evaluations as the narcolepsy group, including polysomnography
and MSLT. Nevertheless, with any of the other neurologic disorders, a concomitant diagnosis of narcolepsy is relatively improbable based on their overall clinical presentations. Low CSF
hypocretin levels were consistently found in all narcoleptic subjects. In addition, these levels were occasionally found prior to
classic narcoleptic signs and symptoms. There may be a true,
relatively independent diagnostic utility in measuring CSF hypocretin levels when narcolepsy is suspected in children.
From the Department of Pediatrics (Dr. Arii), Chiba Rosai Hospital, and
Division of Neurology (Dr. Tanabe), Chiba Children’s Hospital, Departments
of Neuropsychiatry (Drs. Kanbayashi, Mishima, Hishikawa, and Shimizu)
and Pediatrics (Dr. Sawaishi), Akita University School of Medicine, Department of Pediatrics (Dr. Kimura), Akita Red Cross Hospital, and Department
of Pediatrics (Dr. Watanabe), Akita Nakadori General Hospital, Japan; and
Center for Narcolepsy (Dr. Nishino), Stanford University, Palo Alto, CA.
Received July 18, 2003. Accepted in final form July 12, 2004.
Address correspondence and reprint requests to Dr. J. Arii, Department of
Pediatrics, Chiba Rosai Hospital, 2-16 Tatsumidai-Higashi, Ichihara-shi,
Chiba 290-0003, Japan; e-mail: junko-a@muf.biglobe.ne.jp

Aortic dissection presenting with transient
global amnesia-like symptoms
C. Gaul, MD; W. Dietrich, MD; B. Tomandl, PhD;
B. Neundörfer, PhD; and F.J. Erbguth, PhD
Diagnostic criteria of transient global amnesia (TGA) are witnessed attacks, clear-cut anterograde amnesia during the attack,
absent clouding of consciousness and loss of personal identity, no
accompanying focal neurologic symptoms or epileptic features,
resolution of attacks within 24 hours, and no recent head injury or
active epilepsy.1 For the etiology of TGA, four main hypotheses
have been considered: TIA, epilepsy, migraine, and transient venous ischemia.1-3 None of these hypotheses fully explains the
mechanism of this episodic disease, but the accepted neuroanatomic correlate of TGA is the mediobasal temporal lobe and hippocampus. We present two patients with aortic dissection who
provide evidence for an ischemic pathogenesis in TGA.
Case reports. Patient 1. A 47-year-old man was found confused and disoriented. On examination at admission, he asked
repetitive questions and was alert but completely disoriented to
time and place and only partially oriented to person. The cranial
nerve examination showed only a slight anisocoria. The pronator
drift test revealed a discrete motor deficit of the left side, accompanied by a mildly increased reflex activity. The cranial CT was
unremarkable. The EEG revealed no epileptic discharges. Some
hours later, the patient was reoriented with an amnestic gap for
the attack’s duration, and the neurologic deficit had completely
resolved. Because of persistent hypotension, a chest radiograph
was taken, which revealed a widening of the mediastinum. A CT
scan of the chest and abdomen revealed a dissecting aneurysm
(Stanford type A) of the aortic arch starting at the aortic valve,
involving both carotid arteries and the left subclavian artery, and
continuing into both iliac arteries (figure).
Patient 2. A 61-year-old woman was taken to hospital because of acute chest pain. On admission, she had retrograde amnesia for the past few hours, anterograde amnesia with inability
to learn new facts, and repetitious questioning. Neurologic examination revealed a mild right facial paresis. Cranial CT was unremarkable. Five hours after onset of symptoms, she was reoriented
with an amnestic gap. Because of the initial thoracal pain and our
knowledge of the first reported patient, an aortic dissection was
considered. Chest radiograph was normal, but a CT scan of the
chest and abdomen revealed a dissection of the aorta (Stanford
type A) starting at the aortic valve, involving all supra-aortal
branches, and ending above the left renal artery.
Discussion. Patients with TGA can be distinguished into
three groups: “pure TGA” patients who fulfill all diagnostic criteria; patients with probable epileptic amnesia; and patients with
probable transient ischemic amnesia. The third group includes
patients with additional neurologic deficits during the attack as in
our patients.1 Although we found no proof of ischemic lesions in
the cranial CT in our patients, the minor neurologic deficits suggested cerebral ischemia. MRI including diffusion-weighted MRI
2442

NEUROLOGY 63

December (2 of 2) 2004

Copyright © 2004 by AAN Enterprises, Inc.

