Case Report

Kaleidoscopic vision and a jerking leg on the ski slope
Stefan Kiechl, Martin Furtner, Michael Knoflach, Philipp Werner, Johann Willeit
Lancet 2007; 370: 1878
Department of Neurology,
Medical University Innsbruck,
Austria (S Kiechl MD,
M Furtner MD, M Knoflach MD,
P Werner MD, J Willeit MD)
Correspondence to:
Dr Stefan Kiechl, Department of
Neurology, Medical University
Innsbruck, Anichstraβe 35,
A–6020 Innsbruck, Austria
stefan.kiechl@i-med.ac.at

A

In March, 2005, a 48-year-old woman was flown to our
hospital by rescue helicopter after she fell while skiing.
Shortly before she fell, she started to see objects in a
distorted or fragmented way, and to see geometric shapes.
The images of the objects and shapes were constantly
changing, and she described her experience as like
“looking through a kaleidoscope”. She closed her eyes in
turn: vision in her right eye was normal; vision in her left
eye was monochrome, fragmented, and changing. Her
right leg started to jerk briskly and irregularly, causing her
to fall. She did not lose consciousness; nor was she hurt.
She had had migraine attacks, without an aura, for more
than 30 years, but had had no attacks for several weeks.
She had no risk factors for epilepsy. Nonetheless, the emergency physicians provisionally diagnosed a partial
seizure.
The patient was right-handed. Blood pressure was
normal, at 140/90 mm Hg, as was visual acuity. However,
on standing, the patient had vertigo; within seconds, her
right arm and leg started to jerk repeatedly. The jerking
promptly ceased when she lay down. Fundoscopy and
gazing into the sun caused her vision to be as though of a
picture with poor resolution, or one consisting of fine
granules; she would then go blind in her left eye. Vision
returned to normal once the patient stopped gazing at
bright lights. MRI, magnetic-resonance angiography
(MRA), and high-resolution ultrasonography revealed a
high-grade (>90%) stenosis of the left carotid artery, caused
by dissection of the artery wall (figure). Ultrasonography
also showed significant impairment of blood flow in the
left middle cerebral artery, with a loss of autoregulation.
There was evidence of collateral blood supply via the
B

Figure: Carotid artery dissection
(A) MRA, showing a string-like stenosis caused by dissection of the left internal carotid artery (arrow). (B) MRI with
T1 weighting, showing a semilunar area of hyperintensity, typical of haematoma, in the artery wall (arrowhead).

1878

ophthalmic artery, leptomeningeal pathways, and anterior
and posterior communicating arteries. The patient was
given a plasma expander (hydroxyethyl starch) and
norepinephrine to raise her mean arterial pressure above
100 mm Hg, and heparin to reduce the risk of clotting. She
was also told to remain lying in bed, and to wear sunglasses. Her symptoms resolved within 48 h, but recurred
4 days after admission; additionally, she now had brief
episodes of aphasia and right-sided hemiparesis. Repeat
MRI and MRA showed that the wall haematoma had
expanded, so the left carotid artery was now blocked. MRI
also showed two pinhead-sized diffusion abnormalities,
implying small strokes, in the left frontal lobe. Transcranial duplex ultrasonography showed that mean flow velocity in the left middle cerebral artery was now just 60% of
the velocity on the right. We considered placing a stent in
the carotid artery; however, the patient’s condition rapidly
improved, through an increase in collateral blood flow.
10 days after admission, treatment with hydroxyethyl
starch and norepinephrine was discontinued; 4 days later,
heparin was replaced by warfarin, which we prescribed
for 12 months. On further assessment, we found no evidence of fibromuscular dysplasia, polycystic kidney
disease, Ehlers-Danlos syndrome, or Marfan’s syndrome,
all illnesses associated with arterial dissection. When last
seen, in March, 2006, the patient was well.
Although the unilateral limb jerks seemed to signify
epilepsy, the postural onset of clinical signs indicated
that the illness was probably vascular. Low-flow transient
ischaemic attacks (TIAs) can cause limbs to swing, jerk,
or tremble.1 Such movements are nearly always unilateral;
the face is generally unaffected.1 These attacks are
ascribed to a focal low-perfusion state in the carotid artery
territory, and loss of autoregulation.1,2 Similarly, our
patient’s visual symptoms were caused by a vascular
mechanism. Retinal ischaemia delayed regeneration of
visual pigments, leading to kaleidoscopic vision and
blindness. Transient painless amaurosis precipitated by
exposure to bright light is known as retinal claudication.3,4
Retinal claudication and TIAs with limb-shaking are
thought to occur in 3% and 8%, respectively, of patients
with carotid artery occlusion.5
References
1
Baquis GD, Pessin MS, Scott RM. Limb shaking—a carotid TIA.
Stroke 1985; 16: 444–48.
2
Fisher CM. Concerning recurrent transient cerebral ischemic
attacks. Can Med Assoc J 1962; 86: 1091–99.
3
Furlan AJ, Whisnant JP, Kearns TP. Unilateral visual loss in bright
light. An unusual symptom of carotid artery occlusive disease.
Arch Neurol 1979; 36: 675–76.
4
Kaiboriboon K, Piriyawat P, Selhorst JB. Light-induced amaurosis
fugax. Am J Ophthalmol 2001; 131: 674–76.
5
Klijn CJ, Kappelle LJ, van Huffelen AC, et al. Recurrent ischemia in
symptomatic carotid occlusion: prognostic value of hemodynamic
factors. Neurology 2000; 55: 1806–12.

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