Misjudged Bannwarth's syndrome culminating in laminectomy.
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Borrelia burgdorferi Group↗
Biomedical subjects
J Finsterer
Publications and source records attributed to J Finsterer.
Influence of spontaneous activity on peak-ratio analysis.
OBJECTIVES: To prove or disprove this assumption that in neuropathy patients with abundant spontaneous activity, peak-ratio interference pattern analysis may lead to false negative results. METHODS: Spontaneous activity >100 microV, automatically analysed by turn/amplitude analysis and expressed as (turns/second)/2 ((T/S)/2), and interference patterns, analysed by the peak-ratio technique, were recorded, one after the other, from the right anterior tibial muscle of 21 patients with neuropathy, aged 36-87 years. RESULTS: The mean number of spontaneous discharges ((T/S)/2) was 12.3 (range 5.5-26) and its mean amplitude 261 microV (range 146-478 microV). Despite this abundant spontaneous activity, peak-ratio analysis was neurogenic in 81% of the patients. All peak-ratio parameters were independent on the amount and amplitude of spontaneous discharges. CONCLUSIONS: Spontaneous discharges >100 microV could be adequately assessed by means of the turn/amplitude analysis and did not influence peak-ratio analysis in neuropathies.
Adult↗
Genotype-phenotype correlation in myotonic dystrophy.
Myotonic dystrophy (DM) is caused by a mutation in the length of a trinucleotide (CTG) repeat in the 3' untranslated region of the myotonin protein kinase gene located on chromosome 19q13.3. The normal gene has between 5 and 36 CTG trinucleotide repeats, whereas minimally affected individuals have 50 copies and severely affected DM-patients have several thousands of such repeats. Since no information on a genotype phenotype correlation in Austrian DM-patients is available, we examined a small group of these patients for the unstable trinucleotide repeat. Molecular analysis was used to clarify equivocal clinical diagnoses and confirm clinical findings. We studied eight DM-families, a total of 57 individuals, of whom 18 were diagnosed with a trinucleotide repeat expansion. Twenty-six unrelated individuals served as a control. Clinical assessment was based on the muscular disability rating scale (MDRS) and a sum of symptoms score (SSS). There was a significant correlation between the clinical scores (MDRS: Spearman r = 0.51; p = 0.029: SSS: Spearman r = 0.538; p = 0.0259) used and the size of the amplification of the trinucleotide repeat. The largest expansion found in our group of patients was 6 kb. Furthermore, we observed both expansion and contraction of the enlarged fragment during transmission from one generation to the next.
Adolescent↗
Headache as the initial manifestation of acute aortic dissection type A.
The most common initial symptom of aortic dissection is chest pain. Other initial symptoms include pain in the neck, throat, abdomen and lower back, syncope, paresis, and dyspnoea. Headache as the initial symptom of aortic dissection has not been described previously. A 61-year-old woman with a history of migraine and arterial hypertension developed continuous bifrontal headache. Two hours later, right-sided thoracic pain and a diastolic murmur were suggestive of aortic dissection that was confirmed by echocardiography and subsequent surgery. The dissection commenced in the ascending aorta and involved all cervical arteries until the base of the skull. Headache as the initial manifestation of aortic dissection was assumed due to either vessel distension or pericarotid plexus ischemia. Aortic dissection has to be considered as a rare differential diagnosis of frontal headache, especially in patients who develop aortic regurgitation or chest pain for the first time.
Aneurysm↗
Hypertrabeculated left ventricle in mitochondriopathy.
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Aged↗
Progression of cardiac involvement in patients with myotonic dystrophy, Becker's muscular dystrophy and mitochondrial myopathy during a 2-year follow-up.
The aim of this prospective noninvasive follow-up study was (1) to assess the progression of cardiac involvement (CI) in patients with myotonic dystrophy (MD), Becker's muscular dystrophy (BMD) and mitochondrial myopathy (MMP), (2) to find out if CI and neurologic impairment are related and (3) to determine how often cardiac investigations should be performed. Clinical, electrocardiographic, echocardiographic and 24-hour ambulatory electrocardiographic examinations were performed at yearly intervals. CI was assessed qualitatively by the presence of pathologic examinations and quantitatively by the number of pathologic examinations. Qualitatively, CI was present at baseline in 36 of the 38 cases (18-68 years) and the number of cases did not change. Quantitatively, CI progressed after 1 (2) year in 50% (50%) of patients with MD (n = 16), 0% (43%) of patients with BMD (n = 7) and 27% (27%) of patients with MMP (n = 15). CI and neurologic impairment were not related (p = 0.54). Cardiac investigations should be repeated at yearly intervals irrespective of whether there is neurologic deterioration or not.
Adolescent↗
Facial myocloni and stroke as late sequelae of metrizamide myelography.
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Aged↗
Needle electromyography of bulbar muscles in patients with amyotrophic lateral sclerosis: evidence of subclinical involvement.
