1324
bilateral flexor plantar responses. There was
a mild finger/nose, heel/shin and truncal
ataxia. There were no sensory abnormalities
and general examination was normal but his
height was between the 5th-10th percentile
for his age.
Routine investigations at the age of 11
were normal and included a full blood
count, ESR, urea and electrolytes, calcium,
phosphate, alkaline phosphatase, liver function tests, blood sugar, glucose tolerance
test, serum lipids, CK, vitamin B12, folate,
thyroid function, prolactin, cortisol, growth
hormone, urinary protein and respiratory
function tests. The serum magnesium was
low at 0 5 mmol/l (normal 0 7-0-95) with a
24 hour urinary magnesium excretion of
1-46 mmol/l (normal 2 1-6 2). The cerebrospinal fluid protein was 1 13 g/l. An
ECG revealed right bundle branch block
with left axis deviation. An EEG showed a
slight excess of slow forms for his age but no
evidence of epilepsy. Visual evoked
responses were normal. Electromyography
(EMG) showed normal motor and sensory
conduction velocities. Concentric needle
electromyography showed short duration,
polyphasic motor unit action potentials. On
maximal volition there was a full interference pattern of small amplitude with
maximum amplitude of 750 pV. A right
quadriceps muscle biopsy demonstrated a
large number of "ragged red fibres"
affecting only type I fibres. A CT scan was
normal with no evidence of intracranial
calcification.
There have been few previous reports of
the association of the Kearns-Sayre syndrome with hypoparathyroidism.' - The
hypoparathyroidism may predate or postdate the onset of symptoms and signs typical
of the Kearns-Sayre syndrome and may or
may not be associated with seizures (table).
In the three previous cases reports in which
CT scans were performed intracranial
calcification was found, localised to the
basal ganglia. Seigal et al' found intracranial calcification in four of eight patients

Table
Age of presentation of

Author

hypoparathYroidism

Seigal'

2

Toppet eta12
Sachs3
Horwitz4

Pellock5

Present case

7

?
13
9
2

Letter3
with the Kearns-Sayre syndrome, one had
pseudoone
hypoparathyroidism,
hypoparathyroidism, and the remaining two
had normal calcium, phosphate and parathormone levels as did all the patients without intracranial calcification.
The absence of basal ganglia calcification
in our patient with both hypoparathyroidism and the Kearns-Sayre syndrome may be due to the early treatment
with calcium and vitamin D and the return
of the serum calcium and phosphate levels
to normal. This suggestion would be supported by the observation that intracranial
calcification does not occur in secondary
hypoparathyroidism due to previous thyroid surgery where treatment is started early
with the onset of tetany.3 The predilection
of the basal ganglia for calcification in the
recognised disorders associated with intracranial calcification may be due to disorders
of calcium metabolism, increased vascular
permeability, the preferential perfusion of
grey matter and the high rate of blood flow
to the basal ganglia. Furthermore alkaline
phosphatase activity may be regionally
elevated in the basal ganglia in patients
developing intracranial calcification.6
The early treatment of hypoparathyroidism in cases of Kearns-Sayre
syndrome is required to control the symptoms of hypocalcaemia and may prevent
basal ganglia calcification, as in our case.
Whether the calcification in the basal ganglia has a clinical consequence in this syndrome is uncertain and where it is found a
careful search for hypoparathyroidism
should be made.

References
Seigal RS, Seeger JF, Gabrielsen TO, et al. Computerised tomography in oculocraniosomatic
disease. (Kearns-Sayre syndrome). Radiology
1979;130: 159-64.
2Toppet M, Telerman-Toppet N, Szliwowski HB,
et al. Oculocraniosomatic neuromuscular disease with hypoparathyroidism. Am J Dis
Child 1977;131:437-41.
3Sachs C, Sjoberg HE, Ericson K. Basal ganglia
calcifications on CT: relation to hypoparathyroidism. Neurology 1982;32:779-82.
4Horwitz SJ, Roessmann U. Kearns Sayre syndrome with hypoparathyroidism. Ann Neurol
1978;3:51 3-8.
5Pellock JM, Behrens M, Lewis L, et al. Kearns
Sayre syndrome and hypoparathyroidism.
Ann Neurol 1978;3:445-58.
6Adachi M, Wellman KF, Volk BW. Histochemical studies on the pathogenesis of idiopathic non-arteriosclerotic cerebral calcification. J Neuropathol Exp Neurol 1968;23:
483-99.

