J. Neurol. 221,279-283 (1979)
Neurology © by Springer-Verlag 1979
A Case of Priapism with Ruptured Intracranial Aneurysm A. Takaku, O. Fukawa, and J. Suzuki Division of Neurosurgery, Institute of Brain Diseases, Tohoku University School of Medicine, 5-13-1, Magamachine Sendai, Japan 982
Summary. A man of 35 years, who had had three attacks of subarachnoid hemorrhage in the previous 3 years, was admitted to hospital with complaints of headache and priapism. There had been intermittent priapism with abnormal acceleration of sexual desire since the first attack, and erection of the penis had persisted with intolerable pain after the last attack of subarachnoid hemorrhage. A carotid angiogram revealed an aneurysm at the junction of the left internal carotid and posterior communicating arteries. Clipping of the aneurysmal neck was successfully performed. However, priapism continued for 22 days after the operation and resulted in sexual impotence. The neurological problems of priapism are discussed with special reference to a hypothalamic lesion caused by the ruptured intracranial aneurysm in this report. Key words: Priapism - Subarachnoid hemorrhage - Intracranial aneurysm.
Zusammenfassung. Klinikaufnahme eines 35j~ihrigen Mannes mit Klagen fiber Kopfschmerzen und Priapismus. In der Vorgeschichte hatte der Patient drei Attacken von Subarachnoidalblutung. Seit dem ersten Blutungsereignis kam es intermittierend zu Priapismus, verbunden mit fiberh6hten sexuellen Wfinschen. Seit der letzten Subarachnoidalblutung blieb eine Dauererektion des Penis mit unertr~iglichen Schmerzen bestehen. Die Carotisangiographie zeigte ein Aneurysma im Winkel der linken supraclinoidalen A. carotis interna und A. communicans posterior. Der Aneurysmahals wurde mit Erfolg geclippt, jedoch blieb der Priapismus noch fiber 22 Tage nach der Operation bestehen, verbunden mit einer sexuellen Impotenz. Die neuro-pathophysiologischen Probleme des Priapismus werden er6rtert mit besonderer Berticksichtigung einer hypothalamischen L~ision, welche in diesem Falle durch das rupturierte Aneurysma hervorgerufen wurde.
Various kinds of neurological symptoms, in addition to the so-called meningeal symptoms, are seen with subarachnoid hemorrhage due to the rupture of an intracranial aneurysm, but, among these, symptoms related to the autonomic 0340-5354/79/0221/0279/$1.00
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nervous system seem to be rare. A case of priapism after subarachnoid hemorr h a g e d u e t o t h e r u p t u r e o f a n i n t r a c r a n i a l a n e u r y s m is r e p o r t e d a n d t h e l i t e r a t u r e reviewed.
Case Report A man, aged 35, was admitted to hospital with severe headache on 14 February 1968. The cerebrospinal fluid (CSF) was bloody. After conservative therapy for 2 months, he returned to work without neurological deficit. Subsequently, however, an abnormal increase of sexual desire occured several times in a year, and erection of the penis continued for over 3 h after sexual intercourse, even in sleep. On 12 January 1971, he had another attack of severe headache for a few days, and extremely strong sexual desire was experienced thereafter. He performed sexual intercourse in spite of the headache. His wife leit that he was abnormal. On 21 January 1971, he had severe headache for 24h without loss of consciousness, but with painful, persistent erection of the penis, and was admitted to the same hospital 3 days later with priapism. A carotid angiogram revealed an aneurysm at the junction of the left internal carotid and posterior communicating (IC-PC) arteries. He was transferred to our clinic on 30 January 1971. On admission he was conscious, had a slight headache, and complained of a persistent, painful erection. There were no other neurological findings. The patient had no history of local trauma, genitourinary infection or hematologic disorder. Appropriate laboratory and X-ray studies were normal. Hypercoagulopathy was not found. A carotid angiogram on the 6th day after the final hemorrhage revealed an aneurysm of the left IC-PC junction (Fig. 1), but we could see neither angiospasm nor hydrocephalus on the angiograms.
Fig. 1. Preoperative left carotid angiogram with aneurism at junction of internal carotid and posterior communicating arteries
Priapism with Ruptured Intracranial Aneurysm
Fig. 2. Postoperative left carotid angiogram showing no trace of aneurysm
Surgery for the cerebral aneurysm was performed 12 days after the final attack. There was an aneurysm (about 10 x 8 x 12 mm) of the left internal carotid artery which had a broad heck of about 6 mm, and extended posteriorly and downward. The left posterior communicatingartery and anterior choroidal artery were ran in contact with the aneurysm. At the neck of aneurysm, there were 2 bubbles which were at the point of rupture, but the subarachnoid space was clear. No damage of left temporal lobe could be seen. Clipping of the neck of the aneurysm and muscle wrapping for both bubble aneurysms were carried out successfully (Fig. 2). The general postoperative course was uneventful and he left the hospital 15 days after operation. After discharge, urokinase and heparin were administered. Although the pain was relieved, the erection of the penis continued for 22 days after surgery, when the erection began to be relieved gradually and cured. However, erection of his penis is now impossible.
