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Babinski and the diagnosis of amyotrophic lateral sclerosis.

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LETTERS
Babinsh and the Diagnosis
of Amyotrophic Lateral
Sclerosis
Lewis P. Rowland. M D
We [11 and others use the presence of Babinski signs in
amyotrophic lateral sclerosis (ALS) to differentiate that condition from progressive spinal muscular atrophy (PSMA), in
which there are no upper motor neuron signs. Evans [2] does
not dispute this view but expresses discomfort because the
corticospinal tracts may show histological changes even in
patients who have only lower motor neuron signs in life;
conversely, 2 patients had clinically typical ALS but no visible
postmortem lesions in corticospinal tracts 131. He concludes
that the Babinski sign is an unreliable predictor of lesions in
the pyramidal tracts.
Evans infers more than we implied. No one ever said that
a Babinski sign is invariably associated with visible changes
in the pyramidal tracts. The sign may be present in normal
infants or in adults after anesthesia, head injury, hypoglycemic coma, or other transient states. After fixed corticospinal
damage, the sign may be lacking if the toe extensors are
weaker than the flexors [4].
Postmortem evidence of pyramidal tract degeneration in
adult-onset PSMA {3,5] explains why PSMA is equated with
ALS and why both syndromes are considered forms of motor
neuron disease. This view was expressed by Gordon Holmes
as long ago as 1909 and, with little dissent, has been accepted
ever since.
Combining figures from two autopsy series [3, 51, 61 of
63 patients with ALS with Babinski signs had visible lesions
in the corticospinal tracts, which is an impressive consistency.
The other 2 patients provide evidence that there may be
chronic pyramidal dysfunction without visible changes and
that even histologically defined PSMA is also a form of ALS.
Before Medline, there was a lively body of literature on
the Babinski sign [GI, including the inconsistent histological
relation to pyramidal lesions. The issues were set forth by
such luminaries as Gordon Holmes, Sir Francis Walshe, Peter
Nathans, and William Landau, as clearly discussed by DeJong
and Haerer 161. The Babinski sign is still a central concern
in the diagnosis of ALS.
Neurological Institute
Columbia-Presbyterian Medical Center
New York, N Y 10032-2603
References
1. Rowland LP, Santoro M, Lange DJ, et al. Diagnosis of ALS. Ann
Neurol 1991;30:225-227
2. Evans BK. Diagnosis of amyotrophic lateral sclerosis. Ann Neurol 1992;31:574
3. Brownell B, Oppenheimer DR, Hughes JT. The central nervous
system in motor neurone disease. J Neurol Neurosurg Psychiatry
1970;33:338-357
4. Landau WM, Clare MH. The plantar reflex in man, with special
reference to some conditions where the extensor response is unexpectedly absent. Brain 1959;82:321-355
5. Lawyer T, Netsky MG. Amyotrophic lateral sclerosis. Clinicoanatomic study of 53 cases. Arch Neurol Psychiatry 1953;69:
171-192
6. Haerer AF. D e Jong’s the neurologic examination, ed 5. Philadelphia: Lippincott 1992;460-461
Cy closporine-induced
Chorea after Liver
Transplantation for
Wilson’s Disease
0. Combarros, MD,* E. Fhbrega, MD,I J. M. Polo, MD,*
and J. Berciano, MDX
Cyclosporine-induced neurotoxicity has been reported as occurring in up to 25% of patients undergoing liver transplantation [1, 2). Tremors and seizures are possibly the most commonly encountered complications 111. Other less common
side effects include confusion, cortical blindness, quadriplegia, and coma 12). W e have found no reference to movement
disorder in literature concerning cyclosporine-induced neurotoxicity following liver transplantation.
The diagnosis of Wilson’s disease was established in our
patient at age 19 when tremor of the hands and dysarthria
developed. Brain magnetic resonance imaging (MRI) showed
high-signal intensity lesions in both putamen on T2-weighted
images. With penicillamine therapy, neurological symptoms
disappeared, but portal hypertension and marked splenomegaly developed. Liver transplantation was performed 4 years
after the onset of symptoms, because of bleeding esophageal
varices. The postoperative course was unremarkable. He was
treated with intravenous cyclosporine (140-200 mg twice
daily) and blood levels did not exceed 317 ng/mL, as determined by fluorescence polarization immunoassay (therapeutic range, 150-300 ng/mL). Other medications included
azathioprine, prednisone, acyclovir, and ranitidine; no neuroleptic drugs were given. O n postoperative day 2 1, cyclosporine was given orally (525 mg twice daily), and over the
next week levels rose to 505 ng/mL on day 23, 564 ng/mL
on day 25, and 506 ng/mL on day 29. Serum cholesterol,
magnesium, bilirubin, and creatinine were normal.
O n day 31, the patient was continually grimacing, blinking,
chewing, and showing bizarre movements of the mouth and
tongue. His speech was slurred. Orofacial involuntary movements were accompanied by continuous rotation movements
of the neck and choreic movements of the hands. The
cyclosporine dosage was decreased to 350 mg twice daily.
Blood cyclosporine levels promptly decreased to 362 ng/mL,
and the involuntary movements gradually disappeared over
the next 10 days with no recurrence. Repeated brain MRI
revealed no change in the basal ganglia lesions.
The relationship in our patient between the high blood
cyclosporine levels and chorea and the disappearance of the
latter with a decline in blood levels led us to conclude that
108 Copyright 0 1993 by the American Neurological Association
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