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Neurosyphilis presenting with gummatous oculomotor nerve palsy
  1. W W Seeley,
  2. N Venna
  1. UCSF Memory and Aging Center, PO Box 1207, San Francisco, CA 94143-1207, USA
  1. Correspondence to:
 W W Seeley
 UCSF Memory and Aging Center, PO Box 1207, San Francisco, CA 94143-1207, USA; wseeleymemory.ucsf.edu

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Although epidemiological studies suggest that the incidence of primary syphilis is rising,1 neurosyphilis remains an uncommon manifestation of Treponema pallidum infection. In addition, the MRI appearances of this treatable neurological condition are not well known. Many patients with neurosyphilis are asymptomatic, but manifestations include subacute basal meningitis, a meningovascular syndrome of small deep cerebral and cranial nerve infarctions, chronic gummatous inflammation with focal intracranial mass lesions, chronic comportmental dementia of general paresis, and chronic sensory-ataxic myelopathy of tabes dorsalis. We report a case in which a meningeal form of neurosyphilis presented with rapid evolution of a pupil-involving oculomotor nerve palsy to highlight the clinical, CSF, and MRI features and good response to treatment.

Case report

The patient was a 54 year old right handed homosexual man with a history of syphilis of unknown stage, treated with penicillin 25 years previously. He was well until 6 weeks prior to evaluation when he sustained minor head trauma in an automobile accident, followed by intermittent headaches, fatigue, photophobia, and anorexia. Four days before admission he developed worsening and persistent drooping of the right eyelid and double vision. On examination, his mental status was remarkable only for psychomotor slowing. The right pupil was round but enlarged at 6 mm and sluggishly constricted to 5 mm with direct and consensual light stimulation as well as near vision. The left pupil was round and 4 mm and constricted briskly to 2 mm to light. The right eye showed moderate ptosis of the upper lid, and the globe was deviated laterally in primary gaze with markedly impaired adduction and elevation. In the left eye, ptosis was absent and ocular motility was normal. Other cranial nerve, sensory, motor, and reflex functions and gait were normal with the exception of a slight decrease in vibration and position sense in the feet. There were no signs of meningeal irritation. Head computed tomography (CT) and CT angiography revealed neither blood in the subarachnoid space nor evidence of intracranial aneurysm. MRI of the head (fig 1) showed a spheroid contrast-enhancing lesion at the root of the right oculomotor nerve, which extended towards the cavernous sinus. Incidentally noted were right cerebellar and right frontal developmental venous anomalies. CSF examination revealed normal opening pressure at lumbar puncture, 344 white blood cells (WBCs) (95% lymphocytes), 14 red blood cells (RBCs), protein of 167 mg%, and glucose of 39 mg%. CSF Venereal Disease Research Laboratory test (VDRL) was positive at 1:8 whereas serum Rapid Plasma Reagin (RPR) was positive at 1:64. HIV testing was negative. Treatment with intravenous penicillin G (4 million Units every 6 hours) was administered for 2 weeks. By treatment day 4, the adduction and elevation of the right eye were improving. At 1 month follow up, mild fatigue persisted. There was trace right ptosis. Elevation and adduction of the right eye had improved to nearly normal, but the pupil remained 6 mm and sluggishly responsive to light. Repeat CSF examination showed seven WBCs (97% lymphocytes), protein of 