Journal of

raogy

J,Neurol. 221, 127--131 (1979)

~) by Springer-Verlag 1979

Short Communications
Recurrent Transient Global Amnesia
in a Case with Cerebrovascular Lesions
and Livedo Reticularis (Sneddon Syndrome)
E. Rumpl and H. Rumpl
Departments of Neurology and Dermatology, University of Innsbruck, Austria

Summary. Eight attacks of transient global amnesia were observed in a female
patient who suffered from livedo reticularis and a series of other neurological
symptoms, which were transient in most stances. The neurological deficits
include focal epileptic attacks, unilateral loss of vision, paresis of left arm
a n d / o r leg and dysarthria. The first amnestic attack was seen at the age of 19.
The episodes lasted from a few to 3 days. The intervals between the amnestic
episodes varied between a few days and 11 years. The livedo reticularis
became more obvious during each neurological episode and was less pronounced during the time of remission. A benign type of essential hypertension
and paraproteinemia (gamma-M) was found. The investigations failed to
show any evidence of essential thrombocythemia, polyarteriitis nodosa, lupus
erythematodes and other immune complex diseases. The underlaying disease
remained unclear.
Key words: Transient global amnesia - Livedo reticularis - Cerebrovascular
lesions.
Zusammenfassung. Acht Episoden einer transitorischen globalen Amnesic
wurden bei einer Patientin beobachtet, bei der eine Livedo reticularis auch mit
einer Reihe anderer, meist reversibler neurologischer Ausf~ille verbunden war.
Diese setzten sich aus fokalen epileptischen Anf'~illen, einseitigem Visusverlust,
Paresen der linken oberen u n d / o d e r unteren Extremit~t und dysarthrischen
St6rungen zusammen. Die erste amnestische Episode trat im Alter yon
19 Jahren auf. Die Episoden hielten meist nur wenige Stunden, einmal aber
auch drei Tage an. Die Zeitabst~inde zwischen den einzelnen Attacken lagen
zwischen wenigen Tagen und 11 Jahren. Die Auspr~igung der Livedo reticularis
verst~irkte sich in den Perioden mit neurologischen Ausf~llen und nahm in den
Riickbildungsphasen ab. Die Laboruntersuchungen ergaben eine benigne
Form einer essentiallen Hypertonie und Paraproteinamie (Gamma-M). Hinweise ftir eine essentielle Thrombozyt~imie, eine Polyarteriitis nodosa, einen
Lupus erythematodes oder ftir andere Immunkomplex-Erkrankungen waren
nicht zu erheben. Die Ursache der Symptomenkombination blieb ungekl/irt.
Address for offprint requests: Dr. E. Rumpl, Univ.-Klinik ftir Neurologie, Anichstr. 35, A-6020

Innsbruck, Osterreich

0340-5354/79/0221/0127/$ 01.00

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E. Rumpl and H. Rumpl

In 1956 B e n d e r [1] d e s c r i b e d t h e s y n d r o m e o f t e m p o r a r y a m n e s i a t e r m e d by
F i s h e r a n d A d a m s [3] t r a n s i e n t g l o b a l a m n e s i a ( T G A ) . I n its t y p i c a l f o r m T G A is
c h a r a c t e r i z e d by a s u d d e n loss o f r e c e n t m e m o r y , a s s o c i a t e d w i t h r e t r o g r a d e
a m n e s i a f o r e v e n t s w h i c h h a v e t a k e n p l a c e s o m e d a y s to s o m e y e a r s b e f o r e the
a m n e s t i c e p i s o d e . T h e a t t a c k u s u a l l y lasts f o r h o u r s , r e c o v e r s c o m p l e t e l y , b u t
t o t a l a m n e s i a r e m a i n s f o r e v e n t s d u r i n g the e p i s o d e . N u m e r o u s r e p o r t s h a v e b e e n
p u b l i s h e d o n this t h e m e , m o s t o f t h e m r e v i e w e d by F o g e l h o l m et al. [4].
E x t e n s i v e l i v e d o r e t i c u l a r i s has b e e n o b s e r v e d o n the skin o f p a t i e n t s w h o
s u f f e r e d f r o m a series o f c e r e b r o v a s c u l a r lesions. T h e c e r e b r a l i n c i d e n t s w e r e o f
l i m i t e d a n d b e n i g n n a t u r e , o f t e n l e a v i n g n o o r little r e s i d u a l disability. S n e d d o n
[11] first f o u n d t h a t this f o r m o f l i v e d o was r e l a t e d to c e r e b r o v a s c u l a r lesions.

