Abstracts of the Norwegian Neurological Association, Annual Meeting, Rikshospitalet, Oslo November 27-29, 199 1 -

Reduced left ventricular diastolic function in patients with my asthenia gravis

Ecchocardiographic parameters of left ventricular diastolic filling are very sensitive measures of cardiac performance. Using M-mode and Doppler ecchocardiography, we examined left ventricular diastolic and systolic function in 27 patients with myasthenia gravis (MG) and in 27 age and heart rate matched normals. Impaired diastolic func- tion is characterized by a reduced peak diastolic filling rate (PFR) as assessed by digitized M-mode ecchocardiography and a reduced Doppler early left ventricular filling velocity (E), whereas the late (atrial) filling velocity (A) is compensatory increased. Consequently the E:A ration is decreased. In the MG patients PFR was 2.7 k 0.7 s ' as compared to 4.2 1.0 s - ' (pt0.05) in the normals. E was also reduced (p<O.O5) and A was increased (p<O.Ol) resulting in a re-

Mygland i\, Johannessen' KA, Aarli JA, Gilhus NE

Departments of Neurology, 'Clinical Psychology, University of Bergen, Norway

duced E:A ratio of 1.21 + 0.4 cm/s as compared to 1.81 k 0.3 cm/s (p < 0.01) in normals. Systolic measures were normal in patients. PFR in 8 MG patients with antibodies against heart muscle (Hab) (5 with thymoma, 1 with thymic atrophy, 2 not thymectomized) was 2.1 & 0.4 s - 1 as compared to 3.0 0.7 s - 1 (p <0.01) in 19 MG pa- tients without Hab (9 with thymic hyperplasia, l with thymic atrophy, 9 not thymectomized). E:A in MG patients with Hab was 0.86 k 0.3 m/s as compared to 1.36 k 0.3 m/s (p i0 .01) in MG pa- tients without Hab. Conclusion: Diastolic function is impaired in some MG patients. This seems to be correlated to the presence of heart muscle antibodies. The causative relationship is not clear.

Autoimmunity and thymus pathology in myasthenia gravis

We have examined clinical and serological evidence of auto-immunity in 3 groups of thymectomized patients with myasthenia gravis (MG), 16 with thymoma, 16 with thymic atrophy and 32 with follicular hy- perplasia of the thymus. All 16 patients with thymoma, 15/16 with thymus atrophy and 30/32 with follicular hyperplasia had AChR an- tibodies. Non-receptor muscle (CA) antibodies were found in sera of 15/16 patients with thymoma, 3/16 with thymus atrophy and in none of the sera from patients with follicular hyperplasia. There were 2

Aarli JA, Gilhus NE, Matre R

Departments of Neurology, Microbiology and Immunology, The Gade Institute, University Hospital, University of Bergen, Norway

patients with thymoma who had polymyositis, but none of the thy- moma patients had rheumatoid arthritis, systemic lupus erythemato- sus or other autoimmune disorders. Among the 32 patients with fol- licular hyperplasia of the thymus were 2 with SLE, 2 with RA and 1 with juvenile diabetes mellitus. In this study, there was an increased incidence of non-muscle autoimmune disorders among MG patients with follicular hyperplasia of the thymus but not among MG patients with thymoma.

Botulinus toxin treatment in spasmodic torticollis Kerty E

Department of Neurology, Rikshospitalet, Oslo

patients noticed a slight increasing of head tremor. When injections were repeated every third month, we noticed an increase of the symp- tomatic effect. The results of this study indicate that botulinus toxin is an effective means of treatment for torticollis, but it should be used with caution. Some clinical examples will be shown by video.

Botulinus toxin A was administered to 19 patients with spasmodic torticollis. Some improvement in head position and control occurred in all except one patient. The clinical effect was confirmed by blind videotape rating before, and 4 weeks after, injection. All the patients who had pain before botulinus toxin treatment reported relief. Two patients had mild transient dysphagia as side effect and two other

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Abstracts

Local injections of botulinus toxin in the treatment of cervical dystonia Borgmann R

Since december 1990 a total of 20 patients with cervical dystonia have been treated with local injections of botulinus toxin. Fourteen patients have been evaluated at least 3 months after the first injections as to effect and side-effects.Twelve of the 14 patients (85%) had a peak effect ranging from 1 (mild improvement) to 4 (no symptoms left) on a 0-4 scale. Seven of the 14 patients (50%) had still some effect (1-3) 3 months after the last injections. Five patients have been evaluated after the second or more series of injections and it seems to be a tendency to increased improvement both as to the peak effect and the

Department of Neurology, Haukeland University Hospital, Bergen, Norway

effect at least 3 months after the last injections. Five patients did not report any side-effect at all. Five patients had experienced dysphagia, 4 had increased neck pain, 2 had noted neck weakness and 1 had suffered some nausea (some patients had experienced more than 1 side-effect). The side-effects were mild and transient with duration 1-3 weeks, usually starting within the first or second day after the injections.None of the patients wanted to withdraw from further treat- ment because of the side-effects.

