M. De Lima Oliveira 1, J. Luiz Pedroso 2, P. Braga Neto 3, M. Ferreira Machado 4, R. Nogueira 5, O. Graziani Povoas Barsottin 6, E. Bor-Seng-Shu 7
1 Department of Neurolgy, University Hospital of São Paulo,
2 Department of Neurology, Medical Faculty, Federal University of São Paulo,
3 Hospital Israelita Albert Einstein – São Paulo, Brazil
Objective: Several studies have demonstrated increased substantia nigra (SN) in Parkinson's disease (PD), and Machado Joseph disease (MJD). Pathological substrate of PD is characterized by dopaminergic nigrostriatal cell loss, also found in MJD. Also, SN hiperechogenicity might be associated with nigrostriatal disfunction in PD, when comparing dopamine transport binding with SN echogenicity. The present study aimed to correlate the SN echogenicity size and striatal dopamine transporter density in MJD patients.
Material and Methods: We performed TCS in 30 subjects and spect with (99mm TC) - TRODAT-1 in 18 subjects with MJD. Fifteen healthy subjects matched for age and gender formed control group. TCS and (99mm TC) -TRODAT-1 SPECT findings both MJD patients and control group subjects were compared.
Results: There were no differences regarding age (p=0.358) or gender (p=0,566) between groups (MJD versus control group). Mean DAT binding potentials and SN echogenicity were significantly different between groups. There was significant negative correlation with regard to the SN echogenic size and the ipsilateral striatal TRODAT 1 uptake: the higher SN echogenicity, the lower DAT uptake in the isilateral cerebral hemisphere.
Discussion: Increase in SN echogenic size likely correlates with presynaptic dopaminergic nigrostriatal in MJD, suggesting a concurrent in vivo pathophysiological mechanism.
Key words: brain ultrasound Dupplex, Machado Joseph disease, substantia nigra echogenicity, TRODAT.