A pure amnestic syndrome after MDMA ("ecstasy") ingestion.
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Biomedical subjects
Publications and source records attributed to B Mamoli.
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OBJECTIVES: To find out if conventional and automatic needle EMG of the tongue can be helpful in the diagnosis and differentiation of limb and bulbar onset amyotrophic lateral sclerosis. METHODS: Motor unit action potential (MUAP) analysis and peak ratio interference pattern analysis were performed in the right genioglossus muscle of 30 healthy subjects aged 30-81 years, 10 patients aged 49-73 years with limb onset amyotrophic lateral sclerosis, and eight patients aged 52-75 years with bulbar onset amyotrophic lateral sclerosis. Electrical activity was sampled via standard concentric needle electrodes with a commercially available EMG recorder. RESULTS: Normal mean (2SD) MUAP duration was 6.6 (1.5) ms. Normal mean (2SD) MUAP amplitude was 224 (97.4) microV. Normal mean (2SD) peak ratio (PR), turns/second (T/s), amplitude/turn (A/T), and time intervals (TI1, TI2, TI3) were 1.68 (0.56), 732 (303.9), 446 (180.3) microV, 2.62 (0.34), 2.31 (0.14), and 1.01 (0.50) respectively. Mean MUAP duration and amplitude were significantly increased in limb onset (P=0.0001 and P=0.013) and bulbar onset amyotrophic lateral sclerosis (P=0.0001 and P=0.017). Peak ratio indices stayed unchanged in limb onset amyotrophic lateral sclerosis but were significantly decreased (PR, T/s, A/T, TI1, and TI2) or increased (TI3) in bulbar onset disease. The sensitivity of the MUAP analysis was 70% in limb and 75% in bulbar onset amyotrophic lateral sclerosis. The sensitivity of the peak ratio interference pattern analysis was 20% in limb and 100% in bulbar onset amyotrophic lateral sclerosis. Subclinical involvement of the tongue was found in 20% of the patients with limb onset amyotrophic lateral sclerosis and could be more accurately assessed with MUAP analysis than with automatic EMG. CONCLUSIONS: Both conventional and automatic needle EMG of the tongue are valuable electrophysiological devices to assess the clinical and subclinical involvement of the tongue in patients with limb and bulbar onset amyotrophic lateral sclerosis.
Recently, the Austrian Immunoglobulin in Multiple Sclerosis (AIMS) study showed patients with relapsing-remitting multiple sclerosis to benefit from repeated administration of intravenous immunoglobulin (IVIg). To provide a more detailed understanding of IVIg's action we performed further analyses on the time course of treatment effects and in regard to the impact of clinical disability at study entry on patients' response to medication. The AIMS trial was a randomized, placebo-controlled, double blind, multicenter trial. It included 148 patients (IVIg: 75; placebo 73) who suffered from relapsing-remitting MS, were 15-65 years old and scored from 1-6 on the Expanded Disability Status Score (EDSS). IVIg was given over 2 years in a monthly dosage of 0.15-0.2 g/kg body weight Within the first 6 months of the trial clinical disability of IVIg treated patients improved significantly from a baseline EDSS of 3.33 +/- 1.38 to a score of 3.05 +/- 1.73 (P=0.002). This improvement was retained over the subsequent 18 months of the trial (final EDSS: 3.09 +/- 1.62). In contrast, placebo-treated patients showed a slight trend for deterioration over the study period (baseline EDSS: 3.37 +/- 1.67; final EDSS: 3.49 +/- 1.83). IVIg treatment was associated with a significant reduction of relapses throughout the study which was independent of the patients' disability at baseline. The observation of clinical improvement in the early phase of IVIg medication may suggest the activation of repair mechanisms such as the promotion of remyelination while immunoregulatory effects would be expected as the cause of fewer exacerbations throughout the AIMS study. These hypotheses need to be tested in future trials.
OBJECTIVES: to assess the influence of the skin and muscle temperatures on the turns per second (T/s) and the amplitude per turn (A/T) and to find out whether turn/amplitude analysis requires temperature monitoring. SUBJECTS: ten healthy individuals (three women, seven men) aged 23 to 36 years. METHOD: recording of two electromyograms (EMGs) in the right biceps brachii muscle from ten locations each, during isometric contractions with 30% of the elbow flexors' maximum force. The first EMG was recorded after warming the muscle to at least 37 degrees C and the second EMG after cooling the muscle to at least 27 degrees C. RESULTS: lowering the muscle temperature by an average of 10.9 degrees C, the mean T/s decreased from 484 to 436 (P = 0.002), the mean A/T increased from 476 to 513 (P = NS) and the mean ratio T/s:A/T decreased from 1.1 to 0.91 (P =0. 0001). None of the individual results became patholgical after cooling the muscle. CONCLUSION: in a range of 27 to 37 degrees C, turn/amplitude analysis can be performed without temperature monitoring.
