A B N O R M A L L Y INCREASED IRON CONCENTRATION
IN B A S A L GANGLIA IN SHY-DRAGER SYNDROME
M R I M A G I N G A N D A U T O N O M I C S T U D Y
JAYME A. MACIEL Jr.* — CRISTIANE M. DA ROCHA ** SANDRA CABELHO *** — MARIO G. PRADAL ****
S U M M A R Y — R e p o r t o f a n e a r l y c a s e o f S h y D r a g e r s y n d r o m e i n a 6 7 y e a r o l d w o m a n p a -tient. A u t o n o m i c f a i l u r e w a s d i a g n o s e d b y f u n c t i o n a l e v a l u a t i o n a s w e l l a s l a b o r a t o r y tests. M R i m a g i n g d i s c l o s e d a p r o m i n e n t p u t a m i n a h y p o d e n s i t y i n T2- w e i g h t e d i m a g e s a t h i g h field s t r e n g t h d u e t o i r o n i n c r e a s e d d e p o s i t i n g i n t h i s b a s a l g a n g l i a . M R i m a g i n g e v i d e n c e s c o n -f i r m S h y - D r a g e r s y n d r o m e d i a g n o s i s , a n d c o n t r i b u t e s -f o r d i -f -f e r e n t i a l d i a g n o s i s o -f i d i o p a t h i c h y p o t e n s i o n ( p u r e a u t o n o m i c f a i l u r e ) i n s p e c i a l i n S D S e a r l y c a s e s .
Concentração de ferro anormalmente aumentada nos núcleos da base na síndrome de Shy-Drager: RM imagem e estudo autonômico.
R E S U M O — É r e l a t a d o o c a s o d e u n m p a c i e n t e d e 6 7 a n o s d e i d a d e c o m q u a d r o i n i c i a l d a s í n d r o m e d e S h y - D r a g e r . O d i a g n ó s t i c o f o i p o s s í v e l p o r p r o v a s f u n c i o n a i s a u t o n ô m i c a s e e x a m e s l a b o r a t o r i a i s . A r e s s o n â n c i a m a g n é t i c a c e r e b r a l ( c o n t r a s t e b a s e a d o n a d e n s i d a d e d e p r ó t o n s e e m T2) o b j e t i v o u p r o e m i n e n t e h i p o d e n s i d a d e p u t a m i n a l e m T2, s e c u n d á r i a a o au-m e n t o d o d e p ó s i t o d o f e r r o n e s t a r e g i ã o . E s s e a c h a d o d a R M c o n f i r au-m a o d i a g n ó s t i c o d a s í n d r o m e d e S h y D r a g e r e p e r m i t e d i f e r e n c i á l a d a h i p o t e n s ã o o r t o s t á t i c a i d i o p á t i c a , p a r t i c u l a r m e n t e n a f a s e d e i n í c i o d a S S D q u a n d o o s s i n a i s d e c o m p r o m e t i m e n t o d o S N C s ã o d i s -c r e t o s o u e s t ã o a u s e n t e s .
Described in 1960 31, the early clinical features in Shy-Drager syndrome ( S D S ) are postural symptoms during many y e a r s2 4. Central nervous system signs
(extra-pyramidal, pyramidal or cerebellar) are observed two to five years after orthostatic hypotension diagnosis 34,36. The Shy-Drager patients do not respond well to antipar-kinsonian drug therapy, and usually succumb to the disease seven to eigth years after its onset and four to five years after C N S disorders outset 35. Postmortem S D S findings include gliosis and/or neuronal loss in the substantia nigra, caudate nucleus, putamen, cerebellar cortex, pontine nuclei, and inferior olives 28. The neuropathologic SDS findings were divided in two groups 2 4: the first group is characterized by a
striatonigral degeneration with Lewy corps 15,29; the second group by a striatonigral degeneration or olivopontocerebellar atrophy without Lewy corps 31 (two cases in the original Shy-Drager publication). The dominant neurochemical finding in autopsy studies is a severe dopamine decrease in the striatum, substantia nigra, and nucleus accumbens4. In vivo studies by evidence of serotonin decrease (autonomic failure) and
dopamine decrease (extrapyramidal signs) metabolism in CSF is established by pro-benecide t e s t2 4. The autonomic evaluation findings in S D S include a sympathetic
D e p a r t a m e n t o d e N e u r o l o g i a , F a c u l d a d e d e C i ê n c i a s M é d i c a s , U n i v e r s i d a d e E s t a d u a l d e C a m p i n a s ( U N I C A M P ) : * P r o f e s s o r A s s i s t e n t e D o u t o r ; * * R e s i d e n t e d e N e u r o l o g i a ; *** E n - ¬ d o c r i n o l o g i s t a , C R I E S P ; **** E n d o c r o n o l o g i s t a C R I E S P , A u x i l i a r d e E n s i n o d e F i s i o l o g i a d a
F a c u l d a d e d e M e d i c i n a d e S a n t o s .
