Cerebral arterial ectasia with saccular aneurysms.

Abstract

The term “arterial ectasia” is employed in this communication to describe the fusiform vascular dilatation presumably resulting from an abnormality in the elastic component of the vessel wall. Report of a Case History: The patient, a 54-year-old male, was admitted to the Neurology Service of Jefferson Medical College Hospital, Philadelphia, for the evaluation of eye pain and right facial palsy of eight months duration. Fifteen years earlier tinnitus developed in the right ear. Over the succeeding five years he became deaf in that ear, and a tremor of the hands and slurred speech became apparent. Eight months prior to admission, he awoke one morning with a right facial palsy, which was shortly thereafter complicated by an infection of the right eye, necessitating a partial tarsorrhaphy. Four months later the patient experienced episodes of vertigo. Numerous operations had been performed on his legs to relieve a spastic paraparesis with contractures, supposedly the result of cerebral palsy. For the past ten years he had been unable to have an erection. Frequency, urgency, nocturia, and dysuria were of many years duration. The family and social history were unremarkable except that the patient had never worked. The patient was one of twins. Physical Examination: The patient was a thin white male with a yellowish complexion and soft smooth skin. There was an obvious right facial palsy and partial tarsorrhaphy of the right eye. Axillary and pubic hair were sparse, and other body hair was absent. The testes were small and soft. Neurologic Examination: The patient was markedly ataxic on sitting and unable to stand. Motor strength was preserved in the upper extremities, but there was a spastic paraparesis of the lower extremities with ankle clonus and a positive Babinski sign bilaterally. He had a marked bilateral intention tremor with severe impairment in performance of pronation- supination, hand-patting, and finger-tapping. Cranial nerve examination revealed bilateral optic pallor, nystagmus, diplopia which increased on gaze to the left, and weakness of convergence. The right corneal reflex was depressed as well as all sensation in the first division of the right trigeminal. There was a complete peripheral right facial palsy. Taste sensation was absent in the anterior two-thirds of the right side of the tongue. A GO-decibel loss in bone conduction and no response to caloric stimulation were found on the right. The palate pulled to the left on phonation, and the speech was dysarthric. There was no paralysis of the tongue. Laboratory Findings: The patient had a hypochromic microcytic anemia. Azotemia due to chronic pyelonephritis was superimposed on a neurogenic bladder. Endocrine studies revealed panhypopituitarism with no response to Metapirone stimulation.