Organic solvents induce aggregation and secretion through the inositol phospholipid signal system in human platelets

Workers exposed to organic solvents may develop symptoms like concentration problems, sleep disturbances, loss of memory and ir- ritability. With time they present a picture of encephalopathy which can be explained by platelet microembolization in the brain. Such mechanism has recently been proposed for long term effects in pa- tients that have undergone open heart surgery. Gel-filtered human platelets were exposed to a saturated athmosphere of the solvents toluene, xylene, hexane or ethanol. Within 3 min after the exposure, toluene and xylene induced platelet aggregation and dense granule secretion. Hexane induced similar changes within 9 min of exposure whereas ethanol induced platelet permeabilization without activation since liberation of adenine nucleotides in this case also persisted in energy-depleted cells. When the metabolic pool of the adenine nucle-

Tysnes OB, Kyvik KR*

Departments of Neurology, Haukeland Sykehus, Biochemis- try, University of Bergen, Norway

otides was labelled, it became confirmed that the organic solvents toluene, xylene and hexane induced release of granule stored nucle- otides. The cells were in another set up labelled with 32P-Pi prior to exposure. The data demonstrate that the organic solvents induced accumulation of phosphatidic acid which indicates activation of phos- pholipase C . Simultaneously there is an increase in phosphatidylinos- itol4-phosphate together with a decrease in phosphatidylinositol4,5- bisphosphate. These data cannot be explained by changes in the metabolism of metabolic ATP which appeared at constant specific ”P-radioactivity throughout the experiments. The experiments dem- onstrate that platelets become activated upon exposure to organic solvents. This effect may explain some of the symptoms presented in the “solvent-induced encephalopathy” syndrome.

Regulation of mRNA encoding receptors for IgG (FcRIII) in rat sciatic nerve during development, Wallerian degeneration, and experimental allergic neuritis (EAN)

Vedeler CA, Conti G, Bannerman P, Pleasure D

Departments of Neurology, Haukeland Hospital, Norway, and University of Pennsylvania, Philadelphia, USA

EAN elicited by sensitization to a neuritogenic P2 peptide, FcRIII mRNA levels increased during the period when weakness is most severe. Thus, expression of FcRIII mRNA in PNS is developmentally regulated and is transiently increased by physical or immune-mediated injury. By in situ hybridization studies it was evident that macrophages contributed to the increase in FcRIII mRNA levels that occur with Wallerian degeneration and EAN.

FcRIII, a 50-70 kDa plasma membrane glycoprotein, binds aggre- gated IgG and participates in the initiation of cytotoxic responses by NK cells and phagocytosis by macrophages. In the present study, Northern blots were used to examine the effects of maturation, Wal- lerian degeneration, and EAN on expression of FcRIII mRNA in rat sciatic nerves. The ratio of FcRIII mRNA to total sciatic nerve RNA is greater in adults than in 6 or 21 day postnatal rats. In adults, ex- pression of FcRIII mRNA is induced by sciatic nerve transection. In

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Abstracts

Patients with epilepsy and intracranial aneurysms or AV-malformations

A total of 508 patients (259 men and 249 women) with epilepsy, 15 years or more, were seen as in- and outpatients in the Tromse De- partment of Neurology and the department’s ambulatory neurology service in Troms and Finnmark in 1988. Age is referring to Decem- ber 3 1, 1989, as the prevalence day. 26 patients (5.1 % of the total one years material) had aneurysms or AV-malformations and in the ma- jority this was regarded as the most likely cause of epilepsy. Mean age at prevalence day was 53.0 & 15.6. Mean age at debut of epilepsy was 39.5 2 16.1.50% had active epilepsy (with seizures the last two years). There were 12 patients with aneurysms, 8 of them had been bleeding, 14 had AV-malformations - 6 of them with a history of hemorrhage. The debut of epileptic seizures occurred significantly earlier in the group with AV-malformations (mean 33.6 & 16.3 years) compared with the group with aneurysms (mean 46.3 13.3) (p = 0.04). The

