Quando a distrofia miotônica foi separada das miopatias primárias ... We selected patients with the diagnosis of Duchenne muscular dystrophy (DMD), limb-.
MUSCLE BIOPSY CORRELATED WITH ELECTROMYOGRAPHY S T U D Y O F 100 C A S E S
LINEU
CESAR
WERNECK*
—
JOSÉ
GERALDO
CAMARGO
LIMA
**
S U M M A R Y — T o f i n d w h a t t h e c o r r e l a t i o n is a n d v e r i f y i f is p o s s i b l e t o a v o i d e x t e n s i v e e l e c t r o m y o g r a p h i c e x a m i n a t i o n , s t u d y i n g o n l y one muscle, 100 p a t i e n t s w i t h neuromuscular d i s o r d e r s (58 p r i m a r y m y o p a t h i e s , 32 n e u r o g e n i c d i s o r d e r s a n d 10 m y o t o n i c d y s t r o p h i e s ) w e r e submited to quantified e l e c t r o m y o g r a p h y ( E M G ) and muscle b i o p s y ( M B ) with fresh-¬ - f r o z e n s e c t i o n p l u s h i s t o c h e m i s t r y in t h e s a m e m u s c l e , b u t o n t h e o p p o s i t e s i d e . T h e E M G w a s a b n o r m a l in 98% a n d M B in 9 3 % o f t h e c a s e s . E M G a n d M B h a d a c o n c o r d a n c e o f 84.3% in t h e n e u r o g e n i c d i s o r d e r s a n d 84.77% in the p r i m a r y m y o p a t h i e s . A c o r r e l a t i o n of 80% w a s o b t a i n e d b e t w e e n all M B a n d E M G ( i n c l u d i n g t h e c a s e s o f m y o t o n i c d y s t r o p h i e s ) , r e g a r d i n g the origin of the p a t h o g e n i c process ( p < 0 . 0 1 ) . T h e E M G h a d 5% i n c o n s i s t e n c e s a n d t h e M B 1 1 % , w i t h r e s p e c t t o t h e p a t h o g e n i c p r o c e s s . W h e n the m y o t o n i c d y s t r o p h y was separated f r o m the p r i m a r y myopathies and f r o m the denervation disorders, a c o m p l e t e c o n c o r d a n c e w a s f o u n d in all M B a n d h a d o n l y 3.4% i n c o n s i s t e n c e s in t h e denervation d i s o r d e r s a n d 3 . 1 % in t h e p r i m a r y m y o p a t h i e s .
Correlação entre biópsia muscular e eletromiografia: estudo de 100 casos. R E S U M O — A fim d e verificar qual a c o r r e l a ç ã o entre uma b i ó p s i a muscular e a eletro-¬ m i o g r a f i a q u a n d o u m ú n i c o m ú s c u l o é e s t u d a d o , b e m c o m o v e r i f i c a r a p o s s i b i l i d a d e d e evitar eletromiografias e x t e n s i v a s e d o l o r o s a s e m d e t e r m i n a d a s d o e n ç a s , f o r a m i n v e s t i g a d o s 100 pacientes c o m doenças neuromusculares (58 c o m m i o p a t i a s p r i m á r i a s , 32 d o e n ç a s q u e d e t e r m i n a m d e s i n e r v a ç ã o e 10 d i s t r o f i a s m i o t ô n i c a s ) . T o d o s o s c a s o s f o r a m s u b m e t i d o s a e l e t r o miografia quantificada ( E M G ) e a biópsia muscular ( B M ) , utilizando técnicas de colorações a fresco e pela histoquímica, realizadas n o m e s m o músculo, mas e m lados o p o s t o s d o p a c i e n t e . A E M G f o i a n o r m a l e m 98% e a B M in 9 3 % d o s c a s o s . A E M G e a B M t i v e r a m c o n c o r d â n c i a d e 84,3% n a s d o e n ç a s n e u r o g ê n i c a s e 84,77% nas m i o p a t i a s p r i m á r i a s . Foi o b t i d a c o r r e l a ç ã o d e 80% e n t r e a E M G e a B M e m t o d o s o s c a s o s , c o m r e l a ç ã o à p a t o g e n i a do processo ( p < 0 , 0 1 ) . A E M G t e v e 5% d e i n c o n s i s t ê n c i a s e a B M 1 1 % , e m r e l a ç ã o a o diagnóstico patogênico. Quando a distrofia miotônica foi separada das miopatias primárias e dos processos que determinam desinervação, u m a concordância completa foi encontrada e n t r e t o d a s as B M e a E M G m o s t r o u i n c o n s i s t ê n c i a s s o m e n t e e m 3,4% d a s d o e n ç a s q u e determinam desinervação e 3,1% das miopatias primárias, s u g e r i n d o q u e a distrofia miotônica d e v a ser c l a s s i f i c a d a e m u m g r u p o s e p a r a d o (neuromiopatias). Os autores discutem os dados encontrados em relação à literatura existente e concluem que, dependendo da avaliação clínica, é possível submeter os pacientes a somente u m d o s p r o c e d i m e n t o s (geralmente a B M ) , u t i l i z a n d o a m b o s o s p r o c e d i m e n t o s s o m e n t e n o s c a s o s e m q u e n ã o foi p o s s í v e l c h e g a r a u m diagnóstico, sendo então examinados diversos músculos na E M G .
