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diabetes mellitus (4-8), but this association has only occasionally been reported. We report and illustrate a patient with nonketotic hyperglycemia and generalized chorea.

Case History
An 81-year-old woman developed generalized, abnormal, involuntary movements. She had been taking
amiloride (100 mg daily) because of hypertension. She
had never received neuroleptic drugs. One week before
admission she noted intense thirst, polyuria, and involuntary movements of all four limbs and face. The clinical
picture worsened gradually. Physical examination revealed an alert woman with mild signs of dehydratation
and generalized choreic movements. The rest of the examination showed normal results. The serum glucose
level was 630 mg/dl. Glucosuria was found and ketones
were absent. The rest of the routine biochemical blood
test including acanthocytes, thyroid function, calcium
and phosphorus, pH and bicarbonate, muscle and liver
enzymes, and antinuclear antibodies were all normal. A
computed tomography (CT) scan was also normal but
magnetic resonance imaging showed small infarcts in subcortical white matter, which was considered “normal for
an elderly person.” She was treated with intravenous fluids, insulin, and tetrabenazine (100 mg daily) because she
was exhausted. In a few days, serum glucose was normalized and the dyskinesias disappeared. We tried to
withdraw tetrabenazine, but the chorea reappeared and
the patient was discharged with a regimen of tetrabenazine 100 mg daily. One month later, the blood sugar level
remained normal and she was free of chorea. We tried to
slowly reduce the dosage of tetrabenazine again, and the
chorea reappeared. Three months later, the blood glucose
level continued to be normal. The tetrabenazine then was
slowly reduced and stopped. Five months after admission, she was free of dyskinesias with a normal serum
glucose level.

Discussion
Nonketotic hyperglycemia has been associated with
paroxysmal choreoathetosis (4-6), hemichorea (7),
hemiballismus (8), and tonic spasms (9). The case we
have reported has generalized chorea associated with
nonketotic hyperglycemia. However, there was no immediate improvement of the dyskinesia after the control of
blood sugar. There are many other possible causes of
chorea (1-3). Most of them were excluded in our patient
with the exception of senile chorea and chorea of vascular
origin. It is well known that in the elderly population
chorea appears increasingly even in those never exposed
to neuroleptic agents (1). However, chorea in aging is
largely limited to the facial area, sparing the trunk and
extremities, and is more likely a pathologic manifestation
of an as yet undefined vulnerability in this elderly population. Generalized chorea due to small lacunar infarctions in the basal ganglia has only rarely been described
(&13), and it has been suggested that the particular vascular requirement for producing generalized chorea is
ischemia rather than infarction (12).

Movement Disorders, Val. 8 , N o . I , 1993

We suggest that in our patient these two factors (old
age and vascular lesions) have played a predisposing and
a “chorea perpetuating” role. In fact, in the case reported
by Sethi et al. (7), chorea also resolved slowly and the CT
scan showed bilateral hemorragic infarctions in caudates
and putamina.

Legends to Videotape
Segment 1. Generalized chorea. Admission day.
Segment 2. Five months later, without chorea.

G . Linazasoro
M. Urtasun
J. J . Poza
J . A. Suarez
J. F. Marti Mass6
Servicio de Neurologia
Hospital de Arantzazu
San Sebastian, Spain

References
I . Shoulson 1. On chorea. Clin Neuropharmacol 1986;9 (suppl
2): S85-S99.
2. Padberg G, Bruyn GW. Chorea: differential diagnosis. In:
Vinken PS, Bruyn GW, Klawans HL, eds. Handbook of
clinical neurology, vol 49. Amsterdam: Elsevier, 1986:549564.
3. Fahn S. The other choreas. Aspen, Colorado, 1991:281-306.
4. Rector WG, Herlong HF, Moses H. Nonketotic hyperglycemia appearing as choreoathetosis or ballism. Arch Intern
Med 1982;142:154-155.
5. Totoritis M, Cornish D, Thompson F. Nonketotic hyperglycemia. Arch Intern Med 1982;142:1405.
6. Haan J, Kremer HPH, Padberg G. Paroxysmal choreoathetosis as presenting symptom of diabetes mellitus. J Neurol
Neurosurg Psychiatry 1989;52:133.
7. Sethi KD, Allen M, Sethi RK, McCord JW. Chorea in hypoglycemia and hyperglycemia [Abstract]. Neurology
1990;40 (suppl 1):337.
8. Stone LA, Armstrong RM. An unusual presentation of diabetes: hyperglycemia inducing hemiballismus [Abstract].
Ann Neurol 1989;26:164.
9. Maccario M, Brooklyn MD. Neurological dysfunction associated with nonketotic hyperglycemia. Arch Neurol 1968;19:
525-534.
10. Folstein S, Abbot M, Moses R, Parlad I, Clark A, Folstein
M. A phenocopy of Huntington’s disease: lacunar infarcts of
the corpus striatum. Johns Hopkins Med J 1981;148:104113.
11. Tababton M, Mancondi G, Loeb C. Generalized chorea due
to bilateral small deep cerebral infarcts. Neurology 1985;35:
588-589.
12. Sethi KD, Nichols FT,Yaghmai F. Generalized chorea due
to basal ganglia lacunar infarcts. Mov Disord 1987;2:6146.
13. Margolin DI, Marsden CD. Episodic dyskinesias and transient cerebral ischemia. Neurology 1982;32:1379-1380.

