236 zyxwvutsrqp
zyxwv COMMUNICATIONS
the lesion includes the nucleus of the accessory nerve,
located within the posterolateral grey matter of the upper
six cervical segments, descending pathways, afferent and
efferent fibers, as well as local neuron circuits participat-
ing in head and neck movement control.
Jancovic and Patel (10) suggested that in cases of ros-
tral brainstem lesions and blepharospasm, an interruption
of the descending pathways may be responsible for a dis-
inhibition of the facial nucleus and brainstem reflexes. In
our case, we similarly assume that the accessory nucleus
and the reflexes on the cervical level may be disinhibited.
The underlying pathophysiological mechanism might be
the same as the one suggested by Rothwell et al. ( l l ) , who
had demonstrated a reduced reciprocal inhibition of an-
tagonist muscles in various idiopathic dystonias, includ-
ing ST. The cause of this they had seen in abnormal spinal
interneuronal activity, as has been proposed in focal dys-
tonias for the brainstem level as well (12). Disturbed cer-
vical reflexes may also result from lesions of the afferent
fibers, especially those fibers providing proprioceptive in-
formation for control of head posture. The capability of
some patients to alleviate their ST by merely touching
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their head (“geste antagonistique”) supports the signifi-
cance of afferent impulses. Further substantiation of a
possible role of the accessory nerve in the pathophysiol-
ogy of ST comes from surgery; neurosurgeons have re-
peatedly claimed to have alleviated an ST by sectioning
neural anastomoses and freeing the 11 th nerve from neu-
rovascular contact (13,14).
As far as we know, this report of ST is the first to show
a circumscribed lesion inside the upper cervical spinal
cord. In three cases of ST associated with syringomyelia
and spinal cord tumor, the anatomic levels of the latter
were not given, and it was not clear whether it was the
syringomyelia or those tumors that participated in the
pathogenesis of the ST (15). Although in our case neither
the demonstrated spinal cord lesion nor the MS itself can
be definitely proven to have caused the ST, in our opinion
the cervical medulla should be enclosed in the diagnostic
search for possible etiologies of ST.
It should be added that during 2 years of repeated in-
jections of botulinum toxin there was no worsening of the
MS due to a putative immune mediated reaction of the
toxin, as has been proposed in single cases of brachial
plexopathy (16) and polyradiculoneuritis (17).
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Wolfgang Klostermann
Peter Vieregge
Detlef Kompf
Department (7f Neurology
Medical University of Lubeck
References
Lubeck, Germany
1. Bachman DS, Lao-VClez C, Estanol B. Dystonia and
choreoathetosis in multiple sclerosis. Arch Neurol 1976;33:
590.
2. Coleman RJ, Quinn NP, Marsden CD. Multiple sclerosis
presenting as adult onset dystonia. Movement Disorders
1988;3:329-332.
zyxwvutsrq
3. Guillain G, Bize R. Sur un cas de sclkrose en plaques avec
torticolis spasmodique. Rev Neurol 1933;40:133-138.
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Movement Disorders, Vol. 8 , No. 2,1993
4. Herz E, Glaser GH. Spasmodic torticollis. Arch Neurol Psy-
chiatry 1949;61:227-239.
zy
5 . Matthews WB, Acheson ED, Batchelor JR, Weller RO.
McAlpine’s multiple sclerosis. Edinburgh-London-Mel-
bourne-New York: Churchill Livingstone, 1985:118.
6. Riley D, Lang AE. Hemiballism in multiple sclerosis. Move-
ment Disorders 1988;3:88-94.
7. Plant GT, Kermode AG, du Bouley EP, McDonald W1.
Spasmodic torticollis due to a midbrain lesion in a case of
multiple sclerosis. Movement Disorders 1989;4:359-362.
8. Poser CM, Paty DW, Scheinberg L, et al. New diagnostic
criteria for multiple sclerosis: guidelines for research proto-
cols. Ann Neurol 1983;13:227-231.
9. Suchowersky 0, Calne DB. Non-dystonic causes of torticol-
lis. Adv Neurol 1988;50:501-508.
zyxw
10. Jancovic J, Patel SC. Blepharospdsm associated with brain-
stem lesions. Neurology 1983;33:1237-1240.