Accepted 15 February 1986

Meige's syndrome and palatal myoclonus
associated with brain stem stroke. A common
mechanism?

Sir: Meige's syndrome (blepharospasm and
oro-facial dystonia), and palatal myoclonus
are two uncommon movement disorders
whose pathophysiology is poorly understood. Isolated reports have associated each
with focal brain stem lesions and have
suggested denervation hypersensitivity of
AG DEWHURST* brain stem nuclei as the underlying mechD HALLt anism.' -3 We report a patient in whoni
MS SCHWARTZ* both Meige's syndrome and palatal myoRO MCKERAN* clonus developed following upper brain
The Neurology Department, stem strokes. A related origin for these
Atkinson Morley's Hospital,* movement disorders is postulated.
Copse Hill, Wimbledon SW20 ONE,
A 78-year-old right-handed woman, who
and the Department of Child Health, was recovering from a stroke was first seen
St George's Hospital Medical because of involuntary facial movements.
School,j UK Five years previously she had experienced
the sudden onset of rotational vertigo, vomiting and loss of balance, resulting in falling.
A coarse action and intention tremor of the
right upper limb appeared after this episode,
causing difficulty with writing and bringing
food to the mouth. The tremor apparently
Epilepsy CTscan calcification remained unchanged over the next five years
Age of presentation of
Kearns-SaYre syndrome
but was totally abolished following the
recent stroke. Involuntary facial grimacing
Absent
Present
and intermittent forced eye closure had also
9
Absent
?
Present
been present for five years, being aggravated
11
Absent
by concentration or anxiety. Six weeks pre7
Present
Present
viously she developed sudden loss of speech
6
Absent
Present
(anarthria) with right hemiplegia. There was

