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6. Lundberg A. Multisensory control of spinal reflex pathways.
Prog Brain Res 1979;50:11-28.

Hemichorea due to Cryptococcal Meningitis
To the Editor:
We describe a patient with cryptococcal meningitis
who was first seen with hemichorea. The presentation of
cryptococcal meningitis with hemichorea is rare; we
found only one case previously recorded in the medical
literature. We discuss the possible pathophysiology of
hemichorea in this condition.
A 58-year-old company director had confusion and fever over a 2-month period. He complained of headache
and nausea. Examination of the nervous system revealed
a confusional state with a mild right-sided pyramidal
weakness, bilateral upgoing plantar responses, and prominent right-sided hemichorea. There was no evidence of
neck stiffness or photophobia. He had a 10-year history of
an idiopathic panuveitis, treated with prednisolone and
recently with cyclosporin. He was a diet-controlled diabetic.
Lumbar puncture showed a lymphocytic meningitis
with a cerebrospinal fluid (CSF) lymphocyte count of 34
cells; protein, 1,750 mg; glucose, 2.6 mM, compared with
a serum glucose of 7.7 mM. India ink preparation demonstrated the presence of cryptococci, and CSF culture
was positive for Cryptococcus neoforrnans. The fungus
was sensitive to amphotericin and flucytosine but resistant to fluconazole. Serum cryptococcal antigen was positive at 1:8,000, and CSF antigen levels positive at
1:4,000. Computed tomography (CT) head scan showed
several bilateral areas of low attenuation in the cerebral
white matter, consistent with areas of ischaemia or infarction, but no lesions in the basal ganglia.
The patient improved on amphotericin and 5-fluorocytosine. The dose of prednisolone was reduced, but this
led to a recrudescence of his uveitis, and the steroids
were restarted. One year after presentation, his choreiform movements have improved but are still present. He
still has mild cognitive impairment and has not returned
to work. The CSF and serum cryptococcal titres have
remained positive, and he remains on antifungal treatment.
Chorea has been noted to occur in a variety of central
nervous system (CNS) infections. Burstein and Breningstall (1) described choreoathetotic movement disorders in
five children with meningitis caused by different organisms. The development of chorea with tuberculous meningitis is well documented. In one series, 13% of cases of
tuberculous meningitis developed dyskinesias (2). In tuberculous meningitis, basilar meningeal vessels become
enveloped by a purulent exudate. Spasm or thrombosis of
these vessels may result in ischaemic areas in the basal
ganglia, which may not show on CT head scanning (3).
Cryptococcus neoformans causes a chronic basilar
meningitis (4), the pathophysiology of which is not dissimilar to that of tuberculous meningitis (5). The disease
can affect normal individuals, in whom the disease is of
insidious onset or occasionally asymptomatic. In immunosuppressed patients, the course of the disease is more
rapid. The disease has become more frequent with the

Movement Disorders, Vol. 10, No. 4, 1995

immunosuppression of acquired immunodeficiency syndrome (AIDS). In our patient, multiple areas of low attenuation were seen on CT scanning, and we suspect that
the cause of the chorea is disturbance of local blood supply or direct fungal invasion of the striatal to subthalamic
to external pallidal pathway.
Cryptococcal meningitis is a rare cause of movement
disorders. In an extensive review of the literature, we
found only one case of movement disorder secondary to
cryptococcal infection (6). Our case occurred in a 58year-old man who had long-standing immunosuppression.
Cryptococcal meningitis is another cause of hemichorea
and should be suspected when there is evidence of immunosuppression.

R. A. Weeks
*C. G. Clough
Department of Neurology
Hammersmith Hospital
*Department of Neurology
Brook General Hospital
London, England

References
1 . Burstein L, Breningstall GG. Movement disorders in bacterial meningitis. J Paediatr 1986:Aug:260-263.
2. Udani PM, Parekh UC, Dastur DK. Neurological and related syndromes in CNS tuberculosis. Neurol Sci 1971:14:
341-357.
3. Poltera AA. Vascular lesions in intracranial tuberculosis.
Pathol Microbiol 1975;43: 192-194.
4. Sabetta JR, Andriole VT. Cryptococcal infection of the central nervous system. Med Clin North Am 1985;69:333-343.
5. Stockstill MT, Kauffman CA. Comparison of cryptococcal
and tuberculous meningitis. Arch Neurol 1983:40:81-85.
6. Namer IJ, Tan E, Akalin E, Selekler K, Zileli T. Hemiballismus with cryptococcal meningitis. Rev Neurol 1990;146:
1 53-1 54.