1 1 . Rothwell JC, Day BL, Obeso JA, Berardelli A, Marsden
CD. Reciprocal inhibition between muscles of the human
forearm in normal subjects and in patients with idiopathic
torsion dystonia. Adv Neurol 1988;SO:133-140.
12. Tolosa E, Montserrat L, Bayes A. Blink reflex studies in
focal dystonias: enhanced excitability of brainstem interneu-
rons in cranial dystonia and spasmodic torticollis. Move-
ment Disorders 1988;3:61-69.
13. Freckmann N, Hagenah R, Herrmann HD, Miiller D. Bilat-
eral microsurgical lysis of the spinal accessory nerve roots
for treatment of spasmodic torticollis: follow-up of 33 cases.
Acta Neurochir 1986;83:47-53.
14. Shima F, Fukui M, Kitarnura K, Kuromatsu C, Okamura T.
Diagnosis and surgical treatment of 11th nerve origin. Neu-
rosurgery 1988;22:35&363.
15. Kiwak KJ, Deray MJ, Shields WD. Torticollis in three chil-
dren with syringomyelia and spinal cord tumour. Neurology
I983 ;33:94&948.
16. Glanzrnan RL, Gelb DJ, Drury I, Brornberg MB, Truong
DD. Brachial plexopathy after botulinum toxin injections.
Neurology 1990;40:1143.
17. Haug BA, Dressler D, Prange HW. Polyradiculoneuritis fol-
lowing botulinum toxin therapy. J Neurol 1990;237:62-63.
Effect of L-Dopa on OcuIogyric Crises in a Case of
Dopa-Responsive Dystonia
To the Editor:
Dopa-responsive dystonia with marked diurnal fluctu-
ations (DRD), first described by Segawa et al. in 1971 (l),
is characterized by a) onset in childhood or adolescence
of lower limb dystonia with gait disturbances, b) coexist-
ence or subsequent development of parkinsonian signs,
and c) dramatic response to low doses of L-dopa. Dysto-
nia can also involve upper limbs and usually worsens to-
wards evening, especially after exercise (2). Most cases
are familial, suggesting an autosomal dominant transmis-
sion with incomplete penetrance: however, many spo-
radic cases have been described (1-3). The occurrence of
oculogyric crises in DRD has been described only in few
cases with no mention of the effect of L-dopa treatment
(3-7). We report a sporadic case of DRD with a dramatic
effect of L-dopa on oculogyric crises.
A 25-year old woman was admitted to our institute
with a history of dystonia since childhood. There was no
history of encephalitis or any other previous neurological
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COMM UNICATIONS
disease. She was the third of five siblings. Her family
history was completely negative for movement disorders.
At age 6 years, her parents noticed some troubles in her
walking, which consisted of inversion of both feet with
the big toes going up. Over the following years, dystonia
gradually worsened affecting mostly the left leg and also
involving both upper limbs. Her symptomatology pre-
sented as marked diurnal fluctuations with clear worsen-
ing in the evening. At the age of 12, she began to complain
of periodic episodes of involuntary upward eyes deviation
lasting 10-15 min.
Oculogyric crises occurred nearly every day, usually in
the evening, and were associated with emotional stress.
At that time, dystonia made the patient extremely dis-
abled, requiring help for all her daily living activities. Af-
ter the age of 17, the clinical picture was stable. On ad-
mission, she presented obvious dystonia involving all
limbs, more on the left side. Both feet showed a dystonic
posture with inversion and striatal toes. Dystonia made
her gait more and more difficult, especially in the eve-
ning, when it was impossible without help. Examination
also revealed mild rigidity of all limbs and a “poker
face. ” During hospitalization, we observed two oculogy-
ric crises, each lasting -10 min. In addition, the patient
appeared extremely emotional, particularly during oculo-
gyric crises. All investigations, including computed to-
mography (CT) and magnetic resonance (MR) of the
brain, and copper studies, were normal. Treatment with
low doses of L-dopa + PDI (100 mg/day) dramatically
reduced her symptomatology, and within several days,
the patient was able to feed and dress herself, walk, and
even run; also, parkinsonian signs and oculogyric crises
disappeared. After 9 months of treatment, she was seen
as an outpatient and was completely asymptomatic; dur-
ing that period, no oculogyric crises recurred.