References
1. Guilleminault C, Pelayo R. Narcolepsy in prepubertal children. Ann
Neurol 1998;43:135–142.
2. Palm L, Persson E, Elmqvist D, Blennow G. Sleep and wakefulness in
normal preadolescent children. Sleep 1989;12:299 –308.
3. Nishino S, Ripley B, Overeem S, Lammers GJ, Mignot E. Hypocretin
(orexin) deficiency in human narcolepsy. Lancet 2000;355:39 – 40.
4. Ripley B, Overeem S, Fujiki N, et al. CSF hypocretin/orexin levels in
narcolepsy and other neurological conditions. Neurology 2001;57:2253–
2258.
5. Mignot E, Lammers GJ, Ripley B, et al. The role of cerebrospinal fluid
hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol 2002;59:1553–1562.
6. American Academy of Sleep Medicine. International Classification of
Sleep Disorders, rev.: diagnostic and coding manual. Rochester, MN:
American Academy of Sleep Medicine, 2001.
7. Kanbayashi T, Yano T, Ishiguro H, et al. Hypocretin-1 (orexin-A) levels
in human lumbar CSF in different age groups: infants to elderly persons.
Sleep 2002;25:337–339.

(DWI) would have been helpful to characterize the etiology, but
both patients underwent immediate surgery without possibility
for further diagnostics. We presume that an aortic dissection can
cause a TGA subtype with ischemic etiology, which can be called
transient ischemic amnesia. The underlying mechanism may be
an embolic vascular occlusion in the posterior circulation, thus
causing an embolic TIA with an unusual TGA-like TIA syndrome.
The ischemic hypothesis in TGA was enforced by bitemporal hypoperfusion found in brain SPECT.4 However, patients with TGA
have fewer thromboembolic risk factors and smaller risk of cerebral infarction compared with those with TIA.1 The DWI findings
provide conflicting results concerning a possible ischemic mechanism.5 In one study using DWI in 10 patients with TGA, 7 patients showed an elevated signal intensity in the left or in both
temporomesial regions. This was interpreted as a hint of the possible etiologic role of spreading depression.6 One-third of patients
with TGA also have migraine. The low recurrence rate of ~8% in
TGA and the different age distribution are arguments against
migraine as a pathogenic mechanism.1 The weakest evidence is

Figure. Multiplanar reconstructions from spiral CT: multiple dissection membranes within the aortic arch are
demonstrated with involvement of the supra-aortic
branches (arrows).

found for an epileptic genesis because of the longer duration of
TGA attacks and the absence of epileptic changes. Some authors
favor a mechanism of retrograde venous congestion from the superior vena cava to cerebral veins.2 Both of our patients had no
history of migraine, epilepsy, or stroke but did have hypertension
for several years.
As shown, aortic dissection is a possible cause of TGA. For any
patient who does not fulfill the criteria of pure TGA, it is necessary to exclude other probable causes such as aortic dissection,
especially if there is any history of chest pain, unexplained hypotension, or pathologic findings on chest radiographs.7
From the Department of Neurology (Dr. Gaul), Martin-Luther-University,
Halle-Wittenberg; Departments of Neurology (Drs. Gaul, Dietrich, and
Neundörfer) and Neuroradiology (Dr. Tomandl), Friedrich-AlexanderUniversity, Erlangen-Nuremberg; and Department of Neurology (Drs.
Dietrich and Erbguth), Nuremberg, Germany.
Received May 17, 2004. Accepted in final form August 10, 2004.
Address correspondence and reprint requests to Dr. Charly Gaul, Department of
Neurology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Strasse
40, 06097 Halle/Saale, Germany; e-mail: Charly.Gaul@gmx.de

Bilateral carotid artery dissection
with thyrotoxicosis
C.R. Campos, MD; M. Basso, MD; E.F. Evaristo, MD;
F.I. Yamamoto, MD; and M. Scaff, PhD
Spontaneous internal carotid artery dissection (ICAD) is a major
cause of stroke in young adults.1 Environmental factors, such as
minor trauma and mechanical stretching, may trigger the dissection, especially in patients with an underlying arteriopathy. Despite the direct vascular effects of thyroid hormones (THs),2-4 the
predisposition for arterial dissection has not been identified in
Graves disease (GD). We describe two women with bilateral ICAD
and GD with fatal outcome, one with histopathologic study.
Case reports. Patient 1. A 45-year-old woman with irregular
treatment for GD was admitted after 6 hours of right hemiparesis
and aphasia. She had no vascular risk factors. On admission, her
blood pressure was 130/80 mm Hg, and her pulse was 150 beats/
min. She presented a mild nonfluent aphasia and right-sided
hemiparesis. EKG showed sinusal tachycardia. Brain CT showed
an ischemic infarct in left middle cerebral artery (MCA) territory.
Angiography detected bilateral irregular high-grade stenosis
starting ~2 cm distal to the carotid bulbs and extending to the
base of the skull, suggesting ICAD. Anticoagulation was started.
After 24 hours, the patient deteriorated with left-sided hemiparesis and right hemianopia. MRI showed infarctions in the territory
of the right anterior cerebral artery and both MCAs. One month

