WE MOVE



Wednesday, April 27, 2005
Issue 69
VOLUME 1 ISSUE 69

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ARCHIVE
Issue 68
April 21, 2005
Vol. 1 Issue 68

ET and Dementia (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

Essential tremor is associated with an increased risk of dementia in a
community-based longitudinal study
J Benito-Leon, F Bermejo-Pareja, ED Louis
P04.163, A253-254

A door-to-door survey of 4,880 elderly people in central Spain in 1994-1995
revealed 240 ET patients. Follow-up after a mean of 3.2 years indicated that
3.5% of 3,541 non-ET subjects had developed dementia, while 7.4% of 202 ET
patients had (relative risk 2.17, 95% CI 1.27-3.72, p=0.005). The increased
risk remained significant after adjusting for sex, age, and education.
[end]




Smell is Normal in ET (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

Olfaction is normal in essential tremor and can be used to distinguish it
from Parkinson's disease
M Shah, L Findley, N Muhammed, C Hawkes
S27.001, A261

Two forms of smell tests were administered to 59 ET patients, 65 PD
patients, and 74 controls. Tests use were the 40-odorant UPSIT and olfactory
evoked potential in response to hydrogen sulfide. Mean UPSIT scores were 33
for controls, 32 for ET patients, and 18 for PD patients. OEP values were
normal in all but 4 ET patients (which included at least one patient who
didn't receive testing). Forty percent of PD patients had no recordable OEP,
and the remainder had significantly prolonged latency with normal amplitude.
"A normosmic patient with tremor is more likely to have ET than PD," the
authors conclude.
[end]





Pregnancy in Dystonia (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

DYT-1 and dopamine-responsive dystonia and pregnancy
VL Shanker, SB Bressman, D Raymond, K Ram, R Saunders-Pullman
S07.005, A130

Complications of pregnancy were assessed by self-administered questionnaire
in 17 women with DRD (45 pregnancies), 16 women with DYT1 dystonia (39
pregnancies), and 24 unaffected spouses of men with dystonia (66
pregnancies). First-trimester miscarriage rate was highest among DYT1 women
(31%, vs. 16% for DRD and 12% for unaffected spouses, p=0.057). The rate for
caesarian section was lower in women with dystonia than unaffected women. No
women with dystonia experienced premature rupture of membranes.
[end]





Dystonic EMG in the Yips (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

Electrophysiologic evaluation of yips-affected and unaffected golfers:
Evidence for a task-specific dystonia
CH Adler, D Crews, JG Hentz, AS Smith, B Mill, JN Caviness
P06.146, A381

Twenty age- and handicapped-matched golfers, 10 with the yips, 10 without,
were examined with surface EMG at rest, during handwriting, holding a
putter, and while putting. The yips is the inability to complete a golf
stroke, typically during putting or chipping, that occurs in golfers and
worsens with anxiety. No abnormal EMG was observed during any activity
except active putting. Upon putting, 5/10 yips-affected and 0/10 unaffected
golfers had co-contraction of the wrist flexor/extensors 200 milliseconds
prior to contact of ball and putter. "The presence of co-contraction, the
electrophysiologic hallmark of task-specific dystonias such as writer's
cramp and musician's cramp, during putting in 50% of the yips-affected and
none of the yips-unaffected golfers would support the yips possibly being a
focal dystonia," the authors conclude.
[end]




Low CSF Iron in Early-Onset RLS (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

CSF iron status relates to the age of symptom onset for restless legs
syndrome
CJ Early, C Gamaldo, RP Allen
S04.001, A26

Levels of iron were analyzed in 15 patients with early-onset RLS and 15 with
late-onset RLS without iron deficiency and not taking iron medications.
Early-onset patients had slightly worse Johns Hopkins RLS symptoms scores
(2.0 vs. 1.5). Early-onset patients had significantly lower CSF ferritin
(2.1 vs. 2.8 mcg/l, p<0.005) and slightly higher CSF transferrin than
late-onset patients. For all patients, age at symptom onset correlated with
CSF ferritin and CSF transferrin, driven primarily by high correlation among
the subgroup of early-onset patients, with no significant correlation among
the subgroup of late-onset patients. The authors suggest that CNS ferritin
"may provide a measure of the degree of CNS pathology producing RLS, further
supporting a causal relation between compromised CNS iron status and RLS."
[end]




FXTAS Natural History (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

Preliminary study of the natural history of the fragile X associated
tremor/ataxia syndrome (FXTAS)
MA Leehey, D Hall, C Rice, S Jacquemont, L Zhang, J Grigsby, R Hagerman, PJ
Hagerman
P02.053, A93

Medical records were reviewed for 37 FXTAS patients (35 males, 2 females)
with average disease duration of 10 years. Average age of onset was 60
years, with the most common initial symptom either tremor (54%) or gait
ataxia (24%). Median time to first falling was 4 years, initial cognitive
dysfunction 6 years, inability to walk independently 9 years, and inability
to perform most ADLs 9.5 years.
[end]




Low Antibody Formation Rate for BoNT-A (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

Toxin neutralizing antibody formation with botulinum neurotoxin type A (BoNT
A) treatment in neuromuscular disorders
SA Yablon, S Daggett, MF Brin

Serum antibodies to BoNT-A were determined in 880 analyzable samples from
929 patients, enrolled in clinical trials of BoNT-A for cervical dystonia,
spasticity, and headache. Five samples (0.6%) had neutralizing antibodies: 4
from CD patients, treated for up to 24 months with a maximum exposure of
4210 Units, and 1 from a spasticity patient, who had no initial clinical
response to treatment.
[end]




Tetrabenazine for Pediatric Hyperkinetic Disorders (AAN 2005)
E-MOVE reports from the 57th Annual Meeting of the American Academy of
Neurology, 9-16 April 2005, Miami. Poster, session and page numbers are from
Neurology 2005;64(suppl 1).

Treatment of pediatric hyperkinetic movement disorders with tetrabenazine
(TBZ)
S Jain, PE Greene, SJ Frucht
S57.003, A419

Tetrabenazine is effective in a wide variety of pediatric movement
disorders, according to this study. Retrospective chart review was performed
for 30 pediatric patients receiving TBZ for chorea (17), tics (10), and
dystonia (3). Diagnoses included Tourette syndrome, cerebral palsy,
Sydenham's chorea, post-hypoxic injury, Leigh's disease, and others.
Twenty-eight had tried and failed other medications. TBZ significantly
reduced excess movements in 22 patients. Sedation, behavioral changes, and
depression were significant side effects, and caused discontinuation in 9
patients. Parkinsonism occurred in 1 patient, and tardive dyskinesia in
none.
[end]

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