Division of Neurology, Internai Medicine Department, Universidade ( U F P R ) and N e u r o l o g y Department, Escola Paulista de Medicina ( E P M ) : Neurologia, U F P R ; ** P r o f e s s o r T i t u l a r N e u r o l o g i a , E P M . Especialidade Neurologia, sidade Federal do Paraná
Departamento de Clínica — Rua General Carneiro
Médica, Hospital de 191 — 80000 Curitiba
Federal do Paraná * Professor Adjunto
Clínicas PR —
da UniverBrasil.
Since the d e m o n s t r a t i o n of the vaiue o f e l e c t r o m y o g r a p h y ( E M G ) to differentiate the p r i m a r y muscular d i s o r d e r s from the diseases o f n e u r o g e n i c o r i g i n b y Buchthal and Clemmensen *2, several a u t h o r s 10,11-32,34,37 helped to c o n s o l i d a t e the E M G s h o w i n g the value in the d i a g n o s i s o f m y o p a t h i e s and d i s o r d e r s resulting from lesions in the l o w e r m o t o r n e u r o n s and nerves without, h o w e v e r , a l l o w i n g a n o s o l o g i c a l d i a g n o s i s n 34. T h e first o p e n muscle b i o p s y ( M B ) c a r r i e d out b y G r i e s i n g e r and Billroth 33, induced D u c h e n n e 22,23 to study the muscle o f patients to firm an e x a c t d i a g n o s i s and p r o g n o s i s o f muscular d i s e a s e s . Erb defined the essential alterations in d y s t r o p h i c muscles, that w e r e e x t e n d e d and used untill n o w i > i 8 . 2 i . W i t h the introduction o f histochemistry in the analysis o f M B , the h i s t o l o g i c a l d i a g n o s i s o f n e u r o m u s c u l a r disorders was e x p a n d e d and i m p r o v e d 7,8,21,28,58. Several studies in the p a s t w e r e d o n e , trying to c o n f r o n t the results o f M B with E M G 4,9,13,35,37,43,53,58, s o m e t i m e s r e a c h i n g conflicting results, f a v o u r i n g at times the E M G 13,35,53 r in other o c c a s i o n s the M B 4 3 due to different techniques o f investigation ( d i s e a s e s with simultaneous involvement of m u s c l e s and nerves 3 7 lack o f specific date a b o u t M B 3 7 lack o f r e p o r t in w h i c h c a s e s the c o n c o r d a n c e h a p p e n e d b e t w e e n b o t h p r o c e d u r e s 9.37; a b s e n c e o f histochemical techni ques >35,37,53,56; h e t e r o g e n o u s and rare g r o u p o f d i s e a s e s in the analysis 9.13,37; study of several m u s c l e s b y the E M G and o n l y o n e muscle in the M B 4,13,35,37,53,56; several E M G in different times and o n l y o n e M B 13,35,53,56; small n u m b e r o f p a t i e n t s ; M B in the same p l a c e o f p r e v i o u s E M G 9.53; study o n l y o f m y o p a t h i e s 43 r o n l y dener vation d i s o r d e r s 5 6 ) . 2 9
0
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T o c o r r e l a t e the M B p r o c e s s e d b y fresh-frozen section stains and histochemistry with E M G , in relation to the p a t h o g e n i c d i a g n o s i s and verify it is p o s s i b l e to a v o i d extensive and painful E M G studies w h e n o n l y o n e m u s c l e w a s studied in b o t h p r o c e dures, this p r e s e n t s t u d y w a s undertaken using specific pre-stablished criteria to a v o i d the a b o v e difficulties. MATERIAL A N DMETHODS W e selected patients with the diagnosis of Duchenne muscular dystrophy ( D M D ) , l i m b -girdle muscular dystrophy (LGMD), facio-scapulo-humeral dystrophy (FSHD), myotonic d y s t r o p h y ( M D ) , d e r m a t o - p o l y m i o s i t i s ( D P ) w i t h o u t neoplasia o r associated to connective tissue disorders, a m y o t r o p h i c lateral sclerosis (AL.S), infantile spinal muscular a t r o p h y ( I S M A ) and peripheral neuropathy ( P N ) w h o h a d typical clinical and l a b o r a t o r y findings, as well as electromyographical and histological-histochemical data available t o meet the criteria des cribed below. T h e c o n f r o n t a t i o n o f 600 M B w i t h 1500 E M G w e r e c a r r i e d o u t f r o m 1975 t o 1983, l o o k i n g f o r c a s e s w h i c h h a d b o t h p r o c e d u r e s , a n d w h i c h m e t the f o l l o w i n g c r i t e r i a : 1) s y m m e t r i c a l p a t h o l o g y , w i t h c l i n i c a l i n v o l v e m e n t o n b o t h s i d e s o f t h e b o d y ; 2) E M G and M B should have been o n an identical muscle but o n the opposite side; 3) cases w h e r e an E M G had been done on both sides previously, w o u l d n o t b e included, t o avoid the « n e e d l e m y o p a t h y * (5,6,16,2h,k"1,52); 4) E M G and M B should have been done at the same period (within 3 w e e k s ) , based on studies w h i c h evaluate the regeneration and a l t e r a t i o n s i n v o l u n t e e r s after a n E M G ( 6 2 ) ; 5) t h e E M G s h o u l d h a v e b e e n q u a n t i t a t i v e , having registered the insertion activity, spontaneous activity during muscular relaxation, average duration of potentials, register of long and short polyphasic potentials, recruitment of efforts and pattern o f severe efforts, a c c o r d i n g t o criteria which will b e defined; 6) t h e M B s h o u l d h a v e b e e n a d e q u a t e , in q u a l i t y a n d n u m b e r o f m u s c l e f i b e r s , c o n t a i n i n g i n f o r m a t i o n o n t h e p r o l i f e r a t i o n o f c o n n e c t i v e tissue, a d i p o s e t i s s u e i n f i l t r a t i o n , necrose, p h a g o c y t o s i s , d i f f u s e i n f l a m m a t o r y infiltrate, p e r i v a s c u l a r i n f l a m m a t o r y i n f i l t r a t i o n , internal nucleus, splitting fibers, whorls, moth-eaten, snake coils, ring fibers positive fibers and increased of the acid phosphatase in t h e i n t e r s t i c e , p o s i t i v e f i b e r s a n d i n c r e a s e o f t h e a l k a l i n e p h o s p h a t a s e in t h e i n t e r s t i c e , t y p e I a n d I I f i b e r a t r o p h y , t y p e I a n d I I f i b e r hypertrophy, predominance o f type I and I I fibers, type I and I I fiber deficiency, dark a n g u l a r a t r o p h i c f i b e r s in t h e N A D H - t e t r a z o l i u m r e d u c t a s e , d a r k a n g u l a r f i b e r s in t h e n o n s p e c i f i c esterase, targets, g r o u p i n g o f type fibers, fascicles atrophy and perifascicular atrophy, such as the criteria d e f i n e d in o t h e r s t u d i e s (1,1,8,18,21,28,5S); 7) the muscle studied should b e degree 4 ( M R C M ) 4 2 ) , t o avoid the selection of very severe affected cases. E l e c t r o m y o g r a p h y —• T h e E M G w e r e d o n e w i t h a M E D I C e q u i p m e n t , w i t h f r e q u e n c y response between 15Hz and lOKHz, u s i n g normal concentrical needle electrodes and the g r o u n d e l e c t r o d e w a s p u t at t h e r o o t o f t h e e x a m i n e d l i m b . R o o m t e m p e r a t u r e w a s b e t w e e n 20-30°C, w i t h p a t i e n t s e x t r e m i t i e s k e p t w a r m . T h e exam procedure w a s normal, according to t e c h n i q u e s d e s c r i b e d b y s e v e r a l a u t h o r s (10,30,32,38,89). D u r i n g t h e p r o c e d u r e , e a c h one o f