Bifrontal Glioma Presenting as a Gross
Movement Disorder
To the Editor:
We report the case of an 89-year-old woman who presented with gross abnormal movements, identical with

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those seen in tardive dyskinesia, which were secondary
to a large bifrontal glioma. Videotapes displaying her clinical findings pre- and post-treatment are included in two
segments. We are unaware of any previous reports in
which an intracranial tumour has caused such gross
movement abnormalities.
This 89-year-old woman was admitted to St. Charles
Hospital in April 1991. Three weeks prior to admission
she had developed, over a few days, involuntary orofacial
movements that had progressed rapidly to include bilateral involuntary arm and leg movements, which caused
her to fall on a number of occasions. She had difficulty in
swallowing, was sometimes incontinent of urine, and was
unable to speak. Prior to this, she had been a healthy,
independent woman with no medical complaints.
Her past medical history was unremarkable. She was
taking Navidrex K 1 in the morning for mild heart failure.
Apart from an occasional paracetamol, she had never
taken any other medication; in particular, she had never
taken neuroleptics.
On examination, she showed a wide range of involuntary movements including blepharospasm, orofacial dyskinesia, choreiform movements of both arms, hemiballism of the left arm, dystonic posturing of the left arm,
body dystonia, and stereotypy of the legs, more so on the
left (Segment 1 of video). She had a mild pyramidal weakness of the right side and an upgoing left plantar response.
There were no cranial nerve abnormalities and her fundi
could not be visualised because of cataracts. Physical examination was otherwise normal.
Investigations, including a complete blood count, ESR,
biochemical screen, liver function, thyroid function, serum copper, glucose, and a chest X-ray, were all unremarkable.
The involuntary movements decreased somewhat on
tetrabenazine 25 mg twice a day. After 1 week, sulpiride
100 mg twice a day was added. Within 3 days, she showed
considerable improvement, although she continued to
have some orofacial dyskinesia, left arm choreiform
movements, and stereotypy of the left leg (Segment 2 of
video). Despite this, she was now walking more easily
and her swallowing was less troublesome.
An MR scan showed a large bifrontal glioma involving
the corpus callosum and basal ganglia (Fig. 1). Tetrabena-

121

zine and sulpiride were discontinued and dexamethasone
commenced, starting at 4 mg t.d.s., reducing gradually to
4 mg mane after 2 weeks. The result was very interesting
in that the steroids alone now maintained her improvement with regard to dystonia and hyperkinesia and also
led to an improvement in her speech output.
One month after admission, she was discharged home.
She was well at this time, showing fewer abnormal movements and making steady progress. Unfortunately, she
developed a chest infection and passed away peacefully 3
weeks later. Permission for a postmortem examination
was refused. One week prior to death, her dexamethasone had been stopped by her daughter because of her
deteriorating health. According to her, the patient’s abnormal movements returned to the same extent as on her
admission.
Focal brain lesions occasionally produce typical focal
or hemidystonia (1-3). Marsden’s comprehensive paper
on hemidystonia (4)showed that responsible lesions may
include tumours, arteriovenous malformations, infarcts
(post-traumatic, thrombotic, or embolic), or haemorrhages. The lesions involved a variety of cerebral regions
including the thalamus, caudate nucleus, lentiform nucleus, internal capsule and cortex. Focal or hemidystonia
was the only neurological abnormality in most of his patients.
Our patient had a bilateral movement disorder comprising orofacial dyskinesia, chorea, and dystonia, strongly
reminiscent of tardive dyskinesia. However, there was no
history of neuroleptic use at any time in the past. There
are few reports of tumours causing such an unusual complication. Pall reported a case of bilateral Parkinsonism
with long tract signs that were thought to be due to a large
left hemisphere intrinsic tumour with contralateral hydrocephalus (5). There are other short reports similar to this
but more typically describing hemidystonia or hemiparkinsonism (6,7). It is reasonable to assume that the gross
abnormal movements in our patient were due to the mechanical pressure caused by the tumour and its related
oedema, which certainly involved basal ganglia structures
and connections. This is suggested by the response she
had to dexamethasone and the subsequent return of her
abnormal movements when it was stopped by her family
during the final days of her illness.

FIG. 1. There is a large bifrontal glioma centered on the anterior part of the corpus callosum.
including trunk and genu, and extending widely
into both cerebral hemispheres. There is also a
posterior cerebral artery territory infarct.

Movement Disorders, Voi. 8, N o . I , 1993

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Acknowledgments: We wish to thank Miss Jan Kleina
for typing the manuscript and Dr. Rob Hicks for his help
in editing the videotapes.

Legends to Videotape
Segment 1. Patient at presentation showing Orofacial
dyskinesia; blepharospasm; choreoathetoid movements
of the arms and legs L > R; stereotypy of the left leg;
dystonic body posturing; and hemiballism of the left arm.
Segment 2. Ten days after treatment showing a dramatic improvement. She still has some orofacial movements, left arm chorea, and left leg stereotypy.

