(FRIEDREICH’S ATAXIA)
This is a rare hereditary disease, due to sclerosis of the columns of
Goll and Burdach and the pyramidal tracts. There are ataxia, muscular
weakness, nystagmus, speech disorders and loss of knee flex. Almost
invariably there will be found a neuropathic history. Alcoholism,
syphilis and insanity in the parents are predisposing causes.
Tuberculosis may be a factor. Acute diseases, especially infectious
fevers, dentition and injuries to the spine may be exciting causes.
It occurs most frequently in males about the seventh or eighth year
and very seldom after puberty. Several members of the same family are
apt to be affected. The disorder is transmitted by the female. “The
degeneration of the posterior and pyramidal columns seems to occur at
the time of cord development, when malnutrition or hereditary dyscrasia
would disturb it most.”
=Pathologically=, “the spinal cord is smaller throughout than normal;
we have also a combined disease of the posterior and lateral tracts
(Schultze), a degeneration of Goll’s tract in toto, of Burdach’s almost
entirely, and of the direct cerebellar, the crossed pyramidal (?), and
of Clarke’s columns, in which we find not only atrophy of fibers, but
also a degeneration of the ganglion cells. Gower’s tract may likewise
be involved.” (Oppenheim).
=Symptoms.=—Impaired coordination, beginning in the legs and later
extending to the arms, is the first marked symptom. The gait is
peculiar; it is swaying and irregular and it lacks the pronounced
stamping gait of locomotor ataxia. There is a loss of reflexes, while
no sensory symptoms are present as a rule. The sphincters are normal.
Nystagmus is present and is a characteristic symptom. The speech is
scanning. Talipes and lateral curvature of the spine are common. The
mind becomes sluggish in later stages. The course is always very slow.
=Diagnosis.=—This is not difficult as a rule, owing to the usual
family history presented. The spinal curvature, nystagmus,
incoordination, scanning speech, irregular gait, and deformity of the
feet are symptomatic. In =locomotor ataxia= the gait, sharp pains,
anesthesia and Argyll Robertson pupil will differentiate between the
two. Differentiation will also have to be made from chorea, ataxic
paraplegia and multiple sclerosis.
=Treatment.=—The same treatment as in locomotor ataxia is followed.
Lesions presented have been found at the tenth and eleventh dorsals,
and at the second and third cervicals, although, as a rule, the entire
spinal column is quite debilitated. Some improvement will be noted in
these cases, but not much can be expected from treatment; contractures
may be prevented.