the p a r a m e t e r s b e l o w w a s e v a l u a t e d , a c c o r d i n g t o the c r i t e r i a d e s c r i b e d . 1) I n s e r t i o n a c t i v i t y : a b n o r m a l if the d u r a t i o n w a s a b o v e 3C0ms (32,38). 2) F i b r i l l a t i o n : duration from l-5ms, a m p l i t u d e v a r y i n g f r o m 20-300 u V , u s u a l l y b i p h a s l c s , d e t e c t e d o u t s i d e the a r e a o f the m o t o r e n d - p l a t e , w i t h the first c o m p o n e n t b e i n g p o s i t i v e (14,20,32,39). 3) F a s c i c u l l a t i o n : registered during muscular relaxation, without a fixed frequency, amplitude, duration or m o r p h o l o g y (32,39). 4) P o s i t i v e w a v e s : q u i c k p o s i t i v e p o t e n t i a l , f o l l o w e d b y a l o n g n e g a t i v e p h a s e , w i t h a v o l t a g e v a r y i n g b e t w e e n 50uV to l m V , w i t h a d u r a t i o n o f 20-2CGms (14,32,39). 5) H i g h frequency discharge: identical frequency for each muscular fiber group, synchronous, with an a m p l i t u d e o f 50uV t o l m V a n d an a v e r a g e d u r a t i o n o f 150ms (38). 6) M y o t o n i a : c o n t i n u o u s a n d r y t h m i c a l d i s c h a r g e s , w h i c h i n c r e a s e a n d d i m i n i s h the v o l t a g e , f o r l o n g p e r i o d s after h a v i n g s t o p p e d the initial s t i m u l u s , b e i n g a b l e t o a s s u m e the s h a p e o f p o s i t i v e w a v e s o r f i b r i l a t l o n s (JX). 7) A v e r a g e d u r a t i o n o f a c t i o n p o t e n t i a l s : a p p r o x i m a t e l y 20 d i f f e r e n t p o tentials were analysed, obtained during slight contraction and a mean voltage was o b t a i n e d ; the r e s u l t w a s c o m p a r e d t o the. v a l u e s p r o p o s e d b y B u c h t h a l (10), b e i n g c o n s i d e r e d i n c r e a s e d if the d e v i a t i o n w a s o v e r 2 0 % a n d d i m i n i s h e d if it w a s u n d e r 20%. S) A v e r a g e v o l t a g e o f v o l u n t a r y p o t e n t i a l s : i d e n t i c a l a n a l y s i s to t h e p r e v i o u s p o t e n t i a l s w a s m a d e , b u t t h e o n l y p o t e n t i a l s c o n s i d e r e d w e r e t h o s e w h i c h the p o s i t i v e d e f l e x i o n p e a k t o h i g h e s t n e g a t i v e w a s n o t a b o v e 2C3us; t h e r e a r e n o a g r e e m e n t in t h e l i t e r a t u r e a b o u t the n o r m a l v a l u e s — 3GCuV t o 5 u V (32), 1C0..V t o 2 m V (39), h i g h e r t h a n 2 m V a n d l o w e r than 4 m V (13), 100 t o 2000uV (53), i m p o r t a n t w h e n it e x c e e d s 60C0uV(S5), s o m e h u n d r e d s o f u V t o u n d e r 3 m V (56) —• w e u s e the v o l t a g e s f o u n d in n o r m a l i n d i v i d u a l s f r o m o u r l a b o r a t o r y , w h i c h v a r y f r o m 500 to 3000uV ( u n p u b l i s h e d d a t a ) ; t h e y w e r e c o