MRI-disordered neuronal migration and seizures

We report two cases of seizures and disordered neuronal migration. Case 1, 18 years old. Past medical history foot presentation, prema- ture, birth weight 2.2 kgs. From the age of seven months to four years she had febrile convulsions. At the age of ten she began to have sei- zures with automatisms. Clinical examination revealed possible bilat- eral papilloedema. Comprehensive investigations including a CT scan showed a low-density mass in the left occipital region, thought to be a malformation. Although EEG monitoring only showed a slow-wave activity in the left fronto-temporal region, antiepileptic treatment was started. In the subsequent years she suffered from seizures, headache, depression, episodes of agression and learning disabilities. In septem- ber 1989 all medication was withdrawn. Her mother reported a gen- eral improvement in all symptoms, but also mentioned outbursts of unmotivated laughter, groans, grunt, odd movements of her hands and automatic behaviour. Clinical examination was normal apart from an incomplete homonymous hemianopia on the right side. Her intellec-

Albretsen C, Mellgren SI, Joakimsen 0

Department of Neurology, University of Troms~, N-9038 Regionsykehuset i T r o m s ~

mean bleeding age in the AV-group was 40.8 t 17.1 and 43.3 14.5 in the aneurysm group (n.s.). 21 (80.8%) had tonic clonic seizures probably representing secondary generalization of unrecognized par- tial seizures in many cases. Five patients (19.2%) had a recorded history of complex partial seizures and 6 (23.1%) simple partial sei- zures. All patients had pathological EEG and in 17 (65.4%) focal epileptogenic changes were described. CT-scan had been performed in 24 patients and findings in accordance with AV-malformations/ angiomas were reported in 12 (46%). This study supports, as ex- pected, that the debut of epileptic seizures in patients with AV- malformations are significantly earlier than in those with aneurysms. All patients being on antiepileptic drugs and 50% with active epilepsy indicate a relatively “aggressive” epilepsy in these patients.

Strandjord RE, Gjerde 10, 0degArd H

Departments of Neurology, SiR Stavanger, Haukeland Hos- pital, Bergen, Radiology SIR Stavanger

tual ability was found at the lower border of the normal range with marked scattering in the test results indicative of a neurophysiologi- cal dysfunction. MRI demonstrated a complex heterotopia compris- ing partly as schizencephaly, polymicrogyria and pachygyria. Case 2, 33 years old. A past medical history of syncope with convulsions and attacks of bilateral headache followed by nausea, occasionally numb- ness in the right arm. She presented with a seizure starting in the right side, loss of consciousness and generalized convulsions. Clinical ex- amination and EEG recorded awake and during sleep were normal. A CT scan of the brain showed a split shaped left lateral ventricle. MRI demonstrated a left sided frontal heterotopia compressing the lateral ventricle. Based on the medical history and the MRI finding antiepileptic treatment was started. In conclusion: MRI due to its good delineation may show mal-migration that are invisible or not interpretable by CT.

Epilepsy and anomalies of neuronal migration MRI and clinical aspects

Neuronal migration disorders are the result of a disturbed brain de- velopment. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowl- edge on the clinical correlates of neuronal migration. We present 13 patients (aged 12-41, mean age 27) with migrational disorders and epilepsy. They represent 4.3% of the 303 patients with epileptic sei- zures examined with magnetic resonance imaging during a three-year period. Of the patients with known epilepsy, 6.7% and of the men-

Brodtkorb‘.’ E, Nilsen’ G, Smevik’ 0, Rinck’ PA ’MR-Center, University of Trondheim, ’Department of Neu- rology. Trondheim University Hospital

tally retarded patients, 13.7% had migrational disorders. Four pa- tients had schizencephaly as the dominating finding, one was classi- fied as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or polymicrogyria. The clinical pictures are com- plex. Ectopias of grey matter are recognized foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.

334

Abstracts

A poet and his epilepsy Aarli JA

Department of Neurology, Haukeland Hospital, University of Bergen

same visual hallucination - “seeing a face” - changing into an intense shining spot which again was followed by unconsciousness. It is unique that these attacks are described in such a detail by an author who was uninformed about the nature of his disease. The diagnosis was not revealed to him until around 1905-1910. Private letters, recently dis- covered, have revealed the sad depressive effect, often lasting for days and weeks, of the nocturnal seizures upon his creativity.