OBJECTIVE: To determine if quantitative motor unit action potential (MUAP) analysis and peak ratio interference pattern analysis of the fifth, seventh, and eleventh cranial nerve innervated muscles are helpful in the assessment of subclinical bulbar involvement in ALS. METHODS: With both electromyographic (EMG) techniques, electrical activity was recorded via needle electrodes from the right frontalis, masseter, and sternocleidomastoideus muscles of nine ALS patients without clinical bulbar signs (Frenchay score >85%) aged 40 to 87 years; 21 healthy subjects aged 27 to 74 years; and five ALS patients with clinical bulbar signs (Frenchay score <85%) aged 53 to 69 years. RESULTS: The normal mean (2 SD) MUAP duration of the frontalis muscle was 7.4 (2.2) milliseconds; masseter muscle, 9.3 (3.1) milliseconds; and sternocleidomastoideus muscle, 10.9 (4.1) milliseconds. The normal mean (2 SD) peak ratio of the frontalis was 2.3 (1.1) milliseconds; masseter, 1.2 (0.4) milliseconds; and sternocleidomastoideus, 1.5 (0.7) milliseconds. Quantitative MUAP analysis was interpreted as neuropathic if MUAP duration, MUAP amplitude, or both exceeded the mean (+2 SD). Peak ratio interference pattern analysis was interpreted as neuropathic if the peak ratio, the number of small time intervals, or both were below the mean (-2 SD). If a result of either EMG technique was neuropathic in at least one of the three investigated muscles, bulbar involvement was assumed. Subclinical bulbar involvement could be detected by quantitative MUAP analysis in six ALS patients without clinical bulbar signs, and by peak ratio interference pattern analysis in two. CONCLUSIONS: Conventional needle EMG of the fifth, seventh, and eleventh cranial nerve innervated muscles shows subclinical bulbar involvement quite frequently. Peak ratio interference pattern analysis is largely not helpful in detecting this involvement.
Accessory Nerve↗
Malnutrition-induced hypokalemic myopathy in chronic alcoholism.
CASE REPORT: A 42-year-old man with a history of Billroth II-gastrectomy, chronic alcoholism, and malnutrition developed acute tetraparesis, two days before admission. He presented with bilateral, proximal upper and lower limb weakness, limb girdle wasting, bilaterally reduced Achilles tendon reflexes, and bilateral stocking-type sensory disturbances. Laboratory data revealed hypokalemia (2.2 mmol/L), elevated creatine kinase (7282 U/L), metabolic alkalosis and reduced urine potassium, albumin, and total protein. Muscle biopsy showed atrophic, necrotic, and regenerating fibers, endomysial macrophages, and vacuolar degeneration, interpreted as hypokalemic myopathy. With the correction of the serum potassium, tetraparesis rapidly resolved. With other causes excluded, malnutrition and gastrectomy were considered responsible for hypokalemia in this patient with acute tetraparesis and chronic alcoholism.
Acute Disease↗
Dysthyroid ophthalmopathy misinterpreted as ocular myositis.
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Diagnostic Errors↗
[Electromyography in myopathies].
Despite increasing importance of molecular genetics, electromyography has preserved its place as a valuable tool in the diagnostic procedure of myopathies. Conventional electromyography allows the assessment of spontaneous activity, motor unit action potentials and interference patterns. In myopathies, fibrillations and positive sharp waves can be found in the majority of the cases. Motor unit action potentials are of short duration, low amplitude and may show increased polyphasia and number of satellite potentials. The interference pattern may be of low amplitude and compact already at submaximal contraction. Compared to conventional electromyography, automatic interference pattern analysis provides quantitative results and has the higher sensitivity and specificity. Normal conventional or automatic electromyography does not exclude a myopathy. For diagnostic purposes, electromyography will be followed by muscle biopsy and DNA analysis in most of the cases.
Diagnosis, Differential↗
Light chain myeloma with oro-pharyngeal amyloidosis presenting as bulbar paralysis.
A 53-year old woman developed slowly progressive dysarthria, mild enlargement of the tongue and dysphagia since 1 year ago. All neurological differential diagnoses that could have explained the bulbar symptoms were excluded. The swallowing sequence was pathologic and immunoglobulins were markedly reduced. A bone marrow biopsy revealed light chain myeloma grade III. Amyloid deposits were found in the tongue but not in the kidneys. Oro-pharyngeal amyloidosis was held responsible for the described complaints. It is concluded that multiple myeloma must be considered in the differential diagnosis of bulbar paralysis and that biopsy of specific lesions is necessary to confirm local amyloidosis.
Amyloidosis↗
Satellite potentials as a measure of neuromuscular disorders.
The study was carried out to investigate the characteristics of satellite potentials and their validity in clinical electromyography. Conventional needle electromyography was applied to the right biceps brachii and tibialis anterior muscles of 41 controls, 22 neuropathies, and 17 myopathies. Satellites were defined as small extrapotentials, preceding/following the main motor unit action potential (MUAP) component and separated from it by an isoelectrical interval of > 1 ms. The normal mean satellite rate was 1.6% (biceps brachii) and 1.2% (tibialis anterior). In the biceps brachii (tibialis anterior) muscle it was 5 (5) times higher for neuropathies (P = 0.005, P = 0.006) and 5 (6) times higher for myopathies (P = 0.006, P = 0.003). MUAP parameters were not significantly different, whether satellites were considered or ignored. Evaluation of the satellite rate increased detection rates of neuromuscular disorders by up to 13%. The satellite rate proved a valuable and easily available, supplemental electromyographic parameter for the discrimination and detection of neuromuscular disorders.