n s i d e r e d i n c r e a s e d if a b o v e SOOOuV a n d d i m i n i s h e d if u n d e r 5C0uV. 9) P o l y p h a s i c p o t e n t i a l s : v o l u n t a r y p o t e n t i a l s which present m o r e t h a n 4 p h a s e s a n d are c o n s i d e r e d a b n o r m a l if e x c e e d 1 2 % o f p o t e n t i a l s exami n e d (10,15,32), b u t w e c o n s i d e r a b n o r m a l in o u r l a b o r a t o r y , o n l y if t h e y e x c e e d 2 5 % ; if the total d u r a t i o n w a s the s a m e o r l o w e r than t h e a v e r a g e d u r a t i o n f o r the a g e , t h e y w e r e c a l l e d s h o r t : if t h e d u r a t i o n w a s h i g h e r t h a n the a v e r a g e f o r the a g e , t h e y w e r e c a l l e d l o n g ( 3 2 , 3 ? ) . 10) E f f o r t r e c r u i t m e n t : w h e n t h e i n d i v i d u a l i z a t i o n o f s e v e r a l m o t o r u n i t s in the i n t e r f e r e n c e p a t t e r n w i t h r e d u c e d d u r a t i o n d u r i n g w e a k c o n t r a c t i o n w a s p o s s i b l e , it w a s c o n s i d e r e d i n c r e a s e d ; if the t o t a l n u m b e r o f m o t o r u n i t s w a s d i m i n i s h e d , a l l o w i n g v e r i f i c a t i o n of a r e a s without potentials, it w a s c o n s i d e r e d r e d u c e d (39). 11) E f f o r t p a t t e r n : d u r i n g a w e a k , m e d i u m a n d s t r o n g c o n t r a c t i o n , the e f f o r t p a t t e r n w a s a n a l y s e d , b e i n g c l a s s i f i e d as B S A P if the p o t e n t i a l s w e r e o f r e d u c e d d u r a t i o n , l o w voltage, and plentiful o n e f f o r t (19,25), PMUP if t h e m o t o r u n i t p r e s e n t e d p o l y p h a s i c p o t e n t i a l s , w h i c h w e r e i n d i v i d u a l i z e d o n the i n t e r f e r e n c e p a t t e r n , S M U P if the p o t e n t i a l s p r e s e n t e d r e d u c e d d u r a t i o n a n d n o r m a l v o l t a g e , G M U P if t h e r e w e r e p o t e n t i a l s o f h i g h v o l t a g e , u s u a l l y o v e r 5 m V , M U P if t h e y p r e s e n t e d i n d i v i d u a l i z e d m o t o r u n i t s o f n o r m a l d u r a t i o n a n d v o l t a g e a n d M I X E D if b y t e m p o r o - s p a c i a l s o m a t i o n o f the d i s c h a r g e s it w a s i m p o s s i b l e t o d i s t i n g u i s h a n y i n d i v i d u a l i z e d p o t e n t i a l s , as it o c c u r s in the n o r m a l m u s c l e . 12) E l e c t r o m y o g r a p h i c d i a g n o s i s : d e p e n d e d o n t h e a d d i t i o n or absence of some kinds of potentials found, b e i n g divided into specific and non specific, for cases of primary muscular involvement (myopathies) or for denervation (secondary m u s c l e a t r o p h y o r n e u r o g e n i c ) (•?•»); w e r e n e c e s s a r y t o m a k e a d i a g n o s i s , t o h a v e 3 t y p e s o f s p e c i f i c c r i t e r i a in the s a m e c a t e g o r y ; the n o n s p e c i f i c c r i t e r i a w e r e n o t c o n s i d e r e d , unless t h e y a p p e a r s w i t h h i g h i n t e n s i t y a n d f r e q u e n c y ; w h e n an e x a m h a d at s a m e t i m e p o t e n t i a l s b e l o n g i n g t o m y o p a t h i e s o r d e n e r v a t i o n c a t e g o r i e s , it w a s p l a c e d in the c a t e g o r y of m i x e d involvement ( T a b l e 1 ) .