Hemidystonia Consequent upon Ipsilateral Vertebral
Artery Occlusion and
Cerebellar Infarction
To the Editor:
Hemidystonia is often associated with focal basal ganglia lesions (1). We report the case of a female patient
with a mild, predominantly right-sided unexplained
movement disorder, who developed left hemidystonia after ipsilateral vertebral artery occlusion with cerebellar
infarction, probably related to extracranial vertebral artery dissection.
This woman, born in 1952, was first referred to our
Neurological Department in 1984 for predominantly rightsided abnormal movements. Her birth was apparently
normal. Her twin brother is in good health. Abnormal
movements started at 3 or 4 years of age. They consisted

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of choreic and athetotic movements, predominantly affecting the right side and also, although more rarely and
less conspicuously, the left side. Slight left cervical laterocollis was also present, probably resulting from dystonic activity in left-sided neck muscles. Neurological examinations [electroencephalography, computed tomography (CT) scan, biological tests, etc.] yielded normal
results. The abnormal movements were presumed to be
secondary to perinatal cerebral injury. However, they
did not result in severe disability, and the patient was
able to take up a career as a typist. Unfortunately, no
video recordings of her movements at this time are available.
She was readmitted in September 1989, complaining of
laterocervical pain, gait disturbance, and dizziness, all of
which had appeared a few days before. A left cerebellar
syndrome was diagnosed. There was persistence of the
previously described abnormal movements. Angiography
showed an occlusion in the first part of the left vertebral
artery. CT scan showed a large left cerebellar hypodensity affecting the territory of the posterior inferior cerebellar artery and to a lesser extent that of the superior
cerebellar artery. One month later, the cerebellar syndrome had improved, From November 1989, however,
there was a change in involuntary movements: left-sided
dystonic movements appeared, which progressively became more and more incapacitating, such that the patient
could no longer perform her job as a typist and had to take
up a post as a switchboard operator, but found even this
more and more difficult.
In February 1991 (see videotape), dystonia predominantly affected the proximal and distal segments of the
left arm, which was either hyperpronated or flexed at the
elbow with wrist pronated and fingers extended. Her left
foot was also affected, although to a lesser degree. A
tendency toward hyperlordosis was sometimes observed
when she was walking. In addition, spasmodic contraction of the neck muscles caused rhythmic jerks of the
head, mainly toward the most active muscles. The movements affecting her right side were different, more rapid,

523

and at times evocative of choreoathetotic movements,
sometimes of dystonic movements, but less markedly
than on the left side. No pyramidal signs, sensory loss, or
cerebellar axial syndrome were present.
An additional CT scan (difficult to implement, given the
cervical dystonia) confirmed the existence of left cerebellar hypodensities. Doppler ultrasonography confirmed
the occlusion of the left vertebral artery. Magnetic resonance imaging (MRI) (Fig. 1) showed a large lesion in the
lower and posterior left cerebellar hemisphere, affecting
the inferior semilunar lobule and to a lesser degree the
superior semilunar lobule (both regions are supplied by
the posterior inferior and the superior cerebellar arteries).
It also showed several lesions affecting the left basal ganglia, but none were detected on the right side. HMPAO
single-photon emission computed tomography (SPECT)
showed reduced HMPAO uptake in the left cerebellum
and in the contralateral frontoparietal area (Fig. 2). The
administration of several drugs (muscle relaxants, anticholinergic agents, levodopa, etc.) did not induce any noticeable improvement, nor did thalamic stimulation of the
right nucleus ventroposterolateris. Injections of botulinum toxin only led to transient partial improvement.
Since early 1991, her neurologic condition has considerably worsened.
A particular feature of our observation is the modification in the aspect of preexisting abnormal movements and
the development of predominantly left-sided hemidystonia after a left-sided cerebellar infarct. This raises a twofold question as to the physiopathology of this left
hemidystonia and the role played by the cerebellum. Although the etiology of hemidystonia is diverse, it is most
often related to lesions affecting the basal ganglia, the
lenticular nucleus, the caudate nucleus, and the thalamus
(1). The choreic athetotic predominantly right-sided involuntary movements present and evolving since childhood, which were not really incapacitating, can probably
be related to basal ganglia injury due to vascular lesions
or occurring postanoxia, as is suggested by the patient’s
history and MRI.

A-C

FIG. 1. MRI,0.5 T, showed left cerebellar hypodensities (A, TI-weighted, 460, 13-; B, T2-weighted, 2,200, 120-) and small hyperintense lesions affecting the left basal ganglia (C, T2-weighted, 2,200, 120-).

