Neurosyphilis presenting with raeder's syndrome

Abstract

We report a case of Raeder's paratrigeminal syndrome caused by neurosyphilis. A 37-year-old man was admitted to our hospital in July 1986 because of ptosis of the left eyelid and left hemifacial pain. He had had sexual intercourse with a prostitute 4 months before admission. He noticed an erythematous skin eruption on his lower abdomen one month before admission that disappeared in 1 week. Drooping of the left lid and pain around the left eye began 5 days before admission. The pain was deep and persistent with periodic exacerbation accompanied by nausea and vomiting. Examination showed left Horner's syndrome including miosis and ptosis but without anhidrosis on the face. Extraocular muscle movement and optic fundi were normal. Sensory impairment in the area of the upper and middle branches of the left trigeminal nerve was present. Serum serologic test for syphilis (VDU) was positive at a titer of 1:64. Boniuk and Schlezinger 1 1) divided Raeder's syndrome into two groups. Group 1 included cases with neuralgia, oculosympathetic paralysis, and perisellar nerve involvement. Group 2 included patients with neuralgia and oculosympathetic paralysis but without perisellar nerve involvement. The present case had trigeminal neuralgia and Horner's syndrome with a single perisellar nerve disturbance. This patient thus belongs to Group 1 Raeder's syndrome. The causes of Raeder's syndrome are numerous and include trauma 121, brain tumor 137, aneurysm 147, hypertension 151, migraine 161, many kinds of inflammation, and unknown factors. The inflammatory conditions include sinusitis, abscessed tooth, chronic otitis media, lobar pneumonia[l], and syphilitic osteitis 171. Toussaint 177 described a case of Raeder's syndrome with syphilitic osteitis in which the apex of the petrosal bone was destroyed. In the present case, the patient was suffering from neurosyphilis. However, there was no evidence of osteitis as in the case reported by Toussaint, and no distinct vasculitis. It seems possible that perivasculitis in the context of syphilitic meningeal inflammation in the region where the upper and middle branches of the trigeminal nerve pass through the meninges around the superior orbital fissure and foramen rotundum could have caused the Raeder's syndrome observed in our patient. The recent consensus statement on diabetic neuropathy (11 is a useful, but in my opinion, incomplete document. It addressed hypofunction of both the somatic and autonomic nervous systems in diabetes mellitus but omitted an important component of the latter. The autonomic nervous system consists of parasympathetic and sympathochromaffin (or sympathoadrenal) divisions. The sympathochromaffin system includes two components: the sympathetic nerves and the chromaffin cells including those that comprise the bulk of the adrenal medullae [2, 31. Both the sympathetic postganglionic neurons and the adrenal medullae synthesize and release catecholamines, among other products, in response to central nervous system activation of sympathetic preganglionic cholinergic neu...

Numbness matters: A clinical review of trigeminal neuropathy

“…Trigeminal nerve enhancement may be seen on MRI with gadolinium contrast administration, a finding which may not have an overt clinical correlate (83,84). Neurosyphillis may also be included in the differential diagnosis of TNO, and may mimic a tumor involving the trigeminal ganglion (85,86). Remarkably, TNO has been seen as the isolated manifestation of a 1.5-cm by 1.5-cm pontine abscess (87).…”

Ræder's Syndrome

“…Watanabe et al added neurosyphilis to the list of conditions that may cause a proper Ræder's syndrome (11). Their patient had involvement of the two upper divisions of the trigeminal nerve in addition to pain, ptosis and miosis, and became free of symptoms and signs within 2 weeks of the start of treatment with penicillin.…”