CORTICAL - BASAL GANGLIONIC
DEGENERATION (CBGD)
Philip A. Hanna, MD
Parkinson's Disease and Movement Disorders Center
New Jersey Neuroscience Institute
Edison, New Jersey
What is Cortical-Basal Ganglionic Degeneration?
Cortical-basal ganglionic degeneration (or corticobasal degeneration)
(CBGD) is an atypical parkinsonism (parkinsonism plus) syndrome of
unknown cause. CBGD results in gradual loss of function of the cortex
and basal ganglia, usually predominantly on one side of the brain. The
most striking feature is the gradual loss of use (typically learned
functions) of one hand or leg ("apraxia"). Patients may
experience painful stiffness of limbs and muscle spasms, typically
affecting one side of the body more than the other. Other symptoms
include a coarse tremor or jerking movement (myoclonus) of limbs.
Sensation may be affected on one or both sides of the body. In up to
50% of patients, a limb may be perceive by the patient as being
foreign and/or having a "mind of its own", and make
seemingly purposeful movements which are beyond the patient's control
("alien" limb).
Similar to other atypical parkinsonian syndromes, treatment with
levodopa and related medications is rarely, if ever, helpful. Muscle
spasms and myoclonus may be reduced with tranquilizers, or with
botulinum toxin injections.
Articles For Further Reading:
Hanna PA, Doody RS. Alien Limb Syndrome. In: Goetz CG, Lang AE, Litvan
I, eds. Corticobasal Degeneration: Clinical and Research Aspects.
Advances in Neurology Series. Lippincott-Raven Publishers, 1999. (In
press).
Kumar R, Bergeron C, Pollanen MS, Lang AE. Cortical-basal ganglionic
degeneration. In: Jankovic J, Tolosa E, eds. Parkinson's Disease and
Movement Disorders. Baltimore:Williams and Wilkins, 1998:297-316.
Kompoliti K, Goetz CG, Boeve BF, et al. Clinical presentation and
pharmacological therapy in corticobasal degeneration. Arch Neurol
1998;55:957-961.
Jankovic J. Treatment of parkinsonian syndromes. In: Kurlan R, ed. The
Treatment of Movement Disorders. J.B. Lippincott Company,
Philadelphia, 1995:95-114.
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