Adult↗
ECG abnormalities in myopathies, coronary heart disease and controls.
The aim of the study was to compare the prevalence of predefined ECG abnormalities, compiled from the literature, and of increased electrocardiographic myopathy indices (QT/PQs, P/PQs, R/S) among myopathy patients, patients with coronary heart disease and healthy subjects. ECGs from 27 myopathy patients, 35 patients with coronary heart disease and 36 healthy subjects were investigated. ECG abnormalities most often observed in myopathy patients were ST-abnormalities, T-wave abnormalities and tall R and/or S-waves. At least one increased electrocardiographic myopathy index was observed in 19% of the myopathy patients, 20% of the patients with coronary heart disease and 19% of the healthy subjects. At least one predefined ECG abnormality was found in 78% of the myopathy patients, 86% of the patients with coronary heart disease and 33% of the healthy subjects. In conclusion, ECG abnormalities frequently occur in myopathy patients and nearly as often as in patients with coronary heart disease. Electrocardiographic myopathy indices lack specificity and are thus of minor help in assessing myocardial alterations in myopathy patients.
Adolescent↗
Cardiac involvement in patients with myotonic dystrophy, Becker's muscular dystrophy and mitochondrial myopathy.
The aim of this prospective study was to classify cardiac involvement in myopathies by means of a comprehensive cardiac investigation, to determine the rate of cardiac involvement in myopathies according to this classification and to compare the validity of previously reported electro-cardiographic myopathy indices (QT/PQs, P/PQs, R/S) with that of the comprehensive cardiac investigation. We included 14 patients with myotonic dystrophy, 6 patients with Becker's muscular dystrophy and 10 patients with mitochondrial myopathy. Cardiac involvement was classified as either "definite", "equivocal" or "absent" by assessing cardiovascular history, physical examination, electrocardiography, echocardiography and 24-hour ambulatory electrocardiography. "Definite"/"equivocal"/"absent" cardiac involvement was found in 12/2/0 myotonic dystrophy, 3/3/0 Becker's muscular dystrophy and 6/3/1 mitochondrial myopathy patients. Electrocardiographic myopathy indices were pathologic in 3 Becker's muscular dystrophy, 6 mitochondrial myopathy but in none of the myotonic dystrophy patients. The proposed comprehensive cardiac investigation is simple, inexpensive and effective in assessing cardiac involvement in patients with myotonic dystrophy. Becker's muscular dystrophy and mitochondrial myopathy. In case of cardiac involvement, cardiac therapy might be one of the few therapeutic options for these patients.
Adolescent↗
Peak-ratio interference pattern analysis in the detection of neuromuscular disorders.
Peak-ratio interference pattern analysis (peak-ratio method) is said to have a high sensitivity and to be independent of sex and age. This study was carried out to prove or disprove these findings. The peak-ratio method and qualitative motor unit action potential (MUAP) analysis were applied to the right brachial biceps and anterior tibial muscles of 44 healthy subjects, aged 23-87 years, 25 neuropathy patients, aged 21-83 years, and 29 myopathy patients, aged 19-70 years. Peak-ratio parameters were independent of sex and age. They tended to be lower in the anterior tibial muscle than in the brachial biceps muscle. Neuropathy patients typically showed decreased peak-ratio, short time intervals and increased amplitude/turn. Myopathy patients typically showed increased peak-ratio, turns/s and short time intervals. The sensitivity of the peak-ratio method was 72% for neuropathy patients and 59% for myopathy patients. The sensitivity of the peak-ratio method was similar to that of the MUAP analysis in neuropathy patients and higher than that of the MUAP analysis in myopathy patients. The specificity of the peak-ratio method was 80%. The peak-ratio method proved to be a valuable, supplementary electromyographic tool for the detection of neuromuscular disorders.
Action Potentials↗
Quantitative electromyography-guided botulinum toxin treatment of cervical dystonia.
The purpose of this study was to investigate the clinical and electromyographic effect of turn/amplitude analysis (TAA)-guided botulinum toxin administration in patients with cervical dystonia. Involuntary electromyographic activity was recorded from both sternocleidomastoidei, both splenii capites, and both trapezii muscles of 13 torticollis patients, aged 34-73 years, before and after botulinum toxin A (Dysport) application. Dystonic muscles were selected for the injection if mean turns/s exceeded a level of 200. Four weeks after treatment with a mean dose of 223 mu/subject, clinical improvement was observed in 12 patients (92%) and only one patient reported no effect. Electromyographic improvement could be observed in 10 patients (77%). Both turns/s and the amplitude/turn decreased by 27% on the average after treatment. The electromyographic toxin effect showed a good correlation with the clinical toxin effect (r = 0.6). No dose dependency of the changes in turn/amplitude parameters could be observed. We found TAA a valuable modality for targeting and selecting dystonic muscles and for assessing the therapeutic benefit of the toxin.
Adult↗