Oculogyric crises have been described as a peculiar
symptom in postencephalitic parkinsonism due to en-
cephalitis lethargica, and as an acute reaction to neuro-
leptics and other drugs. Oculogyric crises, in association
to parkinsonism, occurred in 20-30% of all postencepha-
litic patients (8); the effect of L-dopa treatment on oculo-
gyric crises was equivocal. In fact, in some patients,
L-dopa produced only a temporary reduction in occur-
rence of the crises, with subsequent worsening (8-9); in
others, there was an appreciable improvement (10).
Moreover, oculogyric crises have been described in a few
cases of DRD (4-7), but the effect of L-dopa against this
sign has never been clearly reported; in addition, some of
these cases presented with atypical features (7).
Our sporadic case completely fulfils the diagnostic cri-
teria of DRD. The patient received L-dopa treatment 19
years after onset of the disease (and 13 years after onset
of oculogyric crises). Despite this long delay in initiation
of treatment, L-dopa at very low doses was extremely
effective for the treatment of both dystonia and oculogy-
ric crises. This observation suggests that for the majority
of cases reported in the literature, early treatment with
L-dopa might have prevented the appearance of oculogy-
ric crises (3).
Finally, the dramatic response of both symptoms to
L-dopa treatment suggests a common pathophysiological
mechanism.
Paolo Lamberti
23 7
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Michele de Mari
Giovanni Iliceto
Maria Caldarola
Luigi Serlenga
Institute of Neurology
University of Bari
Italy
References
1 . Segawa M, Ohmi K , Itoh S, Aoyama M, Hayakawa H.
Childhood basal ganglia disease with remarkable response to
L-Dopa, hereditary basal ganglia disease with marked diur-
nal fluctuation. Shinryo (Tokyo) 1971 ;24;667472.
2. Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H.
Hereditary progressive dystonia with marked diurnal fluctu-
ations. Adv Neurol 1976;14:215-233.
3. Nygaard TG, Marsden CD, Duvoisin RC. Dopa-responsive
dystonia. Adv Neurol 1988;50:377-384.
4. Deonna T. Dopa-sensitive progressive dystonia of childhood
with fluctuations of symptoms-Segawa syndrome and pos-
sible variants. Neuropediatrics 1986;17:81-85.
5. Rajput AH. Levodopa in dystonia musculorum deformans.
Lancet 1973;1:432.
zyxwvu
6. Nygaard TG, Duvoisin RC. Hereditary dystonia-parkin-
sonism syndrome of juvenile onset. Neurology 1986;36:
1424-1428.
7. Rondot P, Ziegler M. Dystonia-L-dopa responsive or juve-
nile parkinsonism? J Neural Transm 1983;19(suppl):273-
281.
8. Sacks 0 , Kohl M. L-Dopa and oculogyric crises. Lancet
1970;2:215-2 16.
9. Clough CG, Plaitakis A, Yahr MD. Oculogyric crises and
parkinsonism. A case of recent onset. Arch Neurol 1983;40:
36-37.
10. Duvoisin RC, Lobo-Antunes J, Yahr MD. Response of pa-
tients with postencephalitic parkinsonism to levodopa. J
Neurol Neurosurg Psychiatry 1972;35:487495.
Acute and Long-Term Response to Apomorphine in
Cranial Dystonia
To the Editor:
Apomorphine, a direct dopamine agonist, is a powerful
antiparkinsonian drug (1) and can also improve various
types of movement disorders (21, including tardive dys-
kinesia (3,4) and, to a lesser extent, spasmodic torticollis
(5). We describe a patient with cranial dystonia in whom
apomorphine induced a marked improvement of abnor-
mal involuntary movements during acute tests and during
a 2-year period of sustained treatment.
A 71-year-old woman had a medical history of depres-
sion, during which she was treated with antidepressants.
She received haloperidol for 2 months. One year later,
she had conjunctivitis of the right eye, which lasted a
month. Subsequently, she developed blepharospasm and
oromandibular dystonia (Meige’s syndrome) and was
treated chronically with clonazepam (3 mg/day) and tri-
hexyphenidyl(2 mg/day) without any benefit. Two years
later, she gradually deteriorated and presented with jerky
Movement Disorders, Vol. 8 , No. 2 , 1993