Letters

1073

prophyla Lxis was given and on conservative

managenr nent with antibiotics and
dexametl hasone the left hemiplegia and the
horizontaal gaze palsy rapidly resolved.

8 Schneider RC, Henderson JW. Penetrating
orbital

wound

with

intracranial

complications. Arch Ophthalmol 1952;47:815.

After se.veral weeks an efferent pupil 9 Svoboda J. Injury of orbit and base of skull with
fatal termination. Ceskoslovenska oftalresponse could be elicited on the right side,
mologie 1948:72-4. Abstracted Ophth Lit
corneal sensation was restored, and
1948,2:422.
consideraable improvement was seen in the °Watson
PG, Holt-Wilson AD. The traumatic
Figure (a) The fencing foil responsible for external ophthalmoplegia. Loss of vision in
orbital apex syndrome, its differential
the right eye was permanent and probably
injury. (b) Enlarged view of the tip of the
diagnosis and treatment. Trans Ophthalmol
Soc UK 1968;88:361-74.
resulted from swelling of the optic nerve
foil.
Evans JP, Scheinker IM. Histologic studies of
within th e confines of the bony optic canal
brain following head trauma: post-traumatic
though the globe was intact and the following contusion.'° " Within a month
cerebral swelling and edema. J Neurosurg
the
c
unbroken.
optic
disc pallor was apparent. Permanent
upper eyelid
1945;2:306-14.
Proptosis was noted but there was no damage tto cerebellar pathways resulted in
2 Larmande A, Descuns P, Margaillan A. Wound
an Id horizontal nystagmus which did
pulsation and a bruit could not
of the orbit with homolateral hemiplegia in a
detected.
The
fencer. Rev Otoneuroophthalmol 1956;28:
right pupil exhibited not resolvve.
It is eviident that the foil entered the orbit
440-1.
complete afferent and efferent defects in
Platt ES. Orbitocranial penetrating injuries. Am
response to light and failed to constrict through the upper conjunctival fornix
J Ophthalmol 1954;37:758-63.
accommodation. The presence in addiproducing g very little external sign of injury.
right-sided ptosis, total external It traversied the orbit and, taking a course
ophthalmoplegia, and corneal anaesthesia determinted by the downward slope of the
suggested a diagnosis of traumatic orbital orbital rcaof, passed through the superior Focal dystonia in association with cerebral
on
the
basis
of orbital fiissure and on, lateral to the
apex
syndrome'0
infarction
the right eye alone. Fundus
cavernou iS sinus, before coming to rest in
examination revealed no defect of
perfusion the regiorn of the pons and right cerebellar Sir: We wish to report the case of a woman
right optic nerve head. Examination hemisphe-re. To date there are few reports who developed bradykinesia and rigidity of
the
left eye at this
stage produced of hem iplegia following transorbital her left arm, and subsequently, a proevidence of intracranial
penetration. The penetratirng injury,3 '213 and so far as is gressive fixed dystonic posture of her left
visual acuity was normal and the
pupil known th e finding of a gaze palsy in such an hand in association with an infarct in the
briskly to light and accommodation. injury haIs not previously been reported. region of the right basal ganglia.
left eye could not be adducted
beyond The posssibility of intracranial extension
In 1974, when 43 years old, the patient
midline
when
the
stimulus
was must alwvays be considered in cases of
with a six month history of propresented
command
or
pursuit, optokinetic, or orbital irnjury. It is wise to admit all gressive weakness of the left hand, with
vestibular
but
adduction
occurred on potential transorbital injuries to hospital for
difficulty in doing up buttons or manipulatattempted convergence. Although the observaticon and the importance of a ing a fork. There was no family history of
complete external ophthalmoplegia affect- careful ne:uro-ophthalmological assessment consanguinity or of neurological disease
ing the* right eye prevented full testing at the initial
i
examination cannot be and the patient's previous health had been
conjugate movements, these findings overstate( d.2
JOHN HUNGERFORD excellent. On examination there was a mild
were considered indicative of a horizontal
University
College Hospital, resting tremor of the left hand, with cogfrom
gaze palsy resulting
damage to the
Gower Street, wheel rigidity at the elbow and wrist, and
supranuclear horizontal gaze centre in the
London,
WC] fine finger movements of the left hand were
pons
adjacent longitudinal bundle. References
moderately impaired. The blood pressure
left fundus was normal. The
patient was
was 230/140 mm Hg.
to
Kjer P. Orbital and transorbital stab wounds.
hospital.
Neurological
An initial diagnosis of Parkinson's
examination at this
Arch
stage demonstrated no
O)phthaInol 1954;51:811-21.
disease was made and the hypertension
I LA,
Jarrett WH.
lntracranial treated, first with methyldopa and later proabnormality other than those already
ications of penetrating orbital injuries. pranolol. No anti-Parkinsonian
noted.
Radiological examination of the
compli
therapy was
phthalmol 1964;71:332-43.
skull, orbits, and optic foramine was normal
C
given and after a year the tremor remitted,
MH,
Baker
Henderson
GS,
JW.
particular there was no evidence of
mnjurie s of the brain caused
by penetration of but there was a slight increase in the rigidity
the region of the
damage
right superior
the orb it. Minn Med 1959;42:1408-13.
and bradykinesia in the left arm. The latter
fissure.
A CT scan showed the
4McClure CC, Jr, Gardner WJ. Transorbital failed to respond to trials of levodopa. brothe

blind

of

skin

evidence of

ataxia

be

the

on

tion

of

examination of

of the
of

reacted

The

the

of

or to the

The

admitted

Bard

and

in

in

orbital

proptosis
Within

observations
of

hours

2

developed

only

the

as

Neurological
a

left-sided

abnormality.
begun.

were

admission

there

hemiplegia and a

showed attenuation in the

further CT scan

region of the brain stem. The absence of a
carotico-cavernous

confirmed
considered to have

fistula

the

signs

were

resulted

from

oedema

and

compression

by

was

rather

than

haemorrhage.

from

Tetanus

intracraanial

stab wounds. Cleve Clin Q

1949;166:118-25.
Guthkelclh AN. Apparently trivial wounds of
eye lids with intracranial damage. Br Med
the

Duff60 0:842-4.
GP, Bhandari
compliccations

YS.

following

Intracranial
transorbital

penetraating injuries. Br JSurg 1969;56:685-8.

