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Early onset Myasthenia Gravis: Report on 59 patients
936
Abstracts
increased
in two cases, in which a severe relapse occurred after 1 and 10 months of benefit respectively. After each PP treatment there were increases of both total IgG (32.0%&23.7X) and anti AChR abs (63.5%+82.7X). From our data we can conclude that anti AChR abs decrease is higher than total IgG decrease, confirming the semi-selective nature of these columns. It seems difficult to explain the different rate of increment of total IgG and anti AChR abs simply with redistribution from extra-to intra-vascular compartments, or with a specific de novo synthesis of anti AChR abs. Dosage of specific anti AChR subclasses, which is now in progress, will probably shed more light on this intriguing problem.
EARLY 59
OhSET
MYASTHEluIA
GRAVIS:
REPORT
Oh
PATIEhTS
AIUI\IA PAOLA UhIVERSITY,
BATOCCHI,
TEL.
06/33054279
A.P.
BATOCCHI,
IhST.
IluST. OF- hEUROLOGY,
L. F. VITO
1, 00168
A. EVOLI,
OF hEUROLOGY
M.T.
AivD *GENERAL
ROME,
CATHOLIC
ITALY
PALMISAIUI, M. LO MOhACO, PATHOLOGY,
CATHOLIC
E. EARTOCCIOhI*,
UhIVERSITY,
ROME,
P. TOluALI ITALY
We studied 59 patients with Myasthenia Gravis (MG) in whom the onset of symptoms between 1 and 17 years of life. The onset of disease was pre-puberal ((13 years) in 26 patients (group A) and post-puberal in 33 (group B). All patients had typical history and signs of MG with positivity of pharmacoThe repetitive supramaximal stimulalogical (Tensilon) test. tion at 3 Hz gave positive result in 46 cases, 18 (70%) in CRAP response to group A and 28 (85%) in group B; repetitive Anti-Acetylsingle nerve stimulus was absent in all cases. choline receptor antibodies were positive in 35 out of 49 19/26 in group A (73%) and 15/23 in group tested cases (71X), received anticholinesterase drugs All patients B (65%). thyncrtomy wac performed in 43 cases: 9 patients in (AChE); lhvmectomy before the 13th year (the the group A underwent. minimum age at surgery was 9 years). Thirty six patients alternate-day prednisone Plasma-exchange received therapy. The follow-up ranged from 1 to 15 was used in six cases. We found a significant difference between years (mean 8.1). the two groups of patients in sex incidence: male/female ratio was l/1.6 in group A and l/5.5 in group B (~(0.08). The frequency of ocular MG was higher in group A (8/26-31%) than in B (3/33-9X).The positivity of the AChR abs and EMG test, the course of the disease and the respond to therapies were Patients with generalized MG similar in the two populations. showed a good response to thymectomy; corticosteroids proved to be very effective with no major side effects: plasmaexchange was very effective to prevent and treat myasthenic crisis. In our experience MG with onset in childhood has the same We recommend thymectomy for generacourse as in adult life. except in very young chillized disease also in childhood, dren because of possible long-term effects on their immunoloshould be conImmunosuppressive therapy gical development. sidered in severely affected cases who do not respond adeguately to other therapies.
Abstracts
increased
in two cases, in which a severe relapse occurred after 1 and 10 months of benefit respectively. After each PP treatment there were increases of both total IgG (32.0%&23.7X) and anti AChR abs (63.5%+82.7X). From our data we can conclude that anti AChR abs decrease is higher than total IgG decrease, confirming the semi-selective nature of these columns. It seems difficult to explain the different rate of increment of total IgG and anti AChR abs simply with redistribution from extra-to intra-vascular compartments, or with a specific de novo synthesis of anti AChR abs. Dosage of specific anti AChR subclasses, which is now in progress, will probably shed more light on this intriguing problem.
EARLY 59
OhSET
MYASTHEluIA
GRAVIS:
REPORT
Oh
PATIEhTS
AIUI\IA PAOLA UhIVERSITY,
BATOCCHI,
TEL.
06/33054279
A.P.
BATOCCHI,
IhST.
IluST. OF- hEUROLOGY,
L. F. VITO
1, 00168
A. EVOLI,
OF hEUROLOGY
M.T.
AivD *GENERAL
ROME,
CATHOLIC
ITALY
PALMISAIUI, M. LO MOhACO, PATHOLOGY,
CATHOLIC
E. EARTOCCIOhI*,
UhIVERSITY,
ROME,
P. TOluALI ITALY
We studied 59 patients with Myasthenia Gravis (MG) in whom the onset of symptoms between 1 and 17 years of life. The onset of disease was pre-puberal ((13 years) in 26 patients (group A) and post-puberal in 33 (group B). All patients had typical history and signs of MG with positivity of pharmacoThe repetitive supramaximal stimulalogical (Tensilon) test. tion at 3 Hz gave positive result in 46 cases, 18 (70%) in CRAP response to group A and 28 (85%) in group B; repetitive Anti-Acetylsingle nerve stimulus was absent in all cases. choline receptor antibodies were positive in 35 out of 49 19/26 in group A (73%) and 15/23 in group tested cases (71X), received anticholinesterase drugs All patients B (65%). thyncrtomy wac performed in 43 cases: 9 patients in (AChE); lhvmectomy before the 13th year (the the group A underwent. minimum age at surgery was 9 years). Thirty six patients alternate-day prednisone Plasma-exchange received therapy. The follow-up ranged from 1 to 15 was used in six cases. We found a significant difference between years (mean 8.1). the two groups of patients in sex incidence: male/female ratio was l/1.6 in group A and l/5.5 in group B (~(0.08). The frequency of ocular MG was higher in group A (8/26-31%) than in B (3/33-9X).The positivity of the AChR abs and EMG test, the course of the disease and the respond to therapies were Patients with generalized MG similar in the two populations. showed a good response to thymectomy; corticosteroids proved to be very effective with no major side effects: plasmaexchange was very effective to prevent and treat myasthenic crisis. In our experience MG with onset in childhood has the same We recommend thymectomy for generacourse as in adult life. except in very young chillized disease also in childhood, dren because of possible long-term effects on their immunoloshould be conImmunosuppressive therapy gical development. sidered in severely affected cases who do not respond adeguately to other therapies.