Copyright © 2004 by AAN Enterprises, Inc.

References
1. Hodges JR, Warlow CP. Syndromes of transient amnesia: towards a
classification. A study of 153 cases. J Neurol Neurosurg Psychiatry 1990;
53:834 – 843.
2. Lewis SL. Aetiology of transient global amnesia. Lancet 1998;352:397–
399.
3. Tong DC, Grossman M. What causes transient global amnesia? New
insights from DWI. Neurology 2004;62:2154 –2155.
4. Stillhard G, Landis T, Schiess R, Regard M, Sialer G. Bitemporal hypoperfusion in transient global amnesia: 99m-Tc-HM-PAO SPECT and
neuropsychological findings during and after an attack. J Neurol Neurosurg Psychiatry 1990;53:339 –342.
5. Huber R, Aschoff AJ, Ludolph AC, Riepe MW. Transient global amnesia.
Evidence against vascular ischemic etiology from diffusion weighted imaging. J Neurol 2002;249:1520 –1524.
6. Strupp M, Brüning R, Wu RH, Deimling M, Reiser M, Brandt T.
Diffusion-weighted MRI in transient global amnesia: elevated signal intensity in the left mesial temporal lobe in 7 of 10 patients. Ann Neurol
1998;43:164 –170.
7. von Kodolitsch Y, Nienaber CA, Dieckmann C, et al. Chest radiography
for the diagnosis of acute aortic syndrome. Am J Med 2004;116:73–77.

later, she deteriorated with decerebrate rigidity and died. Brain
CT revealed diffuse edema and midline shift.
Patient 2. A 33-year-old woman with poorly controlled GD
was admitted after 3 days of left hemiparesis and aphasia. She
had no vascular risk factors. She was febrile; her blood pressure
was 180/110 mm Hg, and her pulse was 146 beats/min. She had
left-sided hemiplegia, right-sided hemiparesis, global hyperreflexia, left Babinski sign, right conjugated gaze palsy, and drowsiness. EKG showed sinusal tachycardia. Brain CT revealed
infarctions in both MCA territories. Angiography showed bilateral
high-grade stenosis starting ~3 cm distal to the carotid bulbs and
extending to the base of the skull, suggesting ICAD. Anticoagulation was started. She deteriorated during the next 24 hours with
decerebrate rigidity. Brain CT showed marked infarctions in both
internal carotid artery territories, edema, and midline shift. She
died 2 days later.
Postmortem examination macroscopic findings were diffuse
and small hemorrhagic lesions at pons, severe infarctions in both
MCA territories, and edema with bilateral uncal and tonsillar
herniation. The circle of Willis and its major branches were unremarkable. Both internal carotid arteries presented multifocal media degeneration with intracellular and extracellular vacuoles,
sometimes coalescing to form cysts, and areas of marked wall
thinning. There were also deposits of Alcian blue/periodic acid–
Schiff-positive substances in the degenerating media. The intima
was focally thickened, and the internal elastic lamina was absent
in some areas and reduplicated and distorted in others (figure).

Figure. Angiography showing right (A) and left (B) internal carotid artery dissection. (C) Cross-section of the right internal carotid artery. There is focal tunica media thinning and an area of duplication and distortion of the internal elastica
lamina on the left edge of the artery (Elastica–Verhoff stain, ⫻25). (D) Cross-section of the left internal carotid artery. Tunica media showing vacuolar degeneration with intracellular and extracellular vacuoles and deposits of Alcian blue/periodic acid–Schiff-positive substances (⫻400).
December (2 of 2) 2004

NEUROLOGY 63

2443

Aortic dissection presenting with transient global amnesia-like symptoms
C. Gaul, W. Dietrich, B. Tomandl, et al.
Neurology 2004;63;2442-2443
DOI 10.1212/01.WNL.0000148581.00912.1A
This information is current as of December 28, 2004
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