Chapter 3-The Physicians Guide To The Management of HD
Myodonus, Tics and Epilepsy
Myoclonus, sudden brief jerks involving
groups of muscles, is more common in juvenile-onset HD, where it may be mistaken for a seizure. Like chorea, myoclonus may
not be disabling or particularly distressing, but may respond to treatment with clonazepam or divalproex sodium if treatment
Tics are brief, intermittent stereotyped
movements such as blinking, nose twitching, head jerking, or transient abnormal postures. Ties which involve the respiratory
and vocal apparatus may result in sounds including sniffs, snorts, grunts, coughs, and sucking sounds.
Patients may be unaware of vocal tics,
but family members may find the incessant noises grating. They should be helped to understand that the tics are not under
voluntary control. Tics generally do not by themselves require treatment, but may respond to neuroleptics, benzodiazepines,
Epilepsy is uncommon, though not unheard of, in adults with HD, but
is said to be present in 30% of individuals with juvenile-onset HD.
A first seizure in an HD patient should not be attributed to HD without
further evaluation as it may be indicative of an additional neurologic problem, such as a subdural hematoma sustained
in a fall.
The workup of a first seizure should include a complete exam, laboratory
studies to rule out an infection or metabolic disturbance, an EEG, and a brain imaging study. The treatment of a seizure disorder in a person with HD depends on the nature of the seizures.
In the juvenile HD patient, myoclonic epilepsy or other generalized
seizures may suggest divalproex sodium as a first treatment choice.
Although seizure management in HD is not usually difficult, for the
occasional patient seizure control is quite difficult to achieve, requiring multiple medications or specialized referral.
Seizure studies to watch
October 16, 2001
Cleveland Clinics Clinical Trials
CAPS my input/Jean Miller
TOPIRAMATE FOR COGNITION?
Topiramate Study The Cleveland Clinic Foundation is beginning a study investigating the effects of the antiepileptic drug
topiramate on cognition and vigilance using self-administered computer neuropsychological batteries, polysomnography and the
multiple sleep latency test. Patients with epilepsy who have incompletely controlled seizures may qualify for treatment with
the new antiepileptic drug. The study requires five visits to The Cleveland Clinic Foundation.
CARBATROL FOR SLEEP & ALERTNESS
for Patients with Newly Diagnosed/Untreated Epilepsy The Cleveland Clinic Foundation is beginning a study investigating the
effects of the antiepileptic drug Carbatrol (carbamazepine) on nighttime sleep and daytime alertness. Carbamazepine is a commonly
used, FDA-approved medication used in the treatment of partial (focal) seizures. Untreated
patients interested in participating
would undergo sleep evaluation including sleep questionnaires and a baseline overnight sleep study followed by a "daytime
nap" test at no charge. Subjects would be treated with carbamazepine for several weeks and then undergo a second sleep
HARMONAL AFFECT ON SEIZURES?
For Women Only Women
and adolescent girls recently diagnosed with a seizure disorder who are not yet taking anti-epileptic medications may be eligible
to participate in a new study being conducted at the Cleveland Clinic. Hormonal, metabolic and ovarian functions will be evaluated
in females using either lamotrigine or valproic acid.
NEW DRUG HARKOSERIDE
For Refractory Patients Adults who continue to have difficulty controlling
their partial seizures may be eligible to participate in a study to begin soon at the Cleveland Clinic. This five month study
will investigate the efficacy of harkoseride as an add-on treatment in the control of partial seizures with or without generalization.
NOTE: Kelly's seizures were typically only during her menstrual cycle when her choretic movements were
accelerated. Earlier discussions with doctors on the relationship to her mentstrual cycle and chorea were dismissed
as a whacko mother trying to the determine NON HD causes when of course they all knew everything was HD related (era late
1980's). In the early 90's I heard there was a study on female HD patients to determine relationship between menstrual
cycles and chorea. I haven't been able to find the outcome of that study.