Volume 16, Number 3
It's a mistake to shrug your shoulders over patients with severe generalized dystonia, says neurologist "Buz" Jinnah. "Or with any dystonia. We can do something for everyone. It's just a matter of degree." Jinnah offers oral drugs like artane, which directly squelch neurotransmission to muscles, or benzodiazepines, well known as overall muscle relaxants.
Fred Lenz (center) confers on
the best brain approach to
Patients with focal dystonias such as torticollis (head and neck) or blepharospasm (eyelid) respond "wonderfully" to botulinum toxin, he says. And Botox can help generalized patients as well, as can a drug- containing pump surgically implanted beneath the skin. The pump typically bathes the spinal cord in the muscle relaxant baclofen.
For torticollis patients who don't respond to Botox or medication, selective nerve sectioning can be very effective, says neurosurgeon Fred Lenz, M.D., who has done many such operations. And a reversible procedure, deep brain stimulation (DBS), has recently won FDA approval for primary generalized dystonia that's otherwise unresponsive. DBS involves easing a fine stimulating electrode into the brain's globus pallidus. "Stimulating that particular area made sense," Lenz says, "after we saw the relief Parkinson's patients with dystonia experienced following DBS for parkinsonian tremor." Lenz has performed 20 of the surgeries for dystonia. Patients who are helped, he says, have a dramatic turnaround.