CMT in Brazil

[Guest guest]

Joana,

Here is a 10 year old study about CMT in Brazil. I will try to get

you the entire article, since you can read Portuguese - then you can

tell me what it says! Anyway, thought perhaps you might want to

contact the authors at the medical center where they are - for more

Brazilian CMT contacts. ~ Gretchen

Arq Neuropsiquiatr. 1995 Sep;53(3-:545-51.

[Charcot-Marie-Tooth disease. Clinical study in 45 patients]

[Article in Portuguese]

de Freitas MR, Nascimento OJ, de Freitas GR.

Servico de Neurologia, Faculdade de Medicina, Universidade Federal

Fluminense, Niteroi RJ, Brasil.

Charcot-Marie-Tooth (CMT) disease is the commonest inherited

peripheral neuropathy. The clinical study of 45 patients with CMT is

presented. They were derived from Pedro Hospital of

Universidade Federal Fluminense in Niteroi, RJ, Brazil. Such patients

could be divided by the motor conduction velocity in two types: a

demyelinating form or type I (11 cases) and an axonal form or type II

(34 cases). The disease was inherited as an autosomal dominant trait

in 23 patients and as an autosomal recessive trait in 7 cases. In 15

patients the disorder was sporadic. The age of onset was in most of

our cases before the 20 years. All of them had distal weakness in

lower limbs. 38.2% had also distal weakness in upper limbs. 80% had

distal wasting of the lower limbs and 50% had distal wasting of upper

limbs. The tendon reflexes were absent in 64% in lower limbs and in

28% in upper limbs. The sensitive impairment in the distal regions of

the extremities was mild in most patients. We found enlargement of

peripheral nerves in 7 patients of type I. Pes cavus was present in

21 cases and scoliosis in 7. We found postural tremor of hands in 6

patients. In 9 cases there were rare features as mental retardation,

trigeminal nevralgia, optic atrophy, deafness and calf enlargement.

In most of our cases the clinical course was very slow progressive. A

greater severity was seen in our sporadic cases.