Acta Neurologica Scandinavica

Andersen, H.

doi: 10.1111/j.1600-0404.1999.tb00383.xpmid: 10510679

In contrast to sensory and autonomic disturbances motor function has seldomly been studied in diabetic neuropathy. Recently quantitative studies of long‐term type 1 diabetic patients have shown that the strength of the ankle and knee extensors and flexors are moderately impaired. The weakness is closely related to the severity of neuropathy. Applying quantitative electromyography the degree of reinnervation is related to the muscle strength in diabetic patients suggesting the reinnervation to be insufficient. Magnetic resonance imaging of the distal part of the leg has revealed substantial muscular atrophy closely related to the degree of muscle weakness. The present findings indicate that diabetic neuropathy often is a mixed sensory‐motor neuropathy.

Räty, L.; Hamrin, E.; Söderfeldt, B.

doi: 10.1111/j.1600-0404.1999.tb00384.xpmid: 10510680

Objectives ‐ This study aimed to illuminate adult's experienced quality of life in newly‐debuted epilepsy and to test the American instrument Quality of Life Index (QLI) for the first time on an epilepsy population. A second aim was to find appropriate questions to measure patient perceptions in epilepsy. Material and methods ‐ All persons 18–65 fulfilling criteria (n=41) and diagnosed during a 15‐month period at 2 Swedish hospitals, answered questionnaires (n= 37/41) on quality of life and perceptions of epilepsy. Results ‐ Patients experienced the highest quality in the “Family” domain and the lowest in the “Psychological/ spiritual”. Significant correlations were found between quality of life and experienced change of life situation, own perceptions of epilepsy, seizure frequency after diagnosis, gender and side effects from antiepileptic drugs. The QLI was well applicable on people with epilepsy. Conclusions ‐ Data indicates that debut of epilepsy has an evident impact on quality of life and a more extensive study is required.

Müller, T.; Eising, E.; Kuhn, W.; Büttner, T.; Coenen, H.‐H.; Przuntek, H.

doi: 10.1111/j.1600-0404.1999.tb00385.xpmid: 10510681

Objective ‐ Studies on reaction time have suggested a selective deficit of slowness in motor readiness and motor programming in Parkinson's disease (PD). Objective of this study was the putative relation between delayed initiation and execution of movement and the striatal dopamine deficiency in PD. Material and methods ‐ We investigated 32 idiopathic, previously untreated parkinsonian patients to evaluate dopaminergic nigrostriatal degeneration by single photon emission tomography in combination with the radiotracer [123I]‐β‐CIT and performed a simple reaction time paradigm on the same day. Results ‐ Significant relations between the [123I]‐β‐CIT‐SPECT‐ratio striatum/cerebellum and reaction ‐ and movement time appeared. Reaction time and movement time of parkinsonian patients were significantly longer compared to age‐matched controls. Conclusions ‐ We conclude that reaction‐ and movement‐time is related to the dopaminergic nigrostriatal degeneration in untreated parkinsonian subjects.

Dizdar, N.; Granérus, A.‐K.; Hannestad, U.; Kullman, A.; Ljungdahl, Å.; Olsson, J.‐E.; Kågedal, B.

doi: 10.1111/j.1600-0404.1999.tb00386.xpmid: 10510682

Objectives ‐ The pharmacokinetics of freel‐dopa in blood and tissue of five parkinsonian patients with malignant melanoma was studied with microdialysis. In one case the effect ofl‐dopa treatment on 5‐S‐cysteinyldopa and the melanoma was studied. Gastric emptying and its effects on free l‐dopa in blood were also investigated in one of the patients. Methods ‐ Five patients were given 100 mg l‐dopa with 25 mg benserazide. Blood and dialysates from the circulation and fatty tissue were collected for analysis. [13C]‐Octanoic breath test was used for analyzing gastric half‐emptying time. Results ‐ Four of the patients had similar pharmacokinetic patterns for l‐dopa and a significant (P < 0.05) increase of serum 5‐S‐cysteinyldopa occurring 30 min after l‐dopa intake. Delayed l‐dopa peaks and slow gastric half‐emptying time were found in 1 patient. A dose‐dependent increase of 5‐S‐cysteinyldopa occurred but no melanoma metastases were seen during long‐term l‐dopa therapy. Conclusion ‐ l‐dopa therapy increases 5‐S‐cysteinyldopa levels but does not seem to cause progress of melanomas. Gastric emptying impacts l‐dopa pharmacokinetics.

