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Immunoglobulin treatment in myasthenia gravis
$85 moved around like wooden dolls. In all the cases, in different arm and leg muscles, during full relaxation, multiplets and serial high-frequency irregular repetitive discharges of small motor unit type were recorded. Neurography pointed in some patients to distal motor and sensory fiber involvement. The finding of generalized limb stiffness and atypical ataxia, along with generalized spontaneous electromyographic activity of the type described, should therefore be considered as pointing to reduced function of the parathyroid glands as much as calcium and phosphate levels, cataract or brain calcifications do.
P 2.06 I M M U N O G L O B U L I N ASTHENIA GRAVIS.
TREATMENT
IN
MY-
strated brief, small amplitude, polyphasic potentials with early recruitment. Motor conduction velocity was normal. The amplitude of the compound muscle action potential (CMAP) was low (0.15 mV). Repetitive stimulation at 3 Hz elicited a 50% decrement in the fifth response. At 50 Hz an increment of 4000% was noted. Guanidine by mouth induced a significant improvement in muscle tone, increased the amplitude of the C M A P to 2 mV and reduced potentiation to 400%. Muscle biopsy showed a fibre-type disproportion with relative atrophy of type II fibers. Lambert-Eaton Myasthenic Syndrome is usually associated with carcinoma or autoimmune disorders. This case could be the first congenital one. It is noteworthy that this child exhibited some features of congenital myopathy.
B. Rossi, G. Siciliano, F. Sartucci, C. loppoli and L. Murri (Pisa, Italy) Five subjects (2 males, 60 and 64 years old and 3 females 20, 62 and 67 years old) affected by myasthenia gravis, grade IIB, following Osserman and Genkins (1971), have been treated with immunoglobulin (veinoglobuline Merieux) i.v., 0.4 g r / k g / d a y for five days; the therapeutic scheme (oral pyridostigmine bromide in four patients, no other treatment in one) was otherwise unchanged during the trial. Before, 10 and 25 days after the beginning of immunoglobulin, patients were evaluated by disability status scale (dss) of motor function and by the repetitive stimulation technique on facial, axillary and ulnar nerves. The percent global mean value of dss improved from 70.2% baseline to 85.7% at the 25th day. An improvement of 18% was detected for ocular scores, of 16% for V, VIi, IX, and X cranial nerves, and of 23% for trunk and limbs. All subjects, save case 1, showed a global increase of strength of 15 19%. The EMG showed a sharp improvement in cases 4 and 5 (80-909;, reduction of decrement), and nonsignificant change in cases 1 and 2.
P 2.07 CONGENITAL LAMBERT-EATON M Y A S T H E N I C SYNDROME.
P 2.08 A N T I C H O L I N E S T E R A S E S A N D SINGLE FIBRE ELECTROMYOGRAPHY.
D.J. Baker and E.M. Sedgwick (Salisbury, UK) In a double blind study, single fibre electromyography was recorded in the extensor digitorum communis of 24 fit, male volunteers taking oral pyridostigmine 3 0 mg t.d.s, who received single low level exposures of the organophosphate Sarin. Corrected red cell acetylcholinesterase levels produced by pyridostigmine and Satin were 58.5% and 84% of normal respectively. The results were analysed using standard clinical criteria of normal electro-myographic jitter and by reciprocal transformation of jitter populations. Satin caused a small but significant change in the mean jitter of all fibre pairs recorded in exposed subjects, which was prevented by pyridostigmine pretreatment. Overall, a paired analysis showed a very small change in mean jitter in all subjects taking pyridostigmine but two of the twelve subjects treated developed abnormal jitter without any detectable clinical abnormality, The results are discussed with respect to possible prejunctional as well as postjunctional effects of the two anticholinesterases.
B. Bad3,, G. Chauplannaz and H. Carrier
P 2.09 S T A N D A R D I Z A T I O N OF C H A N G E S IN M-WAVE AREA TO REPETITIVE STIMULATION OF THE ULNAR NERVE.
(Lyon, France)
I. Aiello, S. Traccis, G.F. Sau, G. Serra and D De Grandis
A 5 year-old girl had been hypotonic with reduced spontaneous motility since birth. At 3 years of age she was unable to sit but slight arm and leg movements were observed. One year later she could sit unaided but was unable to stand unsupported; she had no facial weakness, ptosis, or ophthalmoplegia. Deep tendon reflexes were not obtained. She had kyphoscoliosis and hip dysplasia. There was no family history of muscle disease and no consanguinity. Needle E M G performed at 30 days and 3 years demon-
(Sassari, Italy) The changes in amplitude of the compound muscle action potential (M-wave) in different repetitive nerve stimulation tests have been widely studied in normal subjects and in patients suffering from neuromuscular diseases. Conversely, variations of M-wave area in the same tests have not yet been analysed. This electrophysiological parameter may increase the diagnostic sensitivity of these tests since unlike amplitude, the
P 2.06 I M M U N O G L O B U L I N ASTHENIA GRAVIS.
