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Physiopathology of Myasthenia Gravis: Staging of single muscle involvement by EMG study of fatigability and weakness
946
Abstracts
MYASTHENIA
GRAVIS IN PREGNANCY:
TWO CLINICAL CASES.
Roberta Ricciardi, Clinica Neurologica-Universitd, via Roma 67, 56100 PISA tel. 050/592539 Ricciardi R.,Rossi B.,Mantegazza R?,Cornelio FZ,Muratorio A. Institute of Neurogical Clinic, University of Pisa -Departments of Neuromuscolar Diseases,Istituto Neurologico"C.Besta"
Milan
Osserman and Kaplan (1952) reported that 45% of myasthenia gravis (MG) cases improved during pregnancy, 30% showed a impairment of the clinical picture and 25% resulted unchanged. Others Authors confirmed that the first three months of pregnancy constitute a critical phase in the menagement of MG, both improvement and worsening occuring. No data referred to the trend of acetylcolinic anti-receptors antibodies (AchRab) serum titres, regarding the pregnancy, are reported in the literature. In this work clinical data and AchRab modifications (Bovine antigen method) concerning two MG cases during pregnancy are reported. Case I: female 37 gears old classified anticholinesterasic therapy.
2A according
Case II: female 32 years old classified 28 according anticholinesterasic and steroid therapy.
to Osserman, to Osserman,
in chronic in chronic
Clinical evaluation was performed by an MG score system. The system provides a numerical score for muscular strength and resistance. Four different disability levels are recognized: ocular, generalized, bulbar and respiratory. The final assessment of the condition of a patient is expressed by the total sum of the scores rated for each of the functions taken into account. The sum of the partial scores during pregnancy decreased from 72 to 22 in Case I, from 5110 to 1040 in Case II. The dosage of pyridostigmina could be decreased from 240 mg/die to 45 mg/die in Case I, from 420 mg/die to 165 mg/die in Case II. Also the dosage of prednisone was reduced from 80 mg to 40 mg, every other day, in Case II. AchRab serum titre decreased from 0.138 PM/ml ( N.V. 0 PM/ml) to 0 pM/ml in Case I, from 195.39 pM/ml to 120.97 pM/ml in Case II. This study schowed a clear clinical improvement of myasthenic symptoms in these two patients, with progressive reduction of the titre of the AchRab.
PHYSIOPATHOLOGY
OF MYASTHENIA
OF SINGLE MUSCLE INVOLVEMENT FATIGABILITY AND WEAKNESS
GRAVIS: STAGING BY EMG STUDY OF
C. SCOPPETTA, II Neurological Institute "La Sapienza" University - ROME - ITALY Viale dell'universita S. D'Agostini,
30 - 00185 - ROME-ITALY
tel 4453954
I. La Cesa, C. Casali
In Myasthenia Gravis (MG) patients, at a given moment each affected muscle has its own degree of involvement because of the ever changing combination of the situation at rest with the effects of exaustion phenomena due to synaptic fatigue. In order to study the involvement of single muscles in MG, we performed serial examinations with repetitive stimulation test (RS) of the extensor carpi brevis muscle in 42 MG patients. Four stages of muscle involvement have been identified: STAGE 0 : NORMAL OR REMISSION: all junctions normally function and the first evoked CMAP (Compound Muscle Action Potential) has normal amplitude. The safe_ ty factor (SF) is normal and the CMAP shows no decrease during RS.
Abstracts STAGE 1
: FATIGABILITY: at rest all junctions normally function and the CMAP
has normal amplitude. But SF is reduced and during RS the CMAP amplitude progressively decreases (decremental response). Anticholinesterase drugs are able to reduce or abolish the decremental response. STAGE ZA : FATIGABILITY AND WEAKNESS: some junctions are already blocked at rest and the initial CMAP is therefore reduced; during RS a further decrement is recorded. Anticholinesterase drugs can both increase the initial CMAP ampli _ tude and reduce the decremental response. STAGE 2B : WEAKNESS WITHOUT FATIGABILITY: except for a few normally working junctions both at rest and during RS, most synapses are already blocked in ba sal condition and the corresponding CMAP is low in amplitude. On R'S no further decrement is produced. The administration of anticholinesterase drugs can partially reverse some blocks resulting in an increased initial CMAP amplitude. But in those labile junctions the SF is actually reduced; therefore on RS a decremental response becomes apparent.
