Tremors and CMT

[Guest guest]

(This is from the MDA website " Ask the Experts " section)

SUBJ (06/00): TREMORS WITH CMT/ROUSSY-LEVY

My 7 year-old daughter was recently diagnosed with Roussy-Levy. We knew

that she had Charcot-Marie Tooth disease (CMT), but have never heard of

Roussy-Levy. She did

not have any signs of tremors until she spiked a fever of 105 and the

doctors did a spinal tap. Is it possible that the fever or the spinal

tap caused the symptoms? Is there anything besides beta-blockers that

can control the tremors? Would being exposed

to secondhand cigarette smoke on the weekends make the tremors worse?

My mother, brother and myself all have CMT and none of us have tremors.

I also have other family members with CMT and none of them have tremors.

What causes tremors in some CMT patients and not others?

REPLY [1] from MDA: Pleasure, M.D., Children's Hospital of

Philadelphia, PA

Roussy-Levy syndrome simply means CMT with postural tremor. Several of

the mutations associated with CMT (duplication, PMP22 point mutation)

have led to the Roussy-Levy

phenotype. Whereas in the original family described in the literature,

tremor was a common feature, some families have diversity (some with

tremor, some without). I am not aware of any relationship between tremor

and cigarette smoke (though secondhand smoke is recognized as a risk

factor for cancer, heart disease, etc.).

Various drugs have been tried for the tremor — propanolol, Mysoline,

etc.— but I have no personal experience. Why the tremor only began after

high fever is a mystery to me — the first I've heard of such a

relationship.

REPLY [2] from MDA: Bird, M.D., MDA Clinic Director, University

of Washington Medical Center, Seattle, WA

Why some people with neuropathy develop tremor is not clear. It might be

related to abnormal feedback loops from muscles and joints. Worsening of

CMT with fever is new to me. It might be similar to the worsening of

multiple sclerosis with high temperature, but MS is a very different

disease and this is only speculation. Tremor is not specific for any

particular type of neuropathy or CMT. Of historical note is the

discovery that descendants from the original family of Roussy and Levy

have been found to have CMT1B with a mutation in the myelin P zero gene.