Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Progression of cluster headache to Raeder's syndrome with marked response to corticosteroid therapy: a case report

Takeshi Ikeuchi, M.D.1), Takayoshi Tokutake, M.D.1), Yuuichi Sakamaki, M.D.1), Mineo Takagi, M.D.2) and Masatoyo Nishizawa, M.D.1)

1)Department of Neurology, Brain Research Institute, Niigata University
2)Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University

A 42-year-old man complained of severe left orbital pain for 7 months. The diagnosis of cluster headache was made on the basis of diagnostic criteria formed by the International Headache Society. Sumatriptan was effective in relieving pain to a certain degree, but the frequency of the occurrence of pain gradually increased. Subsequently, he presented sensory disturbances in the left trigeminal nerve, and was admitted to our hospital. On admission, his neurological examination revealed left miosis and paresthesia in the first branch of the left trigeminal nerve. Neither anhidrosis nor ptosis was noted. His autonomic failure was consistent with post-synaptic disturbance as determined by pharmacological analysis for pupil's function. On the basis of the unique combination of neurological sings and symptoms including the unilateral headache, partial Horner's syndrome, and V1 sensory disturbance, we diagnosed him as having Raeder's syndrome. To exclude the possibility of a lesion in the Gasser ganglion of the middle fossa of the cranium or carotid artery causing symptomatic Raeder's syndrome, imaging studies including brain MRI, cervical MRA, and Doppler ultrasonography were performed, which revealed normal findings. We started him on oral prednisolone at 1 mg/kg once a day, which resulted in a rapid and dramatic suppression of pain. Thus, this case showed a progression from cluster headache to idiopathic Raeder's syndrome, which suggests that these two disorders might share common pathological and anatomical lesions.

(CLINICA NEUROL, 45: 321|323, 2005)
key words: Raeder syndrome, cluster headache, partial Horner's syndrome, paratrigeminal sympathetic syndrome

(Received: 1-Jun-04)