Discussion Physiological erection of the penis which results from sexual sensation or s t i m u l a t i o n of the penis, is controlled by the a u t o n o m i c nervous system. It is also considered that the c o n t r o l l i n g center is located at the upper part of the sacral region of the spinal cord and, f u r t h e r m o r e , that the cerebral limbic system a n d a n t e r i o r part of the h y p o t h a l a m u s , which are the centers of male sexual activity, p r o m o t e it. Physiological erection is induced when the excitement, perceived in the c e r e b r u m t h r o u g h the olfactory, visual, tactile or sexual sensations, is t r a n s m i t t e d to the center of erection in the sacral region of the spinal cord. This seems to be related to the fact that the erection is also induced by electrical a n d chemical s t i m u l a t i o n s of the posterior cinglate gyrus or h y p o t h a l a m u s . O n the
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other hand, the penis can also become erect by way of a simple reflex not related to the cerebrum. This reflex erection, due to direct stimulation of the penis, passes along the spinal reflex arc. Priapism, however, is a pathological condition with continuous or intermittent erection of the penis without sexual desire. It is usually accompanied by severe penile pain. Various mechanisms of priapism have been reported. Hinman has discussed prolonged erection from psychic stimuli resulting in continued venous stasis of the corpus spongiosum, increased viscosity of the blood, edema of the trabeculae with priapism . In Ookoshi's series of 156 patients, priapism was caused by tumor (21.1%), leukemia (19.9%), idiopathic erection (11.6%), trauma (7.5%) and cerebrospinal lesions (2.1%), but not details were given on 3 cases of cerebrospinal lesions . According to the Tsuchiya series of 159 cases, 27 cases were caused by lesions of the central nervous system including the spinal cord, or by hypertension, which is supposed to induce cerebrovascular accidents . Among them, only one case apparently had priapism combined with intracerebral hematoma. Recently D o r m a n et al.  reported four cases of phenothiazine induced priapism in which a central nervous system effect must be considered. The present case did not have the findings of a hematologic disorder, tumor or trauma as the mechanism for priapism. The possibility of thrombosis of the corpora cavernosa could be denied because hypercoagulopathy was not seen. It was indicated that the priapism was apparently related to the central nervous system. The fact that the patient had an abnormal increase of sexual desire and an abnormal erection of the penis after the first subarachnoid hemorrhage is very interesting, considering the relation between the diencephalon and priapism. In this case the so-called Klüver-Bucy syndrome must be discussed as a cause of priapism. However, visual agnosia, oral tendency and hypermetamorphosis were not seen and brain damage of the left temporal lobe was not seen at the time of operation in this case. So, this syndrome can be disregarded. In our 1,000 cases of intracranial aneurysm , only this case was accompanied by priapism. Although ruptured intracranial aneurysms have various clinical manifestations, symptoms of the autonomic nervous system have generally been rare. What changes occur in the hypothalamus or the cerebral limbic system in patients with ruptured intracranial aneurysm? The autopsy reports have revealed that these patients have a relatively high frequency of lesions in the hypothalamus. Crompton reported that 65 of 106 autopsied cases of ruptured intracranial aneurysm had lesions in the hypothalamus . Notable among these, 22 of 32 cases of anterior communicating artery aneurysm and 19 of 27 cases of internal carotid posterior communicating junction aneurysm had lesions of the hypothalamus consisting mainly of microhemorrhage and ischemia. Even in cases of relatively mild ruptured aneurysms, such as our cases, a similar mechanism should be considered. We have supposed in this case that the priapism was induced by stimulating effects but not destructive effects on the hypothalamus a n d / o r limbic system. However, in this cases it is not apparent whether or not the target of the stimulating effect was in the hypothalamus or in the cerebral limbic system.
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References 1. Hinman, F. Jr.: Priapism: Reasons for failure of therapy. J. Urol. 83, 420--428 (1960) 2. Ookoshi, M.: Priapism. In: Priapism, Ookoshi, M. (ed.), pp. 1--75. Tokyo: Nankodo 1950 (in Japanese) 3. Tsuchiya, F., Toyoda, Y., Nakagawa, K., Miura, M., Yoshimura, S., Tokue, A.: Priapisms following penile metastasis from cancer of urinary bladder and prostate. Jap. J. Urol. 61, 687--816 (1970) (in Japanese) 4. Dorman, B., Schmidt, J. D.: Association of priapism in phenothiazine therapy. J. Urol. 116, 51--53 (1976) 5. Kayama, T., Yoshimoto, T., Uchida, K., Takaku, A., Suzuki, J.: Prognosis of 1,000 cases of intracranial saccular aneurysms in surgical treatment. Tohoku J. Exp. Med. 126, 117--124 (1978) 6. Crompton, M. R.: Hypothalamic lesions following the rupture of cerebral berry aneurysms. Brain 86, 301--314 (1963)
Received November 28, 1978