S. Roche
S. Godward
A . Middleton
Department of Medicine
for the Eideriy
St. Charles Hospital
London, England
R. J. M. Lane
Regional Neurosciences Centre
Charing Cross Hospital
London, England

References
I . Brett EM, Hoare RD, Sheehy MP, Marsden CD. Progressive hemidystonia due to focal basal ganglia lesion after mild
head trauma. J Neurol Neurosurg Psychiatry 1981;44:460.
2. Norbona J, Obeso JA, Tunon T, Martinez-Lage JM, Marsden CD. Hemidystonia secondary to localised basal ganglia
tumour. J Neurol Neurosurg Psychiarq 1984;47:704-709.
3. Obeso JA, Martinez-Vila E, Dalgado G, Vaamonde J, Maravi E , Martinez-Lage JM. Delayed onset of dystonia following hemiplegic migraine. Headache 1984;24:266-268.
4. Marsden CD, Obeso JA, Zarranz JJ, Lang AE. The anatomical basis of symptomatic hemidystonia. Bruin 1985;108:463483.
5. Pall HS. 74 year old lady who developed bilateral Parkinsonism secondary to an intrinsic cerebral tumour. J Neurol
Neurosurg Psychiatry 1987;50:13861387.
6. Navarro JA, Ezquerro JJR, Albuquerque TL, Gutierrez JC.
Parkinsonism due to corpus callosum astrocytoma. J Neurol
Neurosurg Psychiatry 1986;49:1457-1458.
7. Kulali A, Tugtedin M, Utkur Y, Erkurt S . Ipsilateral hemiParkinsonism secondary to an astrocytoma. J Neurol Neurosurg Psychiatry 1991;54:655.

Alcohol Sensitive Dystonia

mpzm
SU REWENT

To the Editor:
Alcohol intake improves a large proportion of patients
with essential tremor (l), hereditary essential myoclonus
(2,3), and inherited myoclonic dystonia (4). Patients with
idiopathic torsion dystonia (ITD) do not show such striking sensitivity to alcohol, although alcohol-induced relaxation may provide some benefit (1). We recently described a patient with myoclonus and abnormal gait of
sporadic presentation (5) who showed dramatic improvement with alcohol, suggesting that the spectrum of alco-

Movement Disorders, Vol. 8, No. I , 1993

hol-sensitive movement disorders may be wider than previously recognized. We now report a patient with a pure
dystonic syndrome sensitive to alcohol.
The patient was a 40-year-old man from Mallorca (Balearic Islands), with a 20 year history of gait disturbance.
There was no family history of neurological disease or
consanguinity. Symptoms began in 1970 when he started
noticing that his right foot rotated externally while walking. Subsequently, the right arm adopted awkward postures when writing. By 1980, when he was 28, writing was
not possible at all. Around 1986-87, the left leg also began
to deviate on walking and rhythmic flexion-extension
movements of the foot were present. These movements
interfered with stance and gait. Treatment with trihexyphenidyl (12 mgiday) and with carbamazepine (600 mgl
day) provided no relief.
We first saw the patient in 1990 at the age of 38. At that
time he had great difficulty in walking and in keeping still.
Dystonic postures were observed in all four limbs on action, and occasionally in the neck muscles, which showed
a tendency to laterocollis. Rhythmic slow movements of
the left foot were present at rest while lying in bed and
were aggravated by action. These movements were the
main source of problems in walking. A similar slow rhythmic flexion-extension movement of the right hand was
also noticed. A dramatic improvement of these myorrhythmic movements and the dystonic postures was consistently observed after drinking -60 g of alcohol. Spontaneous myoclonus was not observed during action or in
response to any form of sensory stimulation. The rest of
the physical examination was normal. Both CT and MRI
scans of the brain showed atrophy with predominance in
the brainstem and cerebellum. All diagnostic blood tests
were normal.
Surface EMC recording of tibialis anterior and gastronemius muscle in the left leg revealed rhythmic activity at a frequency of 2-3 Hz. Each burst had a duration of
100-150 ms and there was alternating activation of both
muscles. This type of recording is characteristic of myorrhythmia (1,7).
This patient showed a combination of generalized dystonia and myorrhythmic movements, but no actual myoclonic activity was detected. Alcohol-induced improvement of this combination movement disorder was evident. Family history was negative. Slow, undulatory and
repetitive movements of one body part have been well
recognized in patients with ITD. Indeed, it was Hertz (7)
who in his classic description of hereditary torsion dystonia referred to these movements as myorrhythmia.
Thus, all the clinical findings of our patient fell within the
spectrum of motor manifestations of ITD (1,8). This is the
first patient in whom alcohol produced a marked improvement of a purely dystonic syndrome. Recently, two
families with progressive myoclonic epilepsy who responded dramatically to alcohol have been described
(9,lO). The effect of alcohol may therefore not be limited
to patients with essential tremor and myoclonic dystonia.

Legend to Videotape
Segment 1 shows dystonic movements and postures in
the four limbs and repetitive myorrhythmic movements in