Myasthenia gravis and myasthenic syndromes in children Peter Spalsk, MD, Steinerova 32, 811 07 Bratislava, Czechoslovakia
phone: 639-212
Peter Spalek, Daniel Orolin, VBclav Smat Bratislava, Prague, Czechoslovakia In Slovakia (one of two federal republics of Czechoslovakia), a country with population of 6 million, 264 myasthenia gravis patients are registered. 25 had the onset of their myasthenic s mptoms in childhood prior the age of 15 years. 19 children (17 girls, 2 boys) were treated with transsternsl thymectomy because of disease severity. The median follow-up of these patients is 8,4 years. Achieved results: Remission in 9 children (47,4:;) Marked improvement in 4 children (21,0x) Mild improvement in 2 children (10,67&) No effect in 4 children (21,0?,) The results were in strong correlation to preoperative duration of myasthenia gravis. Remissions were achieved in children who had before thymectomy an average duration of myssthenia gravis 8,9 months, while the others 46,1 months. Thymectomy was ineffective in 4 patients. These 4 children improved markedly only after immunosuppressive treatment (prednisone and/or azathioprine). 3 years old girl with acute fulminant myasthenia gravis has been treated by alternate-day dose of prednisone. Soon after its initiation she developed pneumonia and died. Four children have a relatively benign course of myasthenia gravis with spontaneous remissions and there is no need for immunosuppressive tharap or thymectomy. One girl had typical c'i. lnical and electraphysiological findings of Lambert-Eaton myasthenic syndrome associated with myasthenia gravis. She improved clearly after thymectomy, high-doss intravenous methyl-prednisolone pulses and long-term prednisone therapy. Autosomal recessive inherited congenital myasthenic syndrome (familial infantile myasthenia) with non-progressive course has been diagnosed in two pairs of gipsy siblings.
947
Abstracts
MYASTHENIA
GRAVIS IN PREGNANCY:
TWO CLINICAL CASES.
Roberta Ricciardi, Clinica Neurologica-Universitd, via Roma 67, 56100 PISA tel. 050/592539 Ricciardi R.,Rossi B.,Mantegazza R?,Cornelio FZ,Muratorio A. Institute of Neurogical Clinic, University of Pisa -Departments of Neuromuscolar Diseases,Istituto Neurologico"C.Besta"
Milan
Osserman and Kaplan (1952) reported that 45% of myasthenia gravis (MG) cases improved during pregnancy, 30% showed a impairment of the clinical picture and 25% resulted unchanged. Others Authors confirmed that the first three months of pregnancy constitute a critical phase in the menagement of MG, both improvement and worsening occuring. No data referred to the trend of acetylcolinic anti-receptors antibodies (AchRab) serum titres, regarding the pregnancy, are reported in the literature. In this work clinical data and AchRab modifications (Bovine antigen method) concerning two MG cases during pregnancy are reported. Case I: female 37 gears old classified anticholinesterasic therapy.
2A according
Case II: female 32 years old classified 28 according anticholinesterasic and steroid therapy.
to Osserman, to Osserman,
in chronic in chronic
Clinical evaluation was performed by an MG score system. The system provides a numerical score for muscular strength and resistance. Four different disability levels are recognized: ocular, generalized, bulbar and respiratory. The final assessment of the condition of a patient is expressed by the total sum of the scores rated for each of the functions taken into account. The sum of the partial scores during pregnancy decreased from 72 to 22 in Case I, from 5110 to 1040 in Case II. The dosage of pyridostigmina could be decreased from 240 mg/die to 45 mg/die in Case I, from 420 mg/die to 165 mg/die in Case II. Also the dosage of prednisone was reduced from 80 mg to 40 mg, every other day, in Case II. AchRab serum titre decreased from 0.138 PM/ml ( N.V. 0 PM/ml) to 0 pM/ml in Case I, from 195.39 pM/ml to 120.97 pM/ml in Case II. This study schowed a clear clinical improvement of myasthenic symptoms in these two patients, with progressive reduction of the titre of the AchRab.