This study was undertaken to determine whether anti-GM1 titres are raised in polyneuropathies of unknown origin and whether determination of these titres is useful for diagnosing these conditions. The study population comprised 20 controls (aged 36-88 years), 12 patients with polyneuropathies of known origin (aged 31-81 years) and 15 patients with polyneuropathies of unknown origin (aged 40-77 years). Antibody levels were measured using a commercial GM1 enzyme linked immunosorbent assay kit (Buehlmann Laboratories). Mean anti-GM1 IgG and IgM antibody titres were not raised in patients with polyneuropathies of unknown origin. Anti-GM1 IgG antibody titres were raised in one and GM1 IgM antibody titres in none of the patients with polyneuropathies of unknown origin. In conclusion, GM1 antibody levels are rarely raised in polyneuropathies of unknown origin and probably play a minor role in the pathogenesis of these conditions.
We report a 74-year-old woman with progressive cognitial deterioration and changes in personality. She had no clinical signs of an inflammatory CNS process, but brain CT and MRI scans and cytologic examination of the CSF were initially indicative of encephalitis and ventriculitis. Antiviral and antibacterial therapy had no effect on the course of symptoms, and patient became comatose. We established the diagnosis of a primary CNS mantle cell lymphoma (PCNSL) and began corticosteroids. Within a few days the patient became alert and was able to walk again. Nonenhancing and non-space-occupying PCNSLs are rare but must be considered in the differential diagnosis of coma and encephalitis. Comatose PCNSL patients without radiographic evidence for herniation can be successfully treated with corticosteroids even if the EEG has a burst suppression pattern.
We report a 67-year-old man who developed yes/yes head tremor without appendicular tremor six weeks after right occipital and bilateral cerebellar infarction. The tremor was resting-postural. Its activity increased with excitement, decreased either after ethanol, lateroflexion or rest and stopped at sleep. Four-vessel angiography revealed a well collateralised occlusion of both vertebral arteries. Needle-EMG recordings showed rhythmic, synchronous agonist/antagonist activity in both the splenius capitis and sternocleidomastoideus muscles at a frequency of 2-3 Hz. After administration of botulinum toxin A (DysportR), the tremor markedly improved and vanished completely after a booster. Since then the tremor did not reappear. Delayed-onset, yes/yes head tremor without appendicular tremor may be caused by bilateral cerebellar infarction and can be successfully relieved with botulinum toxin A.
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.
Whereas it would be preferable to inform patients with amyotrophic lateral sclerosis (ALS) as early as possible about the possibilities of ventilatory support, not all of them are able to bear the required thorough information on the consequences of the diagnosis. The procedure therefore has to be individualized. Questionnaire studies have revealed, that in general patients hold the opinion that they themselves should decide, whether or not artificial ventilation should be applied. The non-invasive artificial home ventilation has led to advances in the management of patients with ALS, as a longer period of survival with a higher level of quality of life can be achieved. On the other hand, according to the author's opinion. Invasive artificial ventilation can not be the primary goal of medical support in the context of the prognosis (progressive differentiation). Much more this should focus on the appropriate steps to warrant increase of period of survival under conditions of at least minimal quality of life. Other opinions however have to be respected. Appropriate procedures for prevention and therapy of respiratory dysfunctions are discussed.
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.
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.
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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.
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Basilar artery migraine is a distinctive disorder characterized by recurrent occipital headache with typical migrainous phenomena in conjunction with a variety of symptoms referable to a transient dysfunction of the brainstem, the cerebellum and the occipital lobes. Impairment of consciousness is a common feature but is usually too brief to be captured by electroencephalography. Here we report an unusual case of basilar artery migraine with prolonged attacks of impaired consciousness lasting more than two weeks, thus allowing electroencephalographic follow-up. In the acute stage, EEG showed marked generalized slow wave delta activity. After the attack, both the electroencephalographic and clinical findings subsided within the following weeks.