TREATMENT
IN
MY-
strated brief, small amplitude, polyphasic potentials with early recruitment. Motor conduction velocity was normal. The amplitude of the compound muscle action potential (CMAP) was low (0.15 mV). Repetitive stimulation at 3 Hz elicited a 50% decrement in the fifth response. At 50 Hz an increment of 4000% was noted. Guanidine by mouth induced a significant improvement in muscle tone, increased the amplitude of the C M A P to 2 mV and reduced potentiation to 400%. Muscle biopsy showed a fibre-type disproportion with relative atrophy of type II fibers. Lambert-Eaton Myasthenic Syndrome is usually associated with carcinoma or autoimmune disorders. This case could be the first congenital one. It is noteworthy that this child exhibited some features of congenital myopathy.
B. Rossi, G. Siciliano, F. Sartucci, C. loppoli and L. Murri (Pisa, Italy) Five subjects (2 males, 60 and 64 years old and 3 females 20, 62 and 67 years old) affected by myasthenia gravis, grade IIB, following Osserman and Genkins (1971), have been treated with immunoglobulin (veinoglobuline Merieux) i.v., 0.4 g r / k g / d a y for five days; the therapeutic scheme (oral pyridostigmine bromide in four patients, no other treatment in one) was otherwise unchanged during the trial. Before, 10 and 25 days after the beginning of immunoglobulin, patients were evaluated by disability status scale (dss) of motor function and by the repetitive stimulation technique on facial, axillary and ulnar nerves. The percent global mean value of dss improved from 70.2% baseline to 85.7% at the 25th day. An improvement of 18% was detected for ocular scores, of 16% for V, VIi, IX, and X cranial nerves, and of 23% for trunk and limbs. All subjects, save case 1, showed a global increase of strength of 15 19%. The EMG showed a sharp improvement in cases 4 and 5 (80-909;, reduction of decrement), and nonsignificant change in cases 1 and 2.
P 2.07 CONGENITAL LAMBERT-EATON M Y A S T H E N I C SYNDROME.
P 2.08 A N T I C H O L I N E S T E R A S E S A N D SINGLE FIBRE ELECTROMYOGRAPHY.
D.J. Baker and E.M. Sedgwick (Salisbury, UK) In a double blind study, single fibre electromyography was recorded in the extensor digitorum communis of 24 fit, male volunteers taking oral pyridostigmine 3 0 mg t.d.s, who received single low level exposures of the organophosphate Sarin. Corrected red cell acetylcholinesterase levels produced by pyridostigmine and Satin were 58.5% and 84% of normal respectively. The results were analysed using standard clinical criteria of normal electro-myographic jitter and by reciprocal transformation of jitter populations. Satin caused a small but significant change in the mean jitter of all fibre pairs recorded in exposed subjects, which was prevented by pyridostigmine pretreatment. Overall, a paired analysis showed a very small change in mean jitter in all subjects taking pyridostigmine but two of the twelve subjects treated developed abnormal jitter without any detectable clinical abnormality, The results are discussed with respect to possible prejunctional as well as postjunctional effects of the two anticholinesterases.
B. Bad3,, G. Chauplannaz and H. Carrier
P 2.09 S T A N D A R D I Z A T I O N OF C H A N G E S IN M-WAVE AREA TO REPETITIVE STIMULATION OF THE ULNAR NERVE.
(Lyon, France)
I. Aiello, S. Traccis, G.F. Sau, G. Serra and D De Grandis
A 5 year-old girl had been hypotonic with reduced spontaneous motility since birth. At 3 years of age she was unable to sit but slight arm and leg movements were observed. One year later she could sit unaided but was unable to stand unsupported; she had no facial weakness, ptosis, or ophthalmoplegia. Deep tendon reflexes were not obtained. She had kyphoscoliosis and hip dysplasia. There was no family history of muscle disease and no consanguinity. Needle E M G performed at 30 days and 3 years demon-
(Sassari, Italy) The changes in amplitude of the compound muscle action potential (M-wave) in different repetitive nerve stimulation tests have been widely studied in normal subjects and in patients suffering from neuromuscular diseases. Conversely, variations of M-wave area in the same tests have not yet been analysed. This electrophysiological parameter may increase the diagnostic sensitivity of these tests since unlike amplitude, the