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Biomedical subjects

J Finsterer

Publications and source records attributed to J Finsterer.

162 records · Page 9Linked to original sources

Turn/amplitude parameter changes during sustained effort.

Little is known about the effect of sustained effort on turn/amplitude parameters. Therefore, the turn/amplitude analysis was applied at contractions with 50 +/- 5% of the right biceps brachii muscle's maximum force to 31 healthy human subjects (19-67 years old), 15 patients with neuropathies (30-83 years old) and 15 patients with myopathies (16-66 years old). After 3 min of sustained contraction, turns/second (T/S) decreased by 50% in healthy human subjects and by 25% in patients with neuropathies and patients with myopathies. The amplitude/turn (A/T) increased by 25% in healthy human subjects and remained almost unchanged in pathological cases. Compared to its onset values, the ratio A/T:T/S increased by > 150% in healthy human subjects and by 50% in patients with myopathies. With decreasing T/S, the A/T increased in healthy human subjects, decreased in patients with neuropathies and stayed unchanged in patients with myopathies. With this method detection rates of 60% for patients with neuropathies and 67% for patients with myopathies were found. The most reliable turn/amplitude parameters for the identification of pathological cases were the T/S and the A/T. Evaluating these two parameters, the rate of false positive results was 3.2%. The study demonstrated changes during sustained effort to be different between sexes and between healthy human subjects and patients with neuromuscular disorders. It described turn/amplitude analysis during sustained effort as a supplemental electrophysiological device for the detection of patients with neuromuscular disorders. The method is objective, fast and reliable.

Adult↗

[Lactate determination at rest and during bicycle ergometry in healthy probands and in patients with mitochondrial myopathies].

Measuring lactate during moderate exercise is a useful tool in the diagnosis of mitochondrial disorders. It was the aim of this study, to develop reference limits for lactate at rest, during exercise and after the exercise. We investigated 18 healthy subjects and 6 patients with a mitochondrial disorder. In controls, serum lactate levels were comparable to already reported findings. In 4 patients lactate levels were markedly increased during the exercise. Measurement of serum lactate is a simple and useful step in the diagnosis of mitochondrial disorders.

Electron Transport↗

[Heart muscle involvement in myopathies].

By means of a comprehensive cardiologic examination "definite" cardiac involvement was found in 71% of patients with myotonic dystrophy (MD). In 50% of patients with Becker's muscular dystrophy (BMD) and in 70% of patients with mitochondrial myopathy (MMP). "Equivocal" cardiac involvement was found in 21% of patients with MD, in 50% of patients with BMD and in 20% of patients with MMP. The correlation between cardiac involvement and the neurological deficit was weak.

Adult↗

[Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)].

Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory involvement, but without upper motor neuron signs. The cardinal feature and primary pathophysiological basis for the weakness is the multifocal motor conduction block which remains stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal stimulus response without any change in the negative peak duration. Nerves at the site of the conduction block show demyelination, endoneural edema, rudimentary onion bulbs and lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid antibodies, which may block the Na+ channels, produce demyelination or interfere with remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and cyclophosphamide.

Combined Modality Therapy↗

T/A-analysis with or without measuring force: problems in the evaluation of normal limits.

Turn/amplitude-analysis (TAA) has been applied to interference patterns of the right m. biceps brachii and the right m. rectus femoris of 33 healthy women aged 19 to 53 years. Interference patterns were recorded at 20, 40 and 60% +/- 10% of both muscles maximum force. The dependency of the turn/amplitude-parameters (T/A-parameters) on age, muscle and force is discussed. Normal limits for individual means of the T/A-parameters were calculated in form of the +/- 2 SD. Normal limits for single values were calculated in form of clouds. Both methods were applied to the values of each force level (20, 40 and 60%) and of all force levels together (10-70%). Clouds of the present study were clearly different from those of Stålberg. Evaluating individual means rates of false positive results were higher compared to those of single values. Rates of false positive results for single values and individual means of the parameters T/S and A/T were lower at the 20% force level than at the higher force levels and without measuring force. For the application of the TAA we advise every electrophysiological laboratory 1) to evaluate single values instead of individual means, 2) to use clouds instead of the +/- 2 SD as normal limits, 3) to measure force until a modified cloud-method will be applicable and 4) to elaborate its own normal limits.

Adult↗

[Satellite potentials: definition, normal values and validity in the detection of mild myogenic lesions].