M u s c l e b i o p s y — A f t e r c h o o s i n g the m u s c l e w h i c h w i l l b e e x a m i n e d , u s u a l l y d e g r e e 4 ( M R C M ) ( 4 2 ) a n d w h i c h a l s o h a d b e e n t e s t e d o n E M G b u t o n the o p p o s i t e s i d e , a biopsy with stains for haematoxilin-eosin, modified G o m o r i trichrome b y Engel & Cunnin gham, P A S , oil red O and cresyl violet was done. T h e histochemical reactions w e r e also p r o c e s s e d f o r the a l k a l i n e A T P a s e p H 9.4, a c i d A T P a s e s p H 4.3 a n d 4.6, N A D H - t e t r a z o l i u m reductase, non specific esterase, succinic d e h y d r o g e n a s e , myophosphorilase, acid and alkaline p h o s p h a t a s e , a c c o r d i n g t o t e c h n i q u e s u s e d in o u r l a b o r a t o r y (58). T o s y s t e m a t i z e the d i a g n o sis, the m a i n h i s t o l o g i c a l a l t e r a t i o n s w e r e g r o u p e d a c c o r d i n g t o p r e v i o u s r e p o r t s in the l i t e r a t u r e (21,58) a n d the e x a m i n e r ( L . C W ) d i d n o t k n o w in t h i s t i m e the c l i n i c a l d i a g n o s i s . T h e s e h i s t o l o g i c a l a l t e r a t i o n s w e r e t h e n c a t a l o g u e d u n d e r the f o l l o w i n g i t e m s . 1) M y o p a t h i e s : if the M B had p r o l i f e r a t i o n o f the c o n n e c t i v e tissue, a d i p o s e t i s s u e infiltration, internal nucleus, necrose, p h a g o c y t o s i s , excessive diffuse or perivascular inflammatory infiltration, b a s o p h i l i c f i b e r s , i n c r e a s e d a c i d p h o s p h a t a s e in t h e f i b e r s a n d in t h e i n t e r s t i c e , increase o f a l k a l i n e p h o s p h a t a s e in f i b e r s a n d i n t e r s t ' c e , f i b e r s p l i t t i n g , w h o r l s , m o t h - e a t e n , snake coils, ring fibers, predominance of type I fiber atrophy and predominance of type II fibers hypertrophy. 2) D e n e r v a t i o n : if the M B had v e r y rare fibers w i t h necrose and p h a g o c y t o s i s ,
Specific
Primary
muscle
criteria
Non
involvement
Diminished
of
potentials
Increased
voltage
Positive
polyphasic
potentials
High
Myotonia muscle
atrophy
(Denervation
duration
Increased
average
Reduced
of
activity
or
or
Increased
voltage
pattern
insertion
activity
Fibrillation
recruitment
Positive
Fascicullation Excess of long polyphasic
discharges
SMUP
neurogenic)
potentials
potentials
waves
frequency
BSAP
Increased
insertion
Fibrillation
recruitment
Excess of short
Secondary
criteria
(Myopathies) duration
Reduced average Increased
specific
potentials
waves
High
frequency
discharges
MTJP
or P M U P
pattern
GMTJP p a t t e r n Mixed
involvement
(Myopathic
Combinations
Table
and of the
1 — Electromyographic
denervation
components)
specific criteria of the
diagnosis
first
and
secnd
group.
criteria.
rare internal nucleus, frequent d a r k atrophic a n g u l a r fibers in the non specific esterase a n d N A D H - t e t r a z o l i u m r e d u c t a s e , t a r g e t s , f i b e r t y p e g r o u p i n g s , p r e s e n c e o f t y p e I a n d IJ f i b e r h y p e r t r o p h y a n d p r e d o m i n a n c e o f t y p e I I f i b e r a t r o p h y , a t r o p h y o f l a r g e g r o u p s of f i b e r s o r i n v o l v i n g t h e w h o l e f a s c i c l e . 3) M i x e d : w h e n t h e M B h a d e l e m e n t s f o u n d in m y o p a t h i e s a n d d e n e r v a t i o n as d e s c r i b e d a b o v e . 4) A c e s s o r i e s d i a g n o s i s : w h e n o n l y t y p e I o r II fiber atrophy as the only a b n o r m a l i t y f o u n d . RESULTS F u l f i l l i n g t h e a d o p t e d c r i t e r i a w e s t u d y 100 c a s e s , a c c o r d i n g 98 E M G a n d 93 M B a b n o r m a l i n t h e 100 c a s e s ( T a b l e 3 ) .
Number of cases
Neuromuscular
25
Pseudo-hypertrophic
14
Limb-girdle muscular
9
Facio-scapulo-humeral
10
Myotonic
10
Dermato
10 9 13
muscular
dystrophy
dystrophy dystrophy
dystrophy and
Amyotrophic Infantile
disorders
polymyositis lateral
spinal
sclerosis
muscular
Peripheral polyneuritis *
atrophy
Age (years) mean
to
table
There
were
Muscle studied
Sex M
2.
F
Quadr
Bic
9.1
25
0
24
1
21.5
8
6
10
3
28.6
5
4
3
6
34.2
8
2
3
7
28.2
6
4
5
4 1
49.7
8
2
9
4.6
4
5
9
34.9
12
1
11
Gastr
Delt
1
1
1
Table 2 •— Muscle studied, sex and age in 100 cases of neuromuscular disorders. * Etiology •of peripheral polyneuritis: chronic insecticide intoxication, 9 cases; Dejerine-Sottas disease, 2 cases; intermittent acute porphyria 1 case; chronic recurrent polyneuritis, 1 case. Sex: M, male; F, female. Muscle: Quadr, quadriceps; Bic, biceps; Oastr, gastrocnemius; Delt, deltoid.
