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Long latency between the onset of motor and vocal tics in Tourette's syndrome.

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A 6-year-old boy had single seizures unassociated with
fever at 4 months, 5 months, and 6 months of age. O n each
occasion, the parents had given the boy whole milk, and the
seizure reportedly occurred after he had drunk a single bottle
of whole milk. After they had reverted to formula, the seizures ceased until the patient was brought to the hospital at 8
months of age in status epilepticus (on this occasion, he had
not been given whole milk). The status epilepticus was unresponsive to large doses of phenytoin, phenobarbital, and
paraldehyde. He was given 50 mg of intravenous pyridoxine,
and the seizures stopped within minutes. He remained seizure-free for the next six days, when seizure activity recurred
and again promptly stopped with intravenous administration
of pyridoxine.
Results of extensive laboratory testing were normal. Over
the ensuing six months the dosages of anticonvulsants were
tapered and discontinued. The patient remained seizure-free
on pyridoxine, 25 mg a day taken orally.
When the patient was 2 % years of age, pyridoxine was
tapered and discontinued. Within a few days of discontinuing
the pyridoxine, he began having occasional myoclonic jerks,
particularly during sleep. Two days later, he again developed
status epilepticus, which was promptly stopped by intravenous pyridoxine.
At age 6, he remains seizure-free on a daily dose of 25 mg
of pyridoxine. His intellectual development is entirely normal. Interictal electroencephalograms have been normal. Ictal electroencephalograms were abnormal, with nonfocal seizure discharges.
Division of Pediatric Neuro/ogy
Columbus Children’s Hospital
Columbus, OH 43205
Refereace
1, Krishnamoorthy KS: Pyridoxine-dependency seizure: reporr of a
rare presentation. Ann Neurol 13:103-104, 1983
Long Latency Between the
Onset of Motor and Vocal
Tics in Tourette’s Syndrome
Anthony E. Lang, MD, FRCP(C),*
Harvey Moldofsky, MD, FRCP(C),t
and Awad G. Awad, MD, PhD, FRCP(C)+
Most authorities regard the occurrence of involuntary vocal
utterances as necessary for the diagnosis of Tourette’s syndrome. Most patients develop vocal tics during childhood or
shortly after the onset of the disorder, usually within one to
three years [GI.
A 74-year-old woman had a history of simple motor tics
involving her face, neck, and shoulders dating back to before
the age of 20. The tics were of variable intensity, increasing
with emotion and decreasing with relaxation. She had noted
no spontaneous waxing and waning of symptoms and no no-
table variability in distribution. She had not experienced any
vocal or complex motor tics. The tics had interfered with
daily living only minimally, until at the age of 72 she began
shouting the names of friends and her husband involuntarily
several times an hour, producing considerable marital discord. The frequency of the shouting fluctuated with changes
in emotional state but rarely occurred during conversations.
She said that she had first developed rare, involuntary coprolalia and echolalia at age 74, but these tics were not witnessed during her hospitalization. Over the year prior to admission she had suffered from a depressive disorder treated
with arnitriptyline, which had no influence on the tics and was
discontinued one month before admission. Her history was
otherwise unremarkable, except for hypertension controlled
with propranolol. There was no history suggestive of a tic
disorder in blood relatives.
Examination revealed an elderly, mildly depressed woman
with frequent tics involving the left sternomastoid and pectoralis muscles. There was less frequent shoulder abduction,
lip pursing, and grimacing of the left side of the mouth. She
had no vocalizations during the initial interview, but subsequently she would frequently shout out the names of friends,
her husband, and later the ward staff. The remainder of the
neurological examination was normal. Pimozide [ 5 } , 3 mg
daily, controlled both her vocal utterances and her motor tics.
This long delay between the onset of simple motor tics and
complex vocalizations is most unusual. Shapiro and coworkers state that vocal tics usually appear within the first three
years of the onset of the disorder, but coprolalia may be
delayed up to 24 years [6]. Until the age of 72 this patient
would have been classified as having a chronic simple motor
tic disorder [l). This late occurrence of vocalizations is evidence for the need to consider tic syndromes on a continuum
{ 3 ] rather than as a series of separate entities.
Ziegler reported another woman in her seventies with
Tourette’s syndrome and essential tremor and emphasized
the rare documentation of this lifelong disorder in the elderly
171. Interestingly, the first well-described patient, reported by
Itard in 1825 [ 4 ] and included in Tourette’s original paper
[2), was 85 years of age; this remains the oldest documented
case to date. These and all other elderly Tourette patients
reported have had the usual short latency berween the onset
of motor and vocal tics.
*Division of Neurology and ?Department of Psychialty
Toronto Western Hospital
Toronto, Ont, Canada
References
1. Diagnostic and Statistical Manual of Mental Disorders. Third edition. Washington, DC, American Psychiatric Association, 1980
2. Gilles de la Tourette G: Etude sur une affection nerveuse caracterisCe par de l’incoordination motrice accompagnge d’echoialie et
de coproldie. Arch Neurologie 919-42, 158-2OO, 1885
3. Golden GS: Tics and Tourette’s: a continuum of symptoms. Ann
Neurol 4:145-148, 1978
4. Itard JMG: Memoire sur quelques fonctions involontaires des
appareils de la locomotion de prehension et de la voix. Arch Gen
Med 8:385-407, 1825
5. Ross M, Moldofsky H: A comparison of pimozide and
haloperidol in the treatment of Gilles de la Tourerte’s syndrome.