Case Report
A housewife aged 50 dated the onset of neurological deficits to the age of 16. There was a sudden
attack of weakness of the left arm and leg with spontaneous and complete improvement within a
few days. Three years after the initial incident, and immediately after the birth of her first child,
the patient suffered from an acute loss of recent memory lasting for 12 h. She appeared anxious
and helpless. A steady repitition of questions indicated a remarkable loss of recent memory
function. Retrograde amnesia, initially starting 2 days before birth shrunk gradually during the
subsequent hours and remained only between the onset of the attack and full recovery. At the
same time the patient developed livedo reticularis of the limbs. During the next 4 years there
were no neurological deficits and no signs of livedo. After an uncomplicated birth at the age of
23 the patient suffered from four attacks of TGA within 3 months. Each of these episodes lasted
a few h. She also had transient loss of vision not time-locked to the TGAs at the same period. A
widespread livedo reticularis had developed on the limbs, on the thighs and on the face. The
patient noticed a more marked livedo during her attacks. There were two other TGAs in the
next 3 years. After these episodes the livedo remained constant and there were no neurological
deficits in the next 11 years.
At the age of 37 the patient was delivered of a third child. Three months after birth another
amnestic episode occurred lasting for 3 days. During this period the patient already had insight
into her state and made an exact plan to handle the baby. Duties were registered and marked
when done. One year later the patient had a focal epileptic attack lasting a few minutes which
involved the left hand with clonic jerks. After an interval of 5 years she suddenly developed
paresis of the left arm accompanied by left central facial palsy. Marked dysarthria was observed.
There was complete recovery of the neurological deficits with the exception of a slight dysarthric
speech.Three years later another focal epileptic attack involved the left leg and led to a transient
postictal paresis. Several other short focal attacks of the left arm were heralded by clonic jerks
or by paresthesia. Weakness of the left extremities developed after an initial state of focal clonic
jerks at the age of 48. There was some remission during the next 10h, but a residual paresis of
the left extremities persisted. One year later the patient had a short episode of TGA with
complete insight about the condition. She carried on at work as usual. Being afraid of a long
duration of the attack she noted the intake of her medicaments. During each period of
neurological deficits the livedo became more obvious. At the age of 50 the patient had bilateral
reduction of visual acuity, later visual loss of the left eye. At this time the patient was admitted
to the neurological clinic.

Examinations
N e u r o l o g i c a l e x a m i n a t i o n r e v e a l e d h o r i z o n t a l n y s t a g m u s seen o n l o o k i n g to r i g h t
a n d left, r e s i d u a l signs o f c e n t r a l facial palsy, d e v i a t i o n o f the t o n g u e to the left o n
p r o t r u s i o n , d y s a r t h r i c s p e e c h ; spasticity, a t a x i a a n d d i s t u r b a n c e o f f i n e r m o v e -

Fig. 1. Local atrophy in territory of right middle cerebral artery on CT scan. No correlation
between T G A and this lesion, because of other neurological symptoms located in same area

Fig. 2. Widespread purplish mottled reticular livedo on arms (left), buttocks and thinghs (right)

130

E. Rumpl and H. Rumpl

ments of the left extremities, deviation to the left side on walking. Further
investigation including right carotid angiogram, CSF protein, cells and WR, and
EEG were normal. The CT scan revealed local atrophy in the territory of the right
middle cerebral artery. No abnormalities were'seen in the brainstem (Fig. 1).
The dermatological findings were a widespread purplish mottled reticular
livedo symmetrically developed on the arms, buttocks, things and legs (Fig. 2),
and a butterfly-like form of livedo on the face. The histological changes of the skin
biopsy were uncharacteristic. No vascular changes of significance were noted.
General medical investigations revealed transient essential hypertension with
elevated diastolic blood pressure and a benign type of paraproteinemia (gammaM). Laboratory tests include normal hematocrit, ESR, blood cell counts,
prothrombin and partial thromboplastin time, electrophoresis, immunglobulins,
thyroxine, T4 test, renin plasma activity catecholamines (urinary excretion), CPK,
LDH, SGOT, SGPT, gamma-GPT, amylase, alkaline phosphatase, elektrolytes,
creatinin, uric acid and urea nitrogen. Serum antinuclear antibodies, serum
antibodies to smooth and strial muscle and LE cell test were negative. The
rheumatoid factors were positive at admission, negative in a serie of controls. The
agarose-gel electrophoresis revealed an uncharacteristic M-protein. The electrocardiogram and X-ray of the chest showed slight signs of left ventricular
hypertrophy. The creatinine clearance was slightly decreased. The renal plasma
flow (Cpah) was normal. There were signs of chronic constriction of the right
ureter demonstrated by the intravenous pyelogram.