The Norwegian author Tryggve Andersen (1866-1920) suffered from epilepsy from childhood. Except for one seizure that occurred during daytime under exceptional circumstances, he had only nocturnal at- tacks. He wrote two novels, “Days of the Councillor” (1897) and “Towards Evening” (1900), the last carrying a distinct autobiographic character. “Towards Evening” contains detailed descriptions of the initial phenomena of four such attacks, all of them starting with the

Cerebral cysticercosis in Norway

Although endemic to many parts of the world, cysticercosis is rare in the Scandinavian countries. Caused by infestation with the larval state of the tapeworm Tueniu solium, cysticercosis may give clinical mani- festations from various tissues including brain. Recently, three pa- tients with conceivable cerebral cysticercosis have been admitted to our hospital. They were all either immigrants from, or had been vis- itors to, endemic areas. We here described one case where the diag- nosis has been confirmed by serology. A 53-year-old male Norwegian

Tyssvang T, Dietrichs E, Aanonsen NO, Skullerud K, Bakke SJ

Departments of Neurology, Pathology and Radiology, Rikshospitalet, The National Hospital, Oslo, Norway

was admitted with increasing right hemiparesis, headache and vom- iting. Cerebral CT revealed a cystic process with massive surround- ing edema in the left hemisphere. The patient rapidly developed signs of increasing intracranial pressure, and a craniotomy with resection of pathological tissue was performed. Histology showed eosinophilic granulomas, suggestive of parasite infection. The patient was treated with Praziquantel. He has some dysphasia as a sequela after the craniotomy, but has otherwise recovered well.

Transverse branching of olivocerebellar fibres in the cat Dietrichs’ E, Apps’ R, Trott‘ J R Departments of ’Neurology, National Hospital, Institute for Basic Medical Sciences, University of Oslo, Norway, ’Physi- ology, University of Bristol Medical School, Bristol, England

green fluorescent tracer were found in the middle part of the medial accessory olive. Cells double labelled with red and green fluorescence represented only 5-7% of either single labelled cell population within the region which contained intermingled red and green cells. Although some transverse branching within the olivocerebellar projection to the x and lateral c l zones is present, such branching does not appear to be extensive.

Transverse branching in the inferior olivary projection to the parasag- ittal zones x lateral c l of the cerebellar cortex was studied in the cat. The location of the x and c l zones was determined by recording ex- tracellular climbing fibre field potentials after percutaneus stimulation of the left or right forepaws. These recordings were used to guide injections of rhodamine labelled (red) latex microspheres into one zone and coumarin labelled (green) latex microspheres into the other. After one week survival neurones retrogradely labelled with red or

Technical note: a simple device for quantifying finger-, elbow, and ankle movements Bekkelund SI, Winther J, Mellgren SI

Department of Neurology, University of Tromsi , Regionsykehuset i Tromspl

with temperature and non-invasive blood pressure measurement fa- cilities. The finger tapping is recorded on the ECG-monitor as pulse waves. The frequency of pulses and the duration of each pulse give quantitative information of limb movements. A computer program for recording these parameters in under development.

A method of measuring the velocity of limb movements is described. A metal plate and a plastic ring surrounded with copper wire are connected to an electronic box. The patient wears the ring on the index finger and instructed to move the, for example, finger against the plate as fast as possible for a period of 5 s causing the electronic box to give a signal. The electronic box is connected to a portable ECG-monitor

335

Abstracts

Autonomic neuropathy in SLE (Systemic lupus matched controls

The study was performed to investigate features of autonomic neu- ropathy in systemic lupus erythematosus. 34 (2 males, 32 females) with SLE and 34-age and sex-matched healthy controls were exam- ined with determination of neuropathy symptom score, heart rate response to Valsalva maneuver, hear rate response to standing up, heart rate response to deep breathing, blood pressure response to standing up, blood pressure response to sustained handgrip, and over- all autonomic test score. Pancreatic polypeptide (PP) was examined in blood immediately before food intake and then at 30 and 60 min

erythematosus): a study including age and sex-

Omdal R, Jorde R, Mellgren SI, Husby G

Department of Rheumatology and Neurology, University of Trorns~, Regionsykehuset i Troms0

after the meal. Autonomic symptoms were infrequent and there was no difference between SLE patients and healthy controls. There were statistically significant differences between healthy controls and SLE patients in blood pressure response to sustained handgrip and over- all assessment of cardiovascular tests. Surprisingly there was an in- creased basal and stimulated PP in SLE patients. The results indicate that both the sympathetic and parasympathetic nervous system are diffusely affected in SLE, but P P does not seem to be a useful marker of autonomic dysfunction in SLE.

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