Two patients with maysthenic syndrome apparently not associated with carcinoma were studied in detail with repetitive stimulation of motor nerves, single fibre eleetromyography and histology. One patient showed signs of widespread autonomic dysfunction and the other patient had from the presynaptic terminals, usually produces a good relief of the symptoms (Karnenskaya e f al. 1975 a, b, Elmqvisf & Larnberf 1968).We recently had the opportunity to study in some detail two cases of myasthenic syndrome apparently not associated with carcinoma. Both cases fulfilled clinical and electrophysiological criteria of the disorder.CASE REPORTS Case f. A 64-year-old woman with a history of right-sided kidney stones but otherwise previously healthy. Since March 1972 she had experienced multifocal CNS symptoms including a constant disabling tiredness and suddenly developing attacks resembling multifocal epileptic seizures. During the attacks lasting for a few minutes she had tonic and clonic muscle contractions in different sides of the body at different attacks. Consciousness was retained but the ability to speak was lost.Once such an attack proceeded into a grand ma1 seizure. From March 1972 a slowly progressing dysarthria and a weakness of proximal leg muscles were noted. The latter symptoms showed spontaneous day-to-day fluctuations. At the beginning of 1975 she could only walk short distances and often fell in her home. At that time she started to use a wheelchair and the dysarthria was very pronounced.At the first admittance to the ward in November 1972, neurological examination revealcd only a moderate dysarthria, a weakness of proximal limb muscles most pronounced in the legs, and a lack of the ankle jerks whereas the other muscle reflexes were weak. Hemogram and laboratory tests of serum including protein electrophoresis, electrolytes, creatinine, alcaline phosphatases, y-glutamic transferase, aspartate-amino transferase, alaninc-amino transferase, creatine phosphokinase, fasting blood glucose, and VDRL-Meinicke were all normal. Routine tests of urine and tests for porphyria (porphobilinogen and A-amino levolinic acid in urine) were negative. CSF examination for cells, protein concentration, electrophoretic protein pattern, and hemoglobin extinction was also normal. Neurootological examinations performed by Dr. N. G. Henriksson (Neuro-otological Department, University Hospital, Lund) on five occasions during the disease (before and during guanidine treatment, see below) showed a persistent and pronounced impairment of the fast component of the vestibular and optokinetic nystagmus. The pursuit eye movements showed a varying degree of disturbance, while no clear-cut abnormality was found in the voluntary saccades. The findings were interpreted as a general disturbance of the fast phase system in the parapontine gaze centre. Repeated electroencephalography and isotope encephalography examinations were normal. Pneumoencephalography failed on two occasions in 1973 as no air passed intraventricularly. Selective angiography o...
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