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Biomedical subjects

T Kuntzer

Publications and source records attributed to T Kuntzer.

At least 55 records · Page 3Linked to original sources
PubMed1998

Stimulated pressure profile at rest: a noninvasive method for assessing urethral sphincter function.

OBJECTIVES: To validate a method for assessing urethral sphincter muscle function by recording rises in intraurethral pressure during repetitive pudendal nerve stimulations. METHODS: A supine urethral pressure profile at rest was performed on 12 stress-continent and 28 stress-incontinent patients during repetitive pudendal nerve stimulations applied near the ischial spine, and the intraurethral pressure increases were calculated for each third of the urethral functional length. RESULTS: No significant difference in intraurethral pressure increases was seen between continent and stress-incontinent women. On the various regression curves, the intraurethral pressure increases showed a significant correlation with maximal urethral closure pressure values at rest and at stress (r = 0.36 to 0.54) and with the patient's age (r = 0.46), but not with pudendal nerve conduction times to the urethral sphincter on either side (r = 0.14 and 0.19). CONCLUSIONS: This method (1) measures intraurethral pressure increases that correlate well with the anatomic location of the urethral sphincter muscle, (2) shows there is no significant difference between them in continent and stress-incontinent patients, except in patients with a low-pressure urethra, and (3) demonstrates that they correlate well with the maximal urethral closure pressure and the patient's age, but not with pudendal motor latencies to the urethral sphincter. This method gives us a mapping of the urethral sphincter activity, explaining why some patients with a low-pressure urethra have less urinary loss than others with the same urethral closure pressure.

Adult↗
PubMed1998

Familial cardiomyopathy and distal myopathy with abnormal desmin accumulation and migration.

Desminopathies form a heterogeneous group of myopathies characterised by pathological aggregations of desmin. We report a family, where mother and daughter presented with an atrioventricular block and a slowly progressive distal muscular weakness, with non-homogeneous focal atrophy on computed tomography scans. The mother developed a severe global heart insufficiency necessitating a heart transplantation at 56 years of age. Skeletal muscle biopsies were characterised by inclusion bodies strongly expressing desmin and alpha B-crystallin, with a predominantly subsarcolemmal localisation. Ultrastructurally most inclusions corresponded to non-membrane bound granulo-filamentous material with disruption of myofibrils. An immunoblot showed a hyperintense desmin band at 53 kDa and a second band at 49 kDa, the latter being absent in controls. The cardiac muscle of the explanted heart showed very similar inclusions. These cases illustrate that in this distinct subtype of desminopathies the cardiac muscle alterations are comparable with those observed in skeletal muscle, and suggest the possibility of a primary desmin pathology.

Adult↗
PubMed1997

Clinical and prognostic features in unilateral femoral neuropathies.

We have examined the clinical features of patients with femoral neuropathy and the factors that influence the prognosis. Of 80 consecutive patients referred for neurophysiological evaluations of proximal lower limb weakness, 32 fulfilled strict inclusion criteria and had adequate information, including estimates of axon loss (AxL) by stimulation of the bilateral femoral nerve. In 31, the Kaplan-Meier method was used to describe the time course of the outcome, while logistic regression was employed to determine the contributing factors. Excellent, satisfactory, and poor outcomes were seen in 10 (31%), 11 (34%), and 10 (31%) patients, respectively. Logistic regression analysis of seven factors demonstrated that the estimate of AxL was the only significant variable. The best prognostic factor was an estimate of AxL < or = 50%, with all patients fulfilling this criterion showing improvement with 1 year; fewer than half the patients with AxL > 50% should be expected to improve. This study clearly shows that, irrespective of the cause of femoral neuropathy, functional improvement is seen in 2 out of 3 patients within 2 years and that the estimate of AxL is the only factor influencing prognosis.

Adult↗
PubMed1996

Stress urinary incontinence due to a low-pressure urethra: a socially invalidizing disease.