7Webster JE, Schneider RC, Lofstrom JE.
Observations upon management of orbitocranial

wounds. J Neurosurg 1946;3:329-36.

mocryptine or anticholinergic treatment

which was tried over the following two
years. In 1977 the patient first developed an
abnormal posture of the left hand and this
gradually increased during the next three
years. On examination at the end of 1981
she was normotensive and her left hand was
now permanently maintained in a dystonic
posture (fig A). The medial three fingers
were flexed at the metacarpo-phala'ngeal

Letters

1074

4Denny-Brown D. Clinical symptomatology of
basal ganglia disease. In: Handbook of Clinical Neurology Vol 6. Vinken PJ, Bruyn GW
eds, Amstcrdam: North Holland Publishing
Company, 1968.
5 Messimy R, Diebler C, Metzger J. Dystonia de
torsion du membre superieur gauche probablenment a un traumatisme cranien. Rev
Neurol (Paris) 1977;133:199-206.
6 Lobo-Antunes J, Yahr MD, Hilal SK. Extrapyramidal dysfunction with cerebral arteriovenous malformations. J Neurol Neurosurg
Psychiatry 1974;37:259-68.

F 1:!

A6

Association of Klippel-Trenaunay-Weber
syndrome with myotonic dystrophy

joint, which could not be extended beyond
450 and the interphalangeal joints were
hyperextended and could not be flexed passively beyond 20°. Movements of the thumb
and forefinger were normal. There was mild
rigidity at the left wrist and elbow. There
were no other neurological signs. Routine
blood tests includin,g copper studies were
normal as was an EEG. A CT scan (fig B)
demonstrated mild right-sided cerebral
atrophy and a low attenuation lesion in the
region of the right lentiform nucleus compatible with a mature infarct. There was no
clinical event suggestive of a stroke, but the
scan appearance and the history of hypertension were in keeping with a silent infarction involving the basal ganglia on the right
side. Although the time of onset of the infarction is not known, a delay between a
cerebral insult and the onset of dystonia has
been described by other authors. 12 Dooling
and Adams3 have observed that posthemiplegic athetosis only occurs with basal
ganglia lesions when the cortico-spinal
tracts are at least partially intact, but following a stroke the development of such a syn-

is flexed and pronated with the hand open,
but after some years the fingers may
become tightly clenched and the elbow
flexed. Whilst our patient exhibited rigidity
at the left elbow and wrist, her abnormal
posture was restricted to three fingers. In
the absence of the CT scan findings, her
hand posture might have been regarded as
functional.
M TRAUB, A RIDLEY

Department of Neurology,
The London Hospital,
Whitechapel, London
IE IBB, UK
References

Burke RE, Fahn S, Gold AP. Delayed onset

dystonia in patients with static encephalo-

pathy. J Neurol Neurosurg Psychiatry
1980;43:789-97.
2 Brett EM, Hoare RD, Sheehy MP, Marsden
CD. Progressive hemidystonia due to focal
head trauma. J
after mild 1981;44:460.
lesionPsychiatry
basal
Neurosurg
Neurolganglia
3 Dooling EC, Adams RD. The pathological
ana-

tomy

of posthemiplegic athetosis.

1975;98:29-48.

Brain

Sir: Myotonic dystrophy, an inherited
disorder, is associated with cataract, frontal
baldness, cardiac dysfunction, testicular
atrophy, abnormal insulin secretion,
Klinefelter syndrome, and Down's syndrome, in addition to central and peripheral
nervous sytem dysfunction. Cutaneous
manifestations, other than balding, are
rare. Few cases of pilomatrixoma (a benign
calcifying epithelioma) have been reported
in myotonic dystrophy.1 We observed an
extensive systematised vascular naevus in a
segmental distribution with hypertrophy
affecting one upper limb, the characteristic
features of Klippel-Trenaunay-Weber syndrome, in a patient with myotonic dystrophy. An exhaustive review of the
literature has failed to disclose similar cases.
A 39-year-old male Muslim, an
agriculturist, born of a consanguineous
union was seen in December 1980, with
complaints of weakness of lower limbs of 2
yr duration. He had most of the cardinal
signs of myotonic dystrophy such as myotonia, frontal balding, testicular atrophy,
posterior capsular cataract, wasting and
weakness of temporalis, masseter, sternocleido-mastoid muscles of the forearm,
hands and feet (fig). Electromyography
showed well sustained spontaneous, high
frequency discharges, myopathic pattern
and nerve conduction studies showed normal motor and sensory conduction velocity
in the upper and lower limbs. A cutaneous
patch on the right side of the chest and
medial aspect of the arm was noticed since
birth. This was dull red in colour in the
distribution of thoracic 1 and 2 segments
(fig). Global hypertrophy of the right arm
including the shoulder was present. No venous varicosities were seen nor was there any
evidence of associated disorders like syndactyly, polydactyly, scoliosis, pulmonary
hypertension, mental retardation, epilepsy
or paraplegia.