Am J Psychiarry 135:585-587, 1978
Notes and Letters
693
6. Shapiro AK, Shapiro ES, Bruun RD, Sweet RD: Gilles de la
Tourette Syndrome. New York, Raven Press, 1978
7. Ziegler DK. Tourette's syndrome and essential tremor in a septuagenarian. Arch Neurol 39:132, 1982
Apparent Motor Neuron
Disease Following the Use
of Pneumatic Tools
John P. Gallagher, MD," and Murray Sanders, MDT
The report by Alpers and Farmer [ 1] of 2 cases of amyotrophic lateral sclerosis following the use of pneumatic drills
focused interest on the possible role of vibratory tools in
motor neuron disease. From 1972 to 1981 over 1,100 patients with clinically confirmed motor neuron disease sought
treatment at the Sanders Medical Research Foundation.
Complete records were received on all patients. Among
them were 85 male adults whose symptoms had started before the age of 4 5 years. Questionnaires were sent to these 85
men or their survivors asking for information on previous
trauma, prior athletic activities, and whether the subjects had
ever used pneumatic tools. Fifty replies were received.
Among the 50 respondents, 12 (24%) reported having
used an air-driven hammer or drill prior to the onset of their
disease (Table 1).T h e data on the diagnosis and course of the
disease in these 12 men are given in Table 2. In most cases
the initial diagnosis was made by a local neurologist and later
confirmed by specialists at one or more medical centers or
university clinics. Appropriate testing was done in all cases.
All these men exhibited fasciculations of the muscles of the
extremities or trunk, or both, followed by atrophy and weakness of the involved muscle groups. Pyramidal signs preceded
or eventually followed the signs of lower motor neuron disease noted in the involved muscles.
In 7 patients the earliest symptoms were restricted to
the cervical cord, in 3 others the first symptoms were
hemiparetic, and in 2 patients one or both lower extremities
were affected. No patient had bulbar symptoms. In all patients the disease was progressive; it was fatal in 5 . Remarkably for a disease of such severity, some of the men have
survived for rather long periods, i.e., 7, 9, and 14 years.
There seemed to be no clear relation between the degree of
exposure to vibrations from thc air-driven tools and the severity of the disease.
Felmus and colleagues 121 found a history of pneumatic
drill use in 8 of 16 male patients with amyotrophic lateral
sclerosis. Their patients were from a region of extensive oil
exploration where pneumatic drills are commonly used, and
the average age of the patients was 5 1 years. By contrast the
patients reported here were from a broad geographic area,
and all were younger than 45 years of age when the first
symptoms occurred.
Prior observations of the clinical effects of vibration from
heavy air-driven tools have been limited to the well-known
vasospastic condition of the hands, similar to Raynaud's disease, called zibratiotz-induced whztejinger or uhzte band 131. I n
1974 the National Institute of Occupational Safety and
Health concluded that about 2.5 million construction workers and 110,000 underground miners were exposed to vibration from heavy air-driven tools 141. Statistics from the
United States Bureau of Labor numbered the 1974 work
force at about 92 million; assuming that about 6 0 9 of all
workers are men, the incidence of exposure to pneumatic
tools in the male working population is estimated roughly a t
Table 1 Octuputinnd Hzrtoqi and Presenting Symptoms of 12 Men uvth Motor Neuron Dtreusr Dezehptng b t f ~45~ Years of Age
Pneumatic Tool Use
Patient
No.
Age at
Onset (yr)
Occupation
Tool
Years
of Use
Frequency of Use
Presenting Symptoms
Farming/
ranching
Construction
Jackh;unmer
5
Occasional
Paresis, hand
Large drill
1
Occasional
Machine operation
Gasline construction
Construction
Construction
Mining
Construction
Construction
Oil field
drilling
Construction
Road construction
Light hammer
9
Frequent
Paresis, fingers
and arms
Tremors, deltoids
75 ib drill
4
Frequent
Paresis, leg
75 Ib drill
Jackhammer
Drill
Jackhammer
Jackhammer
Jackhammer
< 1
12
10 (!)
8
10 (?)
Frequent
Occasional
Frequent
Occasional
Frequent
Occasional
Paresis,
Paresis,
Paresis,
Paresis,
Paresis,
Paresis,
10 (?I
< 1
Frequent
Frequent
Paresis, arm and leg
Paresis. both arms
~~
1
28
2
10
3
31
4
33
5
6
35
35
7
36
8
10
37
38
42
11
12
42
42
3
Jackhammer
Jackhammer
(?)
4
694 Annals of Neurology Vol 14 No 6 December 1983
hand
hand
hand
arm and leg
both legs
arm and leg
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