Movement Disorders, Vol. 10, No. 4, 1995

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FIG. 2. Reduced HMPAO uptake in the

right frontoparietalarea and left cerebellum,
as shown by HMPAO SPECT.

The occurrence of left cerebellar infarction later decompensated this situation, worsening the preexisting
disorders in the right striatopallidothalamocortical pathway. This modification can be related to either deafferentation of the right-sided basal ganglia due to crossed
cerebello-cerebral diaschisis or possibly to an alteration
of the right prefrontal cortex, considered an “exit” for
the right striatothalamocortical pathway.
This observation therefore raises the question of
the possible role of the left cerebellar infarction in the
modification of abnormal movements and the development of predominantly left-sided hemidystonia. The arguments in favor of a possible role of the cerebellum in
the occurrence of abnormal dystonic movements have
been drawn mostly from animal models of dystonia. In
the dystonia musculorum deformans mouse (2), increased
noradrenergic cerebellar metabolism has been demonstrated in addition to peripheral and spinal lesions. In
rats, locus ceruleus stimulation responsible for the inhibition of the Purkinje cells induces a dystonialike syndrome (3). More importantly, the Sprague-Dawley rat
mutant dystonic (dt), investigated by Lorden et al., is
characterized by a dysfunction in the control of cerebellar
efferents (4).
Cases of dystonia related to cerebellar lesions in humans have rarely been reported. Some cases of focal dystonia (writer’s cramp, spasmodic torticollis) have been
reported in degenerative cerebellar ataxia (9,and blepharospasm can be seen in patients with olivopontocerebellar atrophy (6); one case of cervical and upper limb
dystonia was reported in a patient with primary cerebel-

Movement Disorders, Vol. 10, No. 4, 1995

lospinal degeneration (7). However, the role of the cerebellum in the pathogenesis of dystonic movements is disputable because in all these disorders the pathology is not
confined to the cerebellum but also extends directly or
indirectly compresses the brainstem, where it is known
that lesions can result in cranial dystonia (8). Metabolic
studies performed in dystonia seldom have shown cerebellar abnormalities. The most interesting observation,
and the one most like ours, is a case of left spasmodic
torticollis in a patient exhibiting a cavernous angioma in
the right middle cerebellar peduncle; positron emission
tomography scan showed right cerebellar hypometabolism related to crossed cerebello-cortical diaschisis (9).
Gilman et al. (10) reported increased cerebellar metabolic
activity contralateral to the most affected side in a single
patient with idiopathic generalized torsion dystonia.
Given the considerable number of cerebellar disorders,
particularly of vascular origin, and the scarcity of observations associating cerebellar lesions and dystonia, it can
be wondered whether the hemidystonia and cerebellar
infarction in this patient might be conciliated. However,
our case is similar to that described by Tranchant et al.
(9), who proposed that dystonic movements might be the
consequence of striatal deafferentation from the cortex.
The delay between the cerebellar infarction and the onset
of hemidystonia might reflect a possible role of a metabolic disturbance. In addition, the presumed history of
neonatal anoxia might possibly render basal ganglia vulnerable to any subsequent insult.
In conclusion, our observation provides an additional
case of a relatively rare phenomenon, namely crossed

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cerebello-cerebral diaschisis, associated with another
rare phenomenon, hemidystonia. It raises the question of
possible links between hemidystonia and ipsilateral cerebellar injury and provides a new twist to the already complex puzzle of the etiopathological mechanisms underlying dystonia (1 1).

525

10. Gilman S, Junck L, Young AB et al. Cerebral metabolic
activity in idiopathic dystonia studied with position emission
tomography. Adv Neurol 1988;50:231-326.
11. Marsden CD, Quinn NP. The dystonias. Br Med J 1990;300
139-144.

Legend to Videotape
This tape was first recorded in February 1991,4 months
after the occurrence of the left cerebellar infarction. The
patient’s abnormal movements affect both sides differently: on the right, distal movements are short, quick, and
often “explosive”; on the left, movements are slower, of
a dystonic type, with abnormal postures of the hand, upper limb, and head. In July 1991, her movements are similar, markedly increased on the right side when she performs mental calculation. The aspect of movement disorders is slightly different on the 1992 recordings: head
dystonia is more obvious, and movements are worsened.
Later (June 2, 1992 and February 12, 1993), her functional
status has further deteriorated (e.g., handling an envelope) and the same dissymmetry is found in the nature of
her abnormal movements.