To compare the quality of life and clinical findings of patients with low-pressure urethra (LPU: < or = 20 cm H2O) with those of stress urinary incontinent (SUI) patients without LPU, and to compare the quality of urethral sphincter (US) muscle innervation parameters in LPU patients with those in control continent patients. Historical, clinical, urodynamic, and US muscle innervation parameters were compared in 38 LPU (group 1), 241 SUI (group 2), and 7 control patients (group 3). In comparison with group 2, the incidence of previous surgery and daily incontinence episodes, SUI severity, and pad test values were significantly higher in group 1, whereas the incidence of previous traumatic deliveries was the same in both groups. The mobility of the bladder neck assessed by the Q-Tip test was significantly reduced in group 1. In comparison with group 3, US motor unit potential (MUP) duration and pudendal motor latencies to the urethral sphincter (PMLUS) were increased in LPU group 1 patients, whereas there was no difference in these parameters between LPU patients with or without previous incontinence surgery. Fifty-three percent of our LPU patients had normal PMLUS, but showed signs of abnormal reinnervation on quantitative electromyography. Three nullipara LPU patients had normal MUP durations and PMLUS values, but a decreased area in response to pudendal nerve stimulation. Apart from a rare form of LPU in nullipara patients, probably due to a dysgenesis of their US muscle, LPU patients suffer from neuro-muscular damage responsible for a severe urinary invalidity. Previous incontinence surgery, as well as previous vaginal deliveries, may be responsible for such US damage.

Adult↗
PubMed1996

[Anti-glycoconjugate antibodies and dysglobulinemic or dysimmune peripheral neuropathies].

Different anti-glycoconjugate auto-antibodies are described to be associated with peripheral neuropathies; the anti-MAG monoclonal IgM activity is determined by ELISA or immunoblotting essays, anti-glycolipid activity by ELISA or chromatography and monoclonal gammopathies by commercially available kits. These investigations are limited by possible cross-reactions due to the common glycosilate epitope. The peripheral neuropathies with anti-glycoconjugate auto-antibodies are different: Guillain-Barré syndrome and its variants (anti-GM1, GQ1b, LM1, GD1, GD1a gangliosides or sulfatides), motor neuropathy (anti-GM1 ganglioside), sensory neuropathies (anti-GD3, GD1b, GD1a, GQ1b gangliosides or sulfatides), and chronic myelinic neuropathy with IgM gammopathy (anti-MAG). We review the major clinical and electrophysiological features and discuss the underlying pathophysiological mechanisms.

Autoantibodies↗
PubMed1996

[Peripheral nerve and spinal cord complication in intravenous heroin addiction].

The neurological complications observed in 6 HIV negative intravenous drug users are reported. Four developed acute neuromuscular involvement in a lumbosacral or brachial distribution with rhabdomyolysis, myoglobinuria, hypovolemia, renal and hepatic failure in the 3 most severely affected patients. Despite evidence of immunologic abnormalities and especially presence of anti-heroin antibodies, we feel that causative mechanisms include mixed compression and ischemia with an underlying toxic myopathy, resulting in segmental myopathy with secondary compression of peripheral nerves. Two patients developed myelopathy with acute or chronic onset. The mechanisms were vascular with spinal cord infarction in the acute form and probably infectious with secondary compressive arachnoiditis in the chronic form. In these 2 patients with myelopathy, outcome was poor.

Acute Disease↗
PubMed1995

[Myasthenia-like syndromes: current and future treatments].

Therapeutical aspects of disorders of the neuromuscular transmission are based on differential diagnosis between pre- or post-synaptically localized abnormalities of the neuromuscular junction, recognition of acquired and congenital forms and search for associated diseases and tumors, on prescription of drugs improving the transmission of acetylcholine and immunosuppressive agents, and on regular and quantified follow-up. In this clinical and therapeutic review, the two most common forms, myasthenia gravis and the Lambert-Eaton myasthenic syndrome, are discussed in the light of the 44 patients recently seen in our department.

Adult↗
PubMed1995

[Meningoencephalo-myeloradiculitis due to Flavivirus: bi-brachial paralysis and respiratory insufficiency].

3 patients developed rapid onset of fever and nuchal stiffness. Paresis of brachial muscles occurred within 4 days and all patients had respiratory failure that needed mechanical ventilation. At the peak of the disease there were bilateral asymmetrical severe atrophy of brachial, shoulder and neck muscles, cranial nerve pareses and absent or weak deep reflexes in the upper extremities. CSF analyses showed sterile lymphocytic pleocytosis. In 2 cases the patients suffered a tick bite in Switzerland and the third was probably bitten by an insect while opening a package received from Indonesia. Patients had rapid defervescence and serological tests were found to be highly positive for IgM and then IgG ELISA FSME (Frühsommer-Meningoenzephalitis). The patients were ventilated for 2 to 5 weeks before a progressive improvement was seen. However, on follow-up at 12, 18 and 30 months respectively, proximal muscles were still atrophied and quite weak. Our cases underline that: (1) FSME-ELISA results may cross-react with the Japanese and Central European encephalitis virus species; (2) Flaviviruses do induce unusual and preferential long-term paralysis of the upper extremities simulating poliomyelitis; (3) in the 2 patients studied electrophysiologically, there were signs of axonal reinnervation not seen in lower motor neuron syndrome which were important for reinnervation to permit progressive, but late, motor improvement; (4) there is no evidence of extension of the endemic foci of tick-borne encephalitis in Switzerland.