Letters
some return of power in the right limbs and
dysarthric speech within 24 hours and slow
improvement occurred thereafter, so that
she could walk and dress with assistance.
There was a past history of maturity-onset
diabetes, hypertension and glaucoma and
her medications included prazosin, pindolol,
frusemide, tolbutamide and aspirin. Prochlorperazine had been prescribed for vertigo at the time of the first stroke but had
not been continued.
Examination showed a frail, elderly
woman with intermittent blepharospasm,
worsened by ocular examination, bilatera
grimacing movements of the face, and a
severe spastic dysarthria. The blood pressure was 140/70mmHg and general examination was normal. She was alert and cooperative and there was a full range of
ocular movements without nystagmus, but Fig CT scan performed six weeks after the
with saccadic intrusions on pursuit in all
stroke showing infarct on the left
directions of gaze. Continuous rhythmic second
palatal myoclonus at a rate of 165/min was side of the rostral pons (arrow).
seen on inspection of the oral cavity, and
tongue movements were slow. Facial and
palatal sensation were normal. The jaw jerk
Palatal myoclonus may be caused by a
was not exaggerated. There was a mild variety of lesions involving one limb of the
residual right hemiparesis involving the face, dentato-rubro-olivary (Guillain-Mollaret)
arm and leg, with hyper-reflexia and a triangle, including stroke, demyelination,
Babinski response on the right side. Dys- tumour, arteriovenous malformation,
metria and dysdiadochokinesis were present trauma, encephalitis and syphilis.7 8 A delay
in the left arm and leg. The gait was wide- of 2-49 months has been noted in the onset
based and hemiplegic, with a tendency to of the myoclonus after acute lesions sugfall to the right. Sensory testing showed no gesting that the mechanism of the myoabnormality. A cranial CT scan performed 6 clonus may involve the development of
weeks after the second stroke showed a non- denervation hypersensitivity in olivary neuenchancing low-density lesion on the left rons.3 An alternative mechanism postulates
side of the rostral pons (fig 1). Auditory release of olivary neurons from suprabrain stem evoked responses with monaural segmental inhibition.9 Meige's syndrome is
click stimulation showed attenuation of usually of idiopathic origin and insidious in
waves I-V with left ear stimulation, and onset, but there have been reports of
absence of wave III. With right ear stimu- blepharospasm, and in some cases associlation the I-V interval was prolonged.
ated facial dystonia, developing after uniThe initial episode of vertigo, ataxia and lateral ischaemic or demyelinative lesions of
right upper limb tremor is characteristic of the rostral brain stem or diencephalon.I 2 10
an infarct involving cerebellar nuclei or con- In such cases the onset of the involuntary
nections with probable involvement of the movements was usually delayed for several
,right dentato-rubral tract in the midbrain. months after the acute episode, again raising
The lesion demonstrated in the pons by CT the possibility of a denervation hyperafter the second stroke is likely to represent sensitivity of facial motor neurons or a
an infarct resulting from occlusion of one of release of facial motor neurons from suprathe perforating branches of the basilar nuclear inhibition. The association of the
artery and was appropriately placed to pro- two disorders, as in the present case, has
duce the right hemiparesis, left-sided cere- been reported only rarely2 5 and suggests
bellar signs and palatal myoclonus due to that similar pathophysiological mechanisms
involvement of the central tegmental tract. may be involved in the development of the
On the other hand, the myoclonus may have two involuntary movements.
developed after the first stroke and not have
The abolition of the right-sided tremor by
been noted. The blepharospasm and facial the subsequent development of an incomdystonia which developed after the first plete corticospinal tract lesion is of interest
stroke was typical of Meige's (or Brueghel's) and indicates that the corticospinal tract is
the final common pathway for the expressyndrome.4 -6

1325
sion of this involuntary movement. Parkinsonian tremor and dyskinesias resulting
from basal ganglion lesions may also be
abolished by lesions of the lateral corticospinal tract, usually with some degree of

resulting hemiparesis."

TJ DAY,
RB LEFROY,
FL MASTAGLIA,

University Department of Medicine,
Department of Neurology
and Extended Care Department,
Queen Elizabeth II Medical Centre,
Nedlands, WA 6009,
Australia
References
'Jankovic J, Patel SC. Blepharospasm associated
with brainstem lesions. Neurology (Cleve-

land) 1983;33:1237-40.
2Lang AE, Sharp JA. Blepharospasm associated
with palatal myoclonus. Neurology (Cleveland) 1984;34:1522.
3Matsuo F, Ajax ET. Palatal myoclonus and
denervation supersensitivity in the central
nervous system. Ann Neurol 1979;5:72-78.
'Meige H. Les convulsions de la face: une forme
clinique de convulsion faciale, bilaterale et
mediane. Rev Neurol (Paris) 1910;2:437-43.
'Jankovic J, Ford J. Blepharospasm and
orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. Ann
Neurol 1983;13:402-1 1.
6Marsden CD. Blepharospasm-oromandibular
dystonia syndrome (Brueghel's syndrome). J
Neurol Neurosurg Psychiatry 1976;39:1204-9.
'Nathanson M. Palatal myoclonus. Arch Neurol
Psychiatry 1956;75:285-96.
8Herrmann C, Crandall PH, Fang HCH. Palatal
myoclonus. Neurology (Minneap) 1957;7:
37-51.
9Herrmann C, Brown JW. Palatal myoclonus: a
reappraisal. J Neurol Sci 1967;5:473-92.
'°Powers JM. Blepharospasm due to unilateral
diencephalon infarction. Neurology (Cleve-

land) 1985;35:283-4.
"Adams RD, Victor M. Tremor, myoclonus,
spasm and tics. In: Principles of Neurology.
McGraw-Hill 1977:69-79.
Accepted 8 August 1985