Number
of cases —
Electromyographies
F3HD
D&P
MD
ALS
ISMA
PP
100
25
14
9
10
10
10
9
13
52 38 8 2
25
13
6 2
4
3 7
10
1 8
56 32 3 2 7
25
10
9
80 80%
25 100%
11 2
6 1
1
biopsies:
Myopathic Denervation Mixed T y p e II fiber Normal Accordance EMG
LGMD
:
Myopathic Denervation Mixed Normal Muscle
PHMD
and
x
atrophy
14
7
8 1
1
1
1
2 6 1
7 2 4
1
between MB
2
Table 3 — pathology: dystrophy myoton'.c atrophy;
13 98.85%
5 55.55%
5 50%
5 50%
10 100%
8 88.88%
9 69.23%
96.41 ( P < 0 . 0 1 )
Results of electromyographies PHMD, pseudo-hypertrophic j FSHD, facio-scapulo-humeral dystrophy; ALS, amyotrophic PP, peripheral polyneuritis.
and muscle biopsies grouped according muscular dystrophy; LGMD, limb-girdle dystrophy ; D&P, dermato ar.d polymyositis; lateral sclerosis; ISMA, infantile spinal
to the muscular MD, muscle
I n c o m p a t i b l e E M G w i t h d e f i n i t i v e d i a g n o s i s — I n t h e g r o u p o f 58 m y o p a t h i e s ( D M D , L G M D , F S H D a n d D P ) i n c o m p a t i b l e r e s u l t s w e r e f o u n d in t h e E M G o f 2 F S H D ( E M G o f denervation) and 2 other cases with normal E M G , one b e i n g of L G M D and another of F S H D , with a total of 4 cases (6.89%). I n t h e g r o u p o f 38 d e n e r v a t i o n c a s e s ( A L S , I S M A and P N ) , o n e case o f I S M A h a d E M G s u g g e s t i v e o f m y o p a t h y ( 3 . 1 2 % ) . T h e m y o t o n i c d y s t r o p h i e s w e r e s e p a r a t e d in t h i s c l a s s i f i c a t i o n , as it w i l l b e e x p l a i n e d later. I n o u r o v e r a l l r e s u l t s , t h e r e w e r e 5 d i s a g r e e i n g c a s e s o f E M G w i t h the p a t h o g e n i c d i a g n o s i s ( T a b l e 4 ) . Electromyographic Number of c a s e s
Type of pathology
Myop.
Denerv.
Mix.
diagnosis Norm.
Incomp.
1 1
1 3
2
4 (6.89%)
Myopathies: 25 14 9 10
Pseudo-hypertrophic muse, dystr. L i m b - g i r d l e muse, d y s t r o p h y Facio-scapulo-humeral dystr. Dermato and p o l y m y o s i t i s
58 Denervation 10 9 13
10
A m y o t r o p h i c lateral sclerosis Infantile spinal muscle atrophy Peripheral polyneuritis
Myotonic
2 6
48
2
6
1
10 8 11
2
1
29
2
3
7
52
38
:
32
100
25 13 6 4
dystrophy
Total
Table 4 — Results of Denerv, denervation;
electromyographies. Mix, mixed; Norm,
Electromyographic normal; Incomp,
1
8
1 (3.12%)
2
diagnosis: Myop, incompatible.
5 ( 5.0%) myopathic;
Incompatible t w o had
pathogenic MB
results,
proposition
(PN cases).
were
normal
Some as
M B with
normal
In
incompatible,
typical
for
no in
between The
EMG.
chi-square
cases mixed
the
group
of
one was
the
group
of D P ,
and
38
alterations,
normal.
BMG
or
muscle
MB
and
In
our
between
in
results.
involvement
o f 58 p r i m a r y none
denervation
of incompatibility
E M G and
agreement
way,
The
MD
disorders
dystrophy
(100%
presented
test gave a
four
had
a
basic
pathology
cannot
be
presented
denervation,
the
normal
overall results, seven
cases
and
MB.
catalogued
then
most
which
value
only o f 96.41
the
LGMD, in
ALS,
50%
of
(p