L. Rumbach
P. Barth
A. Costaz
J. Mas
D e p a r t m e n t of Neurology
Hdpital J e a n Minjoz
Besanqorz, France

References
1. Marsden CD, Obeso JA, Zarranz JJ, Lang AE. The anatom-

ical basis of symptomatic hemidystonia. Brain 1985;108:463483.
2. Duchen LW, Strich ST, Falconer DS. Clinical and pathological studies on a hereditary neuropathy in mice (dystonia
musculorum). Brain 1964;87:367-378.
3. Jacquet Y. A dystonia-like syndrome after neuropeptide
(MSHIACTH) stimulation of the rat locus ceruleus. Adv
Neurol 1988;50:299-311.
4. Lorden JF, Lutes J, Michela VL, Ervin J . Abnormal cerebellar output in rats with an inherited movement disorder.
Exp Neurol 1992;118:95-104.
5 . Fletcher NA, Stell R, Harding AE, Marsden CD. Degenerative cerebellar ataxia and focal dystonia. Mov Disord 1988;
3:336-342.
6. Janati A, Metzer WS, Archer RL, Nickols 3, Raval J. Blepharospasm associated with olivopontocerebellar atrophy. J
Clin Neuro Ophtalrnol 1988;9:281-284.
7 . Tyrer JH, Sutherland JM, Eadie MJ. Dystonie musculaire
deformante a s s o c i t e a une dCgCntrescence spinocCrCbelleuse primaire. Rev Neurol 1964;110:406410.
8. Gibb WR, Lees AJ,Marsden CD. Pathological report of four
patients presenting with cranial dystonias. Mov Disord 1988;
3:211-221.
9. Tranchant C, Maquet J, Eber AM, Dietemann JL, Franck P,
Warter JM. Angiome caverneux ctrtbelleux, dystonie cervicale et diaschisis cortical crois6. Rev Neurof 1991;147:599602.

Botulinum A Toxin for the So-called Apraxia of
Lid Opening

m
PP MEW

To the Editor:
So called apraxia of lid opening (AEO) is a nonparalytic
movement disorder frequently associated with Parkinson’s disease (PD), parkinsonism, or blepharospasm (BS)
(1,2). As pointed out by Lepore and Duvoisin (l), it is
characterized by the following combination: (a) transient
inability to initiate lid opening; (b) no overt orbicularis
oculi (00)spasm; (c) vigorous frontalis contraction; and
(d) absence of oculornotor or ocular sympathetic nerve
dysfunction or ocular myopathy. An abnormality of the
normal reciprocal relationship between the levator palpaebre superior (LPS) and the 00 muscles is likely involved in its pathophysiology. In fact, LPS inhibition (2)
and abnormal contraction in the pretarsal00 (3) were the
main electromyographic (EMG) features in different series. Thus, the disorder was also designated as involuntary levator palpebrae inhibition (1) or pretarsal BS (3).
We studied the effect of botulinum A toxin (BTX; Allergan, Irvine, CA) injected into different parts (orbital/
preseptal and pretarsal) of the upper 00 of two patients
with so-called AEO: a 55-year-old man affected by progressive supranuclear palsy (PSP) since 48 years of age
and a 76-year-old woman who had been treated for PD
with carbidopa-levodopa and bornaprine for 8 years. At
ages 53 and 73, respectively, they developed transient
difficulty in voluntarily opening their eyes. So-called
AEO was diagnosed according to Lepore and Duvoisin’s
clinical criteria (1). We did not perform EMG recordings
of pretarsal 00 or LPS because they are unwieldy, require expertise not readily available in our setting, and
seem unnecessary to diagnose so-called AEO (4). Interestingly, eye closure was accompanied by lower lid spastic entropion (5) in the patient with PSP. To our knowledge, no reports exist of such an association, although
both disorders can separately occur in the course of BS or
parkinsonism (5). This observation adds further support
to the hypothesis of an active contraction of some portion
of the 00 as a mechanism for so-called AEO (3).
On baseline, the episodes of lid closure lasted 30-40 s
to 1-3 rnin and there was a trend to increasing duration
from morning to afternoon hours. Sometimes eyes could
be opened by massaging the lids. Edrophonium did not
alter the duration of such episodes. Patients were first
injected with BTX in the upper lid (20 U subdivided in
two puncture sites) at the junction of the preseptal and
orbital portion of the 00, rnedially and laterally to avoid
the midline LPS. They reported slight subjective improvement of visual function, but no objective improvement was found on clinical examination; in fact, no less

Movement Disorders, Vot. 10, N o . 4, 1995