PHYSIOPATHOLOGY
OF MYASTHENIA
OF SINGLE MUSCLE INVOLVEMENT FATIGABILITY AND WEAKNESS
GRAVIS: STAGING BY EMG STUDY OF
C. SCOPPETTA, II Neurological Institute "La Sapienza" University - ROME - ITALY Viale dell'universita S. D'Agostini,
30 - 00185 - ROME-ITALY
tel 4453954
I. La Cesa, C. Casali
In Myasthenia Gravis (MG) patients, at a given moment each affected muscle has its own degree of involvement because of the ever changing combination of the situation at rest with the effects of exaustion phenomena due to synaptic fatigue. In order to study the involvement of single muscles in MG, we performed serial examinations with repetitive stimulation test (RS) of the extensor carpi brevis muscle in 42 MG patients. Four stages of muscle involvement have been identified: STAGE 0 : NORMAL OR REMISSION: all junctions normally function and the first evoked CMAP (Compound Muscle Action Potential) has normal amplitude. The safe_ ty factor (SF) is normal and the CMAP shows no decrease during RS.
Abstracts STAGE 1
: FATIGABILITY: at rest all junctions normally function and the CMAP
has normal amplitude. But SF is reduced and during RS the CMAP amplitude progressively decreases (decremental response). Anticholinesterase drugs are able to reduce or abolish the decremental response. STAGE ZA : FATIGABILITY AND WEAKNESS: some junctions are already blocked at rest and the initial CMAP is therefore reduced; during RS a further decrement is recorded. Anticholinesterase drugs can both increase the initial CMAP ampli _ tude and reduce the decremental response. STAGE 2B : WEAKNESS WITHOUT FATIGABILITY: except for a few normally working junctions both at rest and during RS, most synapses are already blocked in ba sal condition and the corresponding CMAP is low in amplitude. On R'S no further decrement is produced. The administration of anticholinesterase drugs can partially reverse some blocks resulting in an increased initial CMAP amplitude. But in those labile junctions the SF is actually reduced; therefore on RS a decremental response becomes apparent.
Myasthenia gravis and myasthenic syndromes in children Peter Spalsk, MD, Steinerova 32, 811 07 Bratislava, Czechoslovakia
phone: 639-212
Peter Spalek, Daniel Orolin, VBclav Smat Bratislava, Prague, Czechoslovakia In Slovakia (one of two federal republics of Czechoslovakia), a country with population of 6 million, 264 myasthenia gravis patients are registered. 25 had the onset of their myasthenic s mptoms in childhood prior the age of 15 years. 19 children (17 girls, 2 boys) were treated with transsternsl thymectomy because of disease severity. The median follow-up of these patients is 8,4 years. Achieved results: Remission in 9 children (47,4:;) Marked improvement in 4 children (21,0x) Mild improvement in 2 children (10,67&) No effect in 4 children (21,0?,) The results were in strong correlation to preoperative duration of myasthenia gravis. Remissions were achieved in children who had before thymectomy an average duration of myssthenia gravis 8,9 months, while the others 46,1 months. Thymectomy was ineffective in 4 patients. These 4 children improved markedly only after immunosuppressive treatment (prednisone and/or azathioprine). 3 years old girl with acute fulminant myasthenia gravis has been treated by alternate-day dose of prednisone. Soon after its initiation she developed pneumonia and died. Four children have a relatively benign course of myasthenia gravis with spontaneous remissions and there is no need for immunosuppressive tharap or thymectomy. One girl had typical c'i. lnical and electraphysiological findings of Lambert-Eaton myasthenic syndrome associated with myasthenia gravis. She improved clearly after thymectomy, high-doss intravenous methyl-prednisolone pulses and long-term prednisone therapy. Autosomal recessive inherited congenital myasthenic syndrome (familial infantile myasthenia) with non-progressive course has been diagnosed in two pairs of gipsy siblings.
947