From the right m. biceps brachii and the right m. rectus femoris of 28 healthy women aged 19 to 30 years and 16 possible and obligate Duchenne-carriers aged 26 to 40 years we recorded 20 motor unit action potentials each. A satellite potential was defined as electrical activity following or preceding the main component and separated from it by an isoelectrical interval of at least 1, 2 or 3 ms. The most appropriate of these 3 satellite potential definitions is the one that proceeds from a 1 ms minimum interval, because it allows the detection of more satellite potentials than the two others. Applying this definition (minimum interval between the main component and the satellite potential 1 ms) to our controls data we computed a mean interval duration of 5 (m. biceps brachii) respectively 3.4 ms (m. rectus femoris), a mean satellite potential duration of 4.4 (m. biceps brachii) respectively 3.7 ms (m. rectus femoris) and a mean satellite rate of either 3.9 (m. biceps brachii) or 1.6% (m. rectus femoris). We observed up to 3 motor unit action potentials with a satellite potential and up to 1 polyphasic motor unit action potential with a satellite potential per 20 motor unit action potentials. There was little difference between the controls and the Duchenne-carriers satellite parameter means. Only the m. rectus femoris satellite potential duration was increased in Duchenne-carriers.(ABSTRACT TRUNCATED AT 250 WORDS)

Action Potentials↗

[Potential reversal/amplitude analysis: problems of normal value determination].

Turn/amplitude-analysis was applied to the right m. biceps brachii and the right m. rectus femoris of 30 healthy women aged 19 to 39 years. Interference patterns were recorded at 20, 40 and 60% of the individual maximum force of both muscles. We evaluated the turns/s, the amplitude/turn and the ratios amplitude/turns:turns/s and turns/s:amplitude/turn. Normal values of the turn/amplitude-parameter were calculated as means +/- 2 SD at defined levels of force (table 1 and fig. 2) and in form of clouds (fig. 3). Our clouds were clearly different from those of Stalberg. Since normal values of our and other studies were inconsistent we believe that every laboratory has to elaborate its own normal values. Turns/s, amplitude/turn and the ratio turns/s:amplitude/turn increased and the ratio amplitude/turns/s decreased with increasing force (fig. 6 and 7). Turn/amplitude-parameters were partly significantly higher or lower in the m. biceps brachii than in the m. rectus femoris (fig. 8.).

Adult↗

Dysarthria as the leading symptom of hypothyroidism.

Typical symptoms of hypothyroidism are lethargy, cold intolerance, slowing of intellectual and motor activity, declining appetite, increasing weight, and dry skin. A 43-year-old man with hypothyroidism presented with dysarthria as the leading symptom. Further symptoms were cramps in the legs after exercise, dizziness, and stunned feeling. He suffered from severe snoring for 4 years, and obstructive sleep apnea syndrome was diagnosed 2 years before. Creatine phosphokinase was elevated. Electromyography was myogenic. Echocardiography showed a thickened myocardium. An otolaryngologic investigation revealed macroglossia and hypertrophy of the uvula. After administration of L-thyroxine, the symptoms rapidly improved. Dysarthria may be the leading symptom of hypothyroidism and can be promptly resolved after hormone substitution.

Adult↗

Restrictive cardiomyopathy in dermatomyositis.

The association between dermatomyositis and restrictive cardiomyopathy has not been reported before. We present here the clinical, echocardiographic and muscle biopsy data for a patient with dermatomyositis and restrictive cardiomyopathy. In a 78-year-old male with a history of arterial hypertension, recurrent episodes of atrial fibrillation and syncopes, rupture of an infra-renal aortic aneurysm with complications (recurrent QT-prolongation, lumbo-sacral plexopathy, transient ischaemic attack, peripheral embolism), monoclonal gammopathy, subdural haematoma, focal seizures, megaloblastic anaemia, leucopenia, eosinophilia, elevated muscle enzymes and increasing tiredness, dermatomyositis was diagnosed upon clinical presentation, muscle enzyme and muscle biopsy findings. Cardiological examination revealed atrial fibrillation, left anterior hemiblock and restrictive cardiomyopathy. After the exclusion of various differentials for restrictive cardiomyopathy, a causative relationship between restrictive cardiomyopathy and dermatomyositis was assumed. This case suggests the need for suspecting restrictive cardiomyopathy in patients with dermatomyositis. Patients with dermatomyositis should undergo a comprehensive cardiological investigation as soon as the neurological diagnosis is established.

Aged↗

Immunoglobulins are effective in pontine myelinolysis.

Although the exact pathogenesis of central pontine myelinolysis (CPM) is unknown, correction of hyponatremia, thyreotropin releasing hormone, plasmapheresis, and corticosteroids seem to be effective. Assuming intravenous immunoglobulins (IVIG) to also be effective in CPM, 0.4 g/kg body weight/d immunoglobulins were applied to a 48-year-old patient who developed CPM with double vision, dysarthria, dysphagia, and left-sided hemiparesis 3 weeks after spontaneous normalization of hyponatremia. After 5 days of IVIG, his symptoms markedly improved, confirmed by improvement in the Norris score (42%), Frenchay score (19%), Kurtzke score (20%), Disability score (54%), vital capacity (26%), and peak torque (69%). The promising clinical effect of IVIG was assumed to be caused by the reduction of myelinotoxic substances, the development of antimyelin antibodies, and the promotion of remyelination. In conclusion, IVIG appear to be a promising therapeutic option in CPM.