Adult↗
PubMed1995

Chronic relapsing neuropathy associated with Castleman's disease (angiofollicular lymph node hyperplasia).

We report a 17-year-old patient who presented a chronic relapsing sensorimotor demyelinating neuropathy with 6 relapses over a 7-year period, preceding by 4 years the diagnosis of a multicentric angiofollicular lymph node hyperplasia. A role for Epstein-Barr virus (EBV) as a trigger of the neuropathy may be suggested by the presence of EBV DNA in the biopsied abdominal abdominal adenopathies. This unusual reported association seems to have a better prognosis than the known chronic progressive form of neuropathy associated with Castleman's disease and the Crow-Fukase syndrome.

Adolescent↗
PubMed1994

Carpal tunnel syndrome in 100 patients: sensitivity, specificity of multi-neurophysiological procedures and estimation of axonal loss of motor, sensory and sympathetic median nerve fibers.

This prospective study meets all six criteria recently recommended by a quality assurance committee of the AAEM and defines criteria of abnormality, sensitivity and specificity of 19 sensorimotor and sympathetic parameters in 100 patients who were suspected on clinical grounds of having carpal tunnel syndrome (CTS), and in 70 control subjects. Nine parameters reached a specificity of 97%, permitting the electrodiagnosis of CTS in 87% of the patients studied. The results in this study confirm that median sensory nerve conduction studies are more frequently abnormal than are studies of motor nerve conduction. The so far unknown usefulness of parameters such as median F-wave abnormalities and residual latency, terminal latency index and sensory nerve action potential (SNAP) amplitude was assessed; these parameters were not found sensitive enough, yet high specific (SNAP amplitude) or high sensitive yet low specific (F-wave abnormalities, residual latency and terminal latency index) and are therefore of little value in the early clinical electrodiagnostic evaluation of patients with CTS. Finally, in the patients studied, some degree of axonal loss for motor, sensory and sympathetic median nerve fibers was found in 42% of cases and 6 patients had a double-crush syndrome and 6 others had a concomitant ulnar neuropathy at the elbow.

Action Potentials↗
PubMed1994

[Range of neuromuscular involvement in 47 patients infected with the human immunodeficiency virus].

Over a 30 month period, 47 out of 749 patients infected with the human immunodeficiency virus had various neuromuscular symptoms. Based on clinical and electrophysiological data, 47% had distal symmetric polyneuropathy, 11% chronic inflammatory demyelinating polyneuropathy (CIDP), 8.5% toxic neuropathy related to 2-3-dideoxyinosine (DDI), 8.5% cranial neuropathy, 8.5% mononeuropathy multiplex or isolated focal neuropathy, 8.5% progressive lumbosacral polyradiculopathy, and 8.5% myopathy. Half of the patients exhibited previous or concomitant signs of central nervous system involvement and 18 patients died during the study period. CIDP and cranial neuropathies usually appeared early in the course of the disease and consequently showed neurological improvement. Nerve conduction studies of DDI related toxic neuropathies showed distal axono-myelinic sensitivo-motor neuropathy, differing from CIDP by the absence of a conduction block. Distal symmetric polyneuropathies, frequent in the advanced systemic illness, do not systematically require an extended workup, but more unusual peripheral neuropathies which might be treatable necessitate further investigations (electromyography, radiology, serological blood tests; protein chemistry and routine workup of the cerebrospinal fluid). For example, progressive lumbosacral polyradiculopathies responded to early treatment, with a better outcome in one case of herpetic origin than in another case due to cytomegalovirus infection. Our observations suggest that myopathies in HIV infected patients should first be tackled by temporary interruption of virostatic medication, followed by muscle biopsy if the symptoms persist.

AIDS Dementia Complex↗