Transient Global Amnesia With
To the Editor.\p=m-\Short-livedconfusion often follows syncopal episodes, but
unusual unless infarction has occurred. We describe here a patient with transient global amnesia1,2 associated with a syncopal attack at the onset of dissection of an aortic aneurysm.
Report of a Case.\p=m-\A 55-year-old man was hospitalized with loss of memory. He awoke complaining of chest pain radiating into his jaw, and soon had a syncopal episode during which he was "pale" and briefly unresponsive. When seen one hour later, he had no recollection of the chest pain. He was an obese man and not in
distress. Blood pressure was 190/110 mm Hg; pulse rate was 80 beats per minute and regular. A systolic ejection murmur, not previously noted, was heard along the left sternal border. The patient was alert, cooperative, and oriented to place and person, but not to time. He repeated, "I am confused, I don't know what's happening," and "Something is wrong." There was total-amnesia for all events that had occurred during the three days prior to admission. He could repeat seven numbers forward and had intact remote memory. Calculations and fund of knowledge were normal. The remainder of the findings were unremarkable. Results of usual laboratory studies, including blood glucose level, were normal. The ECG was normal, but the chest roentgenogram showed mild cardiomegaly with uncoiling of the ascending aorta. The EEG was normal. Within 24 hours, the amnesic period had decreased to the events surrounding his admission. However, increasing chest pain developed, and roentgenograms showed widening of the mediastinum. On the third day, the patient died suddenly. Necropsy showed a dissecting aneurysm of the ascending aorta with rupture into the pericardium. Permission to examine the brain was denied.
Comraewi.-Chase et al3 have
serious illness was suspected. Transient global amnesia may her¬ ald a life-threatening condition asso¬ ciated with cerebral hypoperfusion, and history of pain or other symptoms may be lost in amnesia.
from that of any other form of this disease noted in the literature, with the exception of one case of a family originally described by Pierre Ma¬ rie.8 These facts led us to speculate that not only is DSPA different from other forms of spinocerebellar degenera¬ tion, but it may even represent, at least in some cases, the pathology of the controversial entity known as Marie's hereditary cerebellar ataxia. We can only hope that the agelong dispute between "splinters" and "lumpers" in relation to spinocerebel¬ lar atrophies will become obsolete as soon as a precise etiology has been found for these diseases.
ported that some patients with cere¬ bral syndromes due to dissecting
aortic aneurysm manifest only tran¬ sient signs of diffuse cerebral dys¬ function without persistent focal signs. Symptoms in this group were attributed to arterial hypotension. Syncope is estimated to occur in 25% of patients with a dissecting aneu¬ rysm, and at times will mask the severe pain.1 Although chest pain generally recurs, in some it is mild. Our patient's initial chest pain was obscured by loss of memory, and it was not until the pain returned that a
G. A. Rosenberg, MD Dept of Neurology Univ of New Mexico School of Med Albuquerque, NM 87131
1. Fisher CM, Adams RD: The transient global amnesic syndrome. Acta Neurol Scand 40(suppl 9):7-82, 1964. 2. Mathew NT, Meyer JS: Pathogenesis and natural history of transient global amnesia. Stroke 5:303-311, 1974. 3. Chase TN, Rosman NP, Price DL: The cerebral syndromes associated with dissecting aneurysm of the aorta. Brain 91:173-190,1968.
To the Editor.\p=m-\Pogacaret al reported in the Archives (35:156, 1978) clinical findings in two members of a family with dominant spinopontine atrophy (DSPA), an entity originally described by us in a study of two other members of the same family.1 On the basis of one
F. Boller, MD Neurobehavior Unit Cleveland VA Hosp Cleveland, OH 44106 J. M. Segarra, MD Dept of Neuropathology Boston VA Hosp Boston, MA 02130
postmortem examination, Pogacar
et al concluded that DSPA is not clear-
ly distinguishable from olivopontocerebellar atrophy (OPCA). Microscopic
examination of the cerebellum and the inferior olivary nuclei of their case undoubtedly showed greater changes than those that occurred in our propositus and in other cases noted in the literature.2.3 The reason for this difference is unclear; however, it is well known that there can be some variation among members of the same family in the pathological features of spinocerebellar atrophies. The description of Pogacar et al suggests cerebellar and olivary lesions considerably less prominent than those found in classical OPCA, in which the loss of cells is usually quite severe or complete.4 Gross findings by Pogacar et al, by us, and by other investigators showed a very strikingly atrophie pons and spinal cord, in clear contrast to the relatively intact olivary nuclei and cerebellum. Finally, the case of Pogacar et al, as well as the other confirmed cases of DSPA, failed to show the changes so often found in the substantia nigra and cortex of patients with OPCA.1 After Holmes' work,5 many modern authors have questioned the existence of Pierre Marie's hereditary cerebel¬ lar ataxia.8 As we pointed out in a recent review,7 the clinical picture of DSPA corresponds more closely to the description given by Pierre Marie than to any other form of spino¬ cerebellar atrophy. On the other hand, the pathology of DSPA is different
1. Boller F, Segarra JM: Spinopontine degeneration. Eur Neurol 2:356-373, 1969. 2. Taniguchi R, Konigsmark BW: Dominant spinopontine atrophy: Report of a family through three generations. Brain 94:349-358, 1971. 3. Ishino H, Sato M, Mii T, et al: An autopsy case of Marie's hereditary ataxia (Japanese). Psychiatr Neurol Jap 73:747-757, 1971. 4. Eadie MJ: Olivo-ponto-cerebellar atrophy, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, North Holland Publishing Co, 1975, pp 433-449. 5. Holmes G: An attempt to classify cerebellar disease with a note on Marie's hereditary cerebellar ataxia. Brain 30:545-567, 1907. 6. Marie P: Sur l'h\l=e'\r\l=e'\do-ataxiec\l=e'\r\l=e'\belleuse. Sem Med 13:444-447, 1893. 7. Boller F, Segarra JM: Spinopontine degeneration, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, North Holland Publishing Co, 1975, pp 389-402. 8. Guillain G, Bertrand J, Godet-Guillain J: Etude anatomique d'un cas d'h\l=e'\r\l=e'\do-ataxie c\l=e'\r\l=e'\belleuse.Rev Neurol 73:609-611, 1941.
Reply.\p=m-\ Boller and Segarra are pointing out the limitations are facing because of the small
correct in we
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number of the cases studied. I would like to reiterate, however, that, not being aware of previously reported members of the same family and limiting ourself to pathological findings, we did have problems diagnosing our autopsy case as dominant spinopontine atrophy. On the other side,
both our reported cases as olivopontocerebellar atrophy. We all hope that eventually the precise etiology for these diseases will be known and perhaps, but not necessarily, make our disagreement irrelevant.
S. Pogacar, MD Div of Neuropathology General Hospital Rhode Island Med Center