Adult↗

Pleural and bone marrow metastasis from supratentorial oligoastrocytoma grade III.

We report a 62-year-old man with symptomatic metastasis to the pleural cavity and the bone marrow from a supratentorial oligoastrocytoma grade III, 24 years after the initial symptoms. Before tumor dissemination, the patient underwent brain surgery four times in 5 years. Six months after the last treatment, extraneural dissemination to the right pleural cavity was discovered. Despite resection of the pleural metastasis, local and distant spread to the bone marrow developed. The patient died 5 months after the occurrence of extraneural tumor metastasis. It is speculated that repeated brain surgery and extended survival may promote extraneural dissemination of supratentorial oligoastrocytoma grade III.

Astrocytoma↗

Atypical paraneoplastic syndrome associated with anti-Yo antibodies.

OBJECTIVE: Polyneuropathy, myopathy and spasticity have not been described as a manifestation of a neurologic paraneoplastic syndrome (NPS) associated with anti-Yo antibodies (anti-Yo). CASE HISTORY: The patient is a 60-year-old woman with a history of ovarectomy, salpingectomy, hysterectomy and omentectomy because of ovarian cancer with peritoneal carcinosis. From May to September 1999, she received chemotherapy with carboplatin and docetaxel. In June 1999, weaknesses of the lower limbs began to appear. Neurologic investigation revealed bilateral ptosis with right-sided predominance, exaggerated deep tendon reflexes, discrete distal weakness, wasting of the upper limbs and diffuse weakness of the lower limbs. She had slight CK elevation, elevated lactate dehydrogenase and aldolase levels. Testing for anti-neuronal antibodies revealed high serum titers of antibodies against the cytoplasm of Purkinje cells, confirmed as anti-Yo by immunoblot with recombinant proteins. CSF investigations showed 12/3 cells and positive oligoclonal bands. MRI of the brain showed bilateral, old ischemic basal ganglia lesions exclusively. Visually evoked potentials gave prolonged P100 latencies bilaterally. Nerve conduction studies and electromyography revealed motor polyneuropathy of the lower limbs. Muscle biopsy from the right anterior tibial muscle showed non-specific myopathic features. CONCLUSION: Polyneuropathy, myopathy and tetraspasticity may be the exclusive manifestations of an atypical NPS associated with anti-Yo. Anti-Yo may persist for years without relapse of the primary tumor.

Antibodies↗

Immunoglobulins in acute, parainfectious, disseminated encephalo-myelitis.

Acute, parainfectious, disseminated encephalo-myelitis (ADEM) is usually treated with corticosteroids. Intravenous immunoglobulins have not been applied to patients with ADEM. This article describes a 22-year old woman who developed progressive paraparesis, ascending sensory dysfunction, and urinary incontinence one week after tonsillitis. Guillain-Barré syndrome was diagnosed initially, and intravenous immunoglobulins (0.4 g/kg daily) were begun. Symptoms deteriorated after the first and second applications, but after the third application the patient's sensory level declined. After the fourth application, the deterioration of symptoms stopped and cerebrospinal fluid (CSF) abnormalities improved. Because of diffuse spinal cord swelling, intramedullary edema, and hyperintense white and grey matter lesions in the basal ganglia and the corpus callosum on MRI scans, the diagnosis was corrected to ADEM. It is concluded that initial administration of intravenous immunoglobulins might be of therapeutic value in patients with ADEM.

Adult↗

Cerebrospinal fluid filtration and immunoglobulins in multifocal motor neuropathy.

Cerebrospinal fluid (CSF) filtration has been shown to be of benefit in chronic inflammatory, demyelinating polyneuropathy, but has not been applied to multifocal motor neuropathy (MMN) so far. Twenty-seven months after a 48-year-old male patient had developed slowly progressive, distally prominent monoparesis of the left arm, MMN was diagnosed. Conduction blocks were found in the left brachial plexus after median, ulnar, and radial nerve stimulation. Serum anti-GM1 antibody titers were markedly increased. Biopsy of the motor long thoracic nerve showed reduction of small caliber myelinated axons and irregularly shaped myelin lamellae. Treatment with immunoglobulins 29, 31, 36, and 39 months after onset was followed by a distinct improvement each time. Thirty-four months after onset, one liter CSF was filtered off by means of a bidirectional syringe pump with only minor therapeutic effect. In conclusion, immunoglobulins had a stronger therapeutic effect than CSF filtration on the MMN patient described.

Case-Control Studies↗