Niebroj‐Dobosz, I.; Jamrozik, Z.; Janik, P.; Hausmanowa‐Petrusewicz, I.; Kwiecinski, H.

doi: 10.1111/j.1600-0404.1999.tb00387.xpmid: 10694928

Objectives ‐ An autoimmune basis has been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). This hypothesis is supported by the presence of antibodies that interact with motoneuron antigens in serum of these patients. Against autoimmunity are the discrepances in the frequency of the antibodies appearance and also failure of immunosuppression. The aim of our study was to evaluate the titer of antibodies against GM1‐gangliosides, AGM1‐gangliosides and anti‐sulfatides in paired serum and cerebrospinal fluid samples in the ALS patients. Material and methods ‐ Serum of 103 and CSF of 79 patients with ALS was examined. The “disease controls” consisted of 22 cases of other motor neuron diseases and 50 healthy, age‐matched normals. CSF was drawn at the same time from 79 ALS patients, 6 cases of the “disease controls” and 50 normals. To study the titer of antibodies against GM1‐gangliosides, AGM1‐gangliosides and sulfatides the ELISA technique has been applied. Results ‐ An increased titer against GM1‐gangliosides, AGM1‐gangliosides and sulfatides in ALS appeared in serum in 18%, 32% and 11%, resp., in the “disease controls” the increased antibodies titer appeared in single cases. In CSF the appropriate values in ALS were 20%, 15%, 8%, resp. In the “disease controls” a high antibodies titer was a rare finding. Conclusions ‐ It is concluded that in some ALS cases and also in some patients with other motor neuron diseases an autoimmune mechanism may contribute to motor neuron injury.

Shindo, K.; Tsunoda, S.; Shiozawa, Z.

doi: 10.1111/j.1600-0404.1999.tb00388.xpmid: 10510684

To evaluate autonomic function in sporadic cases of cerebellar degeneration (CD) with or without mild autonomic dysfunction, we measured muscle sympathetic nerve activity (MSNA) by microneurography in 12 CD patients and 18 healthy subjects. The burst incidence and mean amplitude of MSNA at rest was significantly decreased in CD patients (P < 0.01). There were no significant differences between patients with CD and controls in heart rate or blood pressure at rest. During head‐up tilting, increases in blood pressure and mean amplitude of MSNA were significantly lower in CD patients. In conclusion, a decrease in muscle sympathetic outflow can be observed even in CD patients who are clinically excluded from multiple system atrophy (MSA). It might be difficult to distinguish other CD syndromes from MSA in recordings of muscle sympathetic activity.

Chen, R.‐S.; Huang, C.‐C.; Chu, N.‐S.; Cheng, C.C.; Wei, Y.‐H.

doi: 10.1111/j.1600-0404.1999.tb00389.xpmid: 10510685

Objectives ‐ We report the unusual phenotypic expression in 2 female carriers of a family with X‐linked recessive bulbospinal neuronopathy (X‐BSN). We analyze the methylation pattern of the androgen receptor (AR) gene to inspect the possibility of non‐random X chromosome inactivation to be the underlying mechanism. Material and methods ‐ Twenty‐three members in 3 generations of a Taiwanese family were examined and studied for genomic DNA analysis. We analyzed the sequence of the first exon of the AR gene to identify the numbers of CAG repeats, and to determine the methylation pattern by using the restriction enzymes Hpa II and Hha I. Results ‐ There were 3 probands and 5 carriers and 2 of the carriers manifested clinical symptoms. Sequence analysis revealed that the numbers of trinucleotide repeats ranged from 42 to 45 in one allele of the X‐chromosome in the 5 female carriers. The restriction pattern of the Hpa II and Hha I recognizable sites of the X‐chromosome indicated a random methylation. Conclusion ‐ Our data suggest that molecular genetic studies are important in confirming the diagnosis of X‐BSN and early detection of female carriers, and the random or non‐random methylation pattern of the X‐chromosome is not a determining factor for partial expression of the abnormal AR gene in some carriers.

