, and electromyography documented the amelioration of the myopathic syndrome. CONCLUSION: The prominent clinical symptom of hypokalemia was a dyskalemic paralysis in the absence of other sequelae of electrolyte imbalance, such as cardiac arrhythmias or vegetative disturbances.

Cathartics↗
PubMed1999

European brain death codes: a comparison of national guidelines.

The majority of European countries have published recommendations for the diagnosis of brain death as a necessary prerequisite for organ donation. The concept of brain death as defining the death of the individual is accepted in all European countries; however, the guidelines for determining the total and irreversible loss of all brain functions differ somewhat in the various countries. While the clinical examination and documentation of the clinical signs of brain death are very uniform, there are significant differences in the guidelines for using technical confirmatory tests to corroborate the clinical signs. These range from rejecting all technical tests to accepting of multiple neurophysiological tests alone or in combination. The present study examined the similarities and differences in current concepts of brain death in the various European countries and their recommendations for the diagnosis of brain death. The diagnosis of brain death is based on a number of prerequisites, on the clinical diagnosis of deep coma, loss of all brainstem reflexes, and the demonstration of apnea. Neurophysiological tests are recommended by a number of national professional societies as confirmatory tests to confirm the clinical diagnosis of brain death and shorten otherwise necessary waiting periods of 6-12 h. Most brain death codes allow the use of electroencephalography, which must demonstrate electrocortical silence over a certain period. Evoked cerebral potentials can demonstrate the successive loss of activity of various afferent pathways and are accepted in some countries as a confirmatory test. Other neurophysiological tests which demonstrate the loss of cerebral perfusion can be implemented. Brain scintigraphy can confirm the loss of isotope uptake into the brain. Doppler sonography also demonstrates cessation of brain perfusion. Cerebral panangiography may also be used to demonstrate the loss of brain perfusion but is less desirable since it might endanger the patient.

Brain Death↗
PubMed1999

Brain death and organ donation in Germany: analysis of procurement in a neurosurgical unit and review of press reports.

OBJECTIVE: A prospective study was undertaken to determine the number of potential organ donors in a neurosurgical intensive care unit and to record the actual number of organ donations. The reasons for refusal of organ donations were analysed with respect to the controversial public discussion of the brain death concept and the transplantation law in Germany. METHODS: From 1994 to 1997, the number of cases of brain death, and the number of cases in which relatives consented or refused to organ donation as well as the number of actual organ explantations was recorded. Over the same period, press reports of 5 German language newspapers were analysed with regard to the authors' position on brain death and transplantation law, their qualification, intention, and mode of presentation. RESULTS: Annually, about 70 deaths occurred on the department's intensive care unit of which almost 30% of the patients were determined to have suffered brain death without any obvious contraindications against organ donation. The refusal rate of relatives rose from 56% up to 78%, and the rate of organ donors thus decreased from 5% in 1994 to 4% presently in the department. About 100 reports were published each year in the analysed newspapers, of which about 90% voiced approval of organ transplantation. During the review period, the depiction became more objective (57% in 1994 to 82% in 1997) and the suspicious attitude toward the technique of brain death diagnosis declined. Whereas 50% of the articles rejected the brain death concept in 1994, this figure decreased to 39% in 1997 and conversely the concept of consent rose from 33% in 1994 to 56% in 1997. CONCLUSION: In Germany, the relatives refusal rate in organ donation is continuously high, presumable due to a depressing 39% of press reports rejecting the brain death concept, and resulting in a very low number of organ donations. There is hope that the transplantation law which has finally passed German parliament in 1997, confirming the brain death concept as well as the legal principle of prior consent by the donor or consent by the relatives will eventually result in an increase of organ donation especially when supported by an educational campaign which is embodied in the transplantation law.

Adult↗
PubMed1999

Cerebral glucose metabolism in acute and persistent vegetative state.

Regional cerebral glucose metabolism (rCMRglc) was investigated with 18F-2-fluoro-2-deoxy-D-glucose (FDG) and positron emission tomography (PET) in 24 patients with acute (AVS, duration <1 month, n=11) or persistent (PVS, duration >1 month, n=13) vegetative state (VS) following prolonged anoxia due to cardiorespiratory arrest. After a follow-up period of twelve months, 8 patients had died, 13 remained in a permanent vegetative state and three showed moderate improvement of consciousness, without however regaining independence for activities of daily life. As expected, overall glucose utilization (CMRglc) was significantly reduced in VS in comparison to age matched controls. Infratentorial structures showed a less distinct hypometabolism. Differences in metabolic rates between patients who died or remained in a PVS were small and insignificant and probably reflect different age structures of the two groups. A statistically significant correlation between the degree of evoked potential or EEG alterations in VS and the reduction of global or regional cortical metabolic rates for glucose could not be established. Cortical metabolic rates in patients with PVS were significantly reduced when compared to patients studied in AVS (p<0.05 for all cortical regions of interest except the frontal lobe). This phenomenon reflects the progressive loss of residual cortical function following anoxic brain injury that corresponds to the neuropathological findings of progressive Wallerian and transsynaptic degeneration as sequelae of anoxic brain injury in PVS.

Activities of Daily Living↗