mise à jour du 11 avril 2002
No To Shinkei
 cas cliniques
 Progressive dysautonomia in hemangioblastoma of the fourth ventricle region
Kimihito Arai, Kohei Kita, Atsushi Komiyama, Keizo Ilirayaina, Naokatsu Saeki and Ko-ichi Nagao
Brain Research Institute, Chiba University School of Medicine, Japan


Yawning and stroke

No To Shinkei

Tumors of the posterior fossa presenting orthostatic hypotension are rare and only nine cases have been reported so far. The locations of almost all these tumors were near the fourth ventricle and three of them were hemangioblastoma. A case of a tumor of the fourth ventricle showing autonomic disturbances mainly composed of orthostatic hypotension is reported. A 42-year-old male was admitted to the Department of Neurology of Chiba University Hospital on June 25th, 1981 because of three years' history of autonomic disturbances including orthostatic syncope, impotence, urinary disturbance and bowel dysfunction such as vomiting, diarrhea and constipation. He also complained of weight loss and staggering of gait to the left side. On admission, the patient was emaciated being 50 kg in weight and 172 cm in height. Neurological examination revealed hippus of bilateral pupils in light reflex, saccadic eye movement, slightly hypoactive deep tendon reflexes, mild terminal oscillations in bilateral finger-to-nose test, oscillation in the left heel-to-knee test, staggering tendency of gait to the left, slightly impaired tactile and thermal sensations in distal parts of the legs. Autonomic disturbances were showed by orthostatic hypotension (BP 104-50 in supine and 70-40 in sitting position), impotence, weight loss, anorexia, decrease of sweating, spontaneous yawning and loss of sensation of bladder fullness. About 5 weeks after admission, he began to complain of temporal headache and showed impairment of memory, drowsiness, paroxysmal apnea and papilledema.

Autonomic function tests showed BP fall without increase of heart rate and insufficient orthostatic increase of plasma noradrenaline during head-up tilting, no response of BP rise in cold pressor test, mild BP rise in noradrenaline infusion test, low R-R interval variation of ECG, pupillary dilatation to 1.25% epinephrine and loss of dilatation to 5% tyrarnine, both of which became normalized later, and atonie bladder in urodynamic study. Acetylcholine intradermal injection at extremities showed almost normal local sweating of axonal reflex. These results, though net even in part, suggested that the autonomic disturbances were mainly derived from central lesion. Motor nerve conduction velocity and needle electromyogram were normal.

Brain CT scan showed a round-shaped and highly contrast-enhanced tumor in the lower hall of the fourth ventricle. Suboccipital craniotomy was carried out and a vascular tumor was shown to exist from dorsal part of medulla to cerebellar vermis. On biopsy, histological diagnosis of hemangioblastoma was made.

The feature of this case was that the main symptoms were not cerebellar ones, but autonomie disturbances mainly composed of orthostatic hypotension. The lesions responsible for the autonomic disturbances were thought te be mainly in the lower brainstem including vasornotor center of the medulla oblongata and partly in the cerebellar vermis which has been demonstrated to play some role in the control of the autonomic nervous system. Autonomic disturbances can be such important clinical features in cases of posterior fossa tumor that we should always keep this possibility in mind in the case of orthostatic hypotension.