Kallela, M.; Wessman, M.; Färkkilä, M.; Palotie, A.; Koskenvuo, M.; Honkasalo, M.‐L.; Kaprio, J.

doi: 10.1111/j.1600-0404.1999.tb00390.xpmid: 10510686

Objectives ‐ The aim of the study was to search for clinical differences between migraine with and without aura. Materials and methods ‐ From a population‐based Finnish Twin Cohort we studied 51 migraine concordant monozygotic twin pairs. Results ‐ There were 20 pairs concordant for migraine with aura, 6 pairs concordant for migraine without aura and 12 “mixed” pairs. In the remaining 13 pairs the aura of at least 1 twin could not be classified. All 20 migraine with aura pairs were concordant for visual aura and 19 for moderate or severe headache while all 6 pairs with migraine without aura were concordant for headache duration of 4 to 24 h, moderate or severe headache and nausea. The 12 “mixed” pairs had more often unilateral and pulsating headache compared to both the migraine with or without aura pairs. Overall individual migraine with aura twins had more photophobia (P= 0.032) and the migraine without aura twins more nausea (P= 0.025). Conclusions ‐ The difference between migraine with and without aura is not explained entirely by genetical factors: 12 genetically identical twin pairs were discordant for the aura. The headache phase in migraine with and without aura is very similar, but not identical. Probably there are several and different liability loci for the migraine aura and the migraine headache. The distribution of these several loci along with acquired factors will decide whether the phenotype is migraine with or without aura.

Schwenkreis, P.; Vorgerd, M.; Malin, J.‐P.; Tegenthoff, M.

doi: 10.1111/j.1600-0404.1999.tb00391.xpmid: 10510687

The aim of our present study was to detect whether a generalized disturbance of intracortical inhibitory mechanisms as assessed by transcranial magnetic stimulation (TMS) can be observed in a movement disorder with localized clinical expression, that is, in focal cervical dystonia. We measured motor threshold intensity, central motor conduction time and the duration of postexcitatory inhibition evoked by single and paired stimuli TMS from a small hand muscle in 20 patients with idiopathic cervical dystonia, and 21 healthy volunteers. A significant difference could not be found in any of the neurophysiological parameters between patients and controls. These findings are unlike the observations made in Parkinson's disease and Huntington's disease, where significant changes of postexcitatory inhibition after TMS can be observed. This suggests a lack of widespread change in activity of underlying cortical inhibitory mechanisms, as seen in other diseases of the extrapyramidal system with more generalized clinical involvement.

Awada, A.; Rajeh, S.

doi: 10.1111/j.1600-0404.1999.tb00392.xpmid: N/A

Objectives ‐ To determine the types of stroke, their risk factors and their most likely causes in Saudi patients. Methods ‐ Data on stroke cases admitted to 2 major hospitals in Saudi Arabia since 1982 were collected retrospectively up to 1991 then prospectively since then. By January 1995, the number of cases with first‐ever‐stroke stored in our Saudi Stroke Data Bank reached 1280. This article describes the findings in the first 1000 Saudi patients investigated by brain computed tomography. Results ‐ Males (68%) outnumbered females. There was no significant difference between the retrospective cases and the prospective ones in relation to the types of stroke or the risk factors. Ischemic strokes accounted for 76% of the cases and one third of them were lacunar infarcts. Most of the hemorrhagic strokes were intracerebral hemorrhages (ICHs) and only 2% of all strokes were subarachnoid hemorrhages (SAHs). Hypertension (52%), diabetes mellitus (41%) and cardiac disorders were common risk factors. The commonest causes of cerebral infarcts were atherosclerosis 36%, hypertensive and/or diabetic arteriolopathy 24% and cardiac embolisms 19%. Hypertensive arteriolopathy accounted for two‐thirds of the cerebral hemorrhages. Strokes related to small artery disease, i.e. lacunar infarcts and ICHs accounted for 47% of the cases. Conclusion ‐ The overall distribution of stroke types in Saudis is not very different from that reported in western studies, except for the low frequency of SAH. However, the important role of small artery disease in stroke pathogenesis and the high number of diabetic patients are quite distinctive.