Discussion

Most authors have supported the vascular theory for the genesis of TGA,
especially as a result of transient cerebral ischemia [1,4, 8, 9]. The short duration
of most of the attacks of our patient and the complete or nearly complete recovery
of the neurological deficits within hours to a few days confirmed this view. The
neurological signs of our patients suggested lesions of the internal carotid and
basilar artery territory. Although the right carotid angiogram showed no
abnormalities, an otherwise normal CT scan demonstrated local atrophy in an
area supplied by the right middle cerebral artery. However, any correlation
between T G A and this atrophy is hampered by the other neurological symptoms,
which can also be localized in this cerebral region.
Recurrent attacks of TGA are rather uncommon [6, 8, 9]. Our patient had eight
amnestic episodes with an interval from a few days to 11 years. Only few authors
[6, 8] have seen episodes longer than 3 days. The onset of the first TGA at the age
of 19 was a further uncommon event. Usually the syndrome appeared in patients
in the fifth to seventh decade of life [3, 6, 8,9]. The significance of livedo was
primarily overlooked by neurologists. The age of the patient and the "typical"
varied clinical course with remissions and relapses led to the diagnosis of multiple
sclerosis, although this diagnosis never was confirmed by characteristic changes
in the cerebrospinal fluid.
A relationship of livedo and cerebrovascular lesions was demonstrated in
several singular patients [2, 5, 7, 10]. Sneddon [11] first pointed out that "there is

Recurrent Transient Global Amnesia

131

sufficient evidence to link unusual cerebral incidents with the skin livedo". This
association seemed to be as c o m m o n as livedo reticularis in polyarteriitis nodosa,
disseminated lupus erythematodes and essential t h r o m b o c y t h e m i a . Most of these
patients were y o u n g adults and had persistent, more often transient neurological
deficits. These deficits were classified as sudden visual loss, h o m o n y m o u s
hemianopia, hemiparesis and hemiplegia, aphasia, asterognosia and dysarthria.
N o s y m p t o m s comparable to T G A were described in these cases. A more obvious
livedo at the time o f neurological deficits was f o u n d in most cases [2, 7, 11].
The clinical findings in our patient corresponded on the whole to the observations of Sneddon [11]. The most remarkable finding was a moderate and
benign type of hypertension with no renal change of significance. There was
exclusion o f essential t h r o m b o c y t h e m i a , of disseminated lupus erythematodes, of
polyarteriitis n o d o s a and other well-known immune complex diseases including
different forms o f vasculitis. The underlying disease o f our patient remained
unclear. Therefore therapy consisting o f cerebral vasodilators and beta-adrenergic
blocking agents was a s y m p t o m a t i c one.
Livedo reticularis o f the degree demonstrated by our patient is extremely
u n c o m m o n . H o w e v e r the livedo m a y not be marked during the time o f remission
from neurological s y m p t o m s and therefore m a y be overlooked by neurologists.
Especially in y o u n g patients with sudden development and remission o f different
focal cerebral lesions, with a benign type o f hypertension and skin lesions the
s y n d r o m e of cerebrovascular lesions and livedo reticularis (Sneddon syndrome)
should be considered in the differential diagnosis.

References
1. Bender, M. B.: Syndrome of isolated episode of confusion with amnesia. J. Hillside Hosp. 5,
212--215 (1956)
2. Church, R. E.: Reticular livedo with cerebro-vascular lesions. Brit. J. Derm. 74, 156--157
(1962)
3. Fisher, C. M., Adams, R. D.: Transient global amnesia. Trans. Amer. Neurol. Ass. 83,
143--146 (1958)
4. Fogelholm, R., Kivalo, E., Bergstr6m, L.: The transient global amnesia syndrome. Eur.
Neurol. 13, 72--85 (1975)
5. Fuhs, H.: Livedo racemosa. Zbl. Haut- u. Geschl.krkh. 31,290p (1929)
6. Heathfield, K. W. G., Croft, P. B., Swash, M.: The syndrome of transient global amnesia.
Brain 96, 729--736 (1973)
7. Kimmig, J.: 5. Arteriolopathie: Livedo racemosa. Dermatol. Wschr. 139, 211p (1959)
8. Mumenthaler, M., von Roll, L.: Amnestische Episoden; Analyse von 16 eigenen Beobachtungen. Schweiz. med. Wschr. 99, 133--139 (1969)
9. Shuttleworth, E. C., Morris, C. E.: The transient global amnesia syndrome. Arch. Neurol.
(Chicago) 15, 515--520 (1966)
10. Smelov: Livedo racemosa. Zbl. Haut- u. Geschlechts.krkh. 26, 37p (1920)
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(1965)
Received March 30, 1979