and parasympathetic disorder 24 due to degenerative neuron loss in the intermedio-lateral column of spinal cord responsible for central disorder of cardiovascular reflex.
Classically S D S w a s distinguished from Parkinson autonomic failure 12 and
idiopathic orthostatic h y p o t e n s i o n4
by chronological clinical features, autonomic eva-luation and follow-up of the potients2. M o r e recently 6,10,26,30 high tield MR imaging used for analysing the iron distribution in the brain establishes abnormally increased
iron concentration (decreased T2 relaxation times) in the putamen and lateral pars
compacta of the substantia nigra in Shy-Drager patients. T h e s e MR imaging evi-dences confirm S D S diagnosis and may also potentially serve to differentiate this syndrome from idiopathic orthostatic hypotension (pure autonomic failure) in special in the early course of the disease when other clinical manifestations may be minimal or lacking 6.
CASE REPORT
(1.5 mg and 3 mg intravenous) corroborated the sympathetic branch disorder of cardiovas-cular reflex (Figs. 3 and 4). The serum aldosterone was normal. The plasmatic renin activity was decreased in supine position (0.29^g/ml/h, normal range 1.2+1.1) and increased in or-thostatism (0.48^g/ml/h, normal range 2.6+1.3). Plasma noradrenaline was slightly low at rest (86pg/ml, normal range 100-350) little increasing after two minutes of standing up (92 pg/ml) and falling after five minutes of orthostatism (77 pg/ml). Plasma adrenaline was normal in supine position (21 pg/ml, normal range 20-50), slowly increasing after two mi-nutes of standing up (26 pg/ml) and falling after five mimi-nutes in orthostatic position (15 pg/ml). Plasma dopamine was normal at rest (34 pg/ml, normal range 20-50), increasing after two minutes in orthostatism (49 pg/ml) and falling after five minutes of standing up (27 pg/ml). The routine laboratory tests were normal. MR imaging disclosed a putaminal hypo-intensity in T^-weighted images at high field strenght (Fig. 5). The decrease signal inten-sity of putameh is more prominent than that of the globus pallidus, and it is due to iron increasing distribution in this place.
COMMENTS
Piorry27 in 1826 is the first to describe an orthostatic syncope. Babinski and
L a u b r y l in 1924 are the first to trace the outline of postural hypotension, and Bradbury and Eggleston 4 in 1925 described an orthostatic hypotension with failure of increasing the heart rate in three cases. T h e incomplete autopsy of one of their cases in 1927 is normal, and Bradbury and Eggleston proposed a hypothesis of a peripheral sympathetic abnormality to explain the postural hypotension In 1960 Shy and Drager 31 described a clinical-pathologic study of three cases of orthostatic hypo-tension associated to diffuse lesions in the C N S . T h i s disorder is due to a central type lesion of autonomic system 14,21,24 with sympathetic branch and vagal branch disorders in autonomic functional evaluation 24. S D S is a primary preganglionic disorder 9.31. It is suggested by relatively normal resting noradreline in contrast to the marked reduction in other autonomic disorders (acute panautonomic neuropathy 22,
diabetic autonomic neuropathy 8 ) . T h e poor upright response in S D S3 7
T h e increased iron concentration evidenciated by MR imaging in putamen and in pars compacta of substantia nigra 6,10,26,30 confirms S D S and distinguishes from idiopathic orthostatic hypotension (progressive autonomic failure) particularly in early c a s e s6
. There are consonant findings on brain iron concentration between MR ima-ging !0 and autopsy studies 16. "»
Increased deposition of iron in C N S has been reported in degenerative disorders
which affect the basal ganglia (Hallervorden Spatz disease 13
'3 3
, Huntington disease 20,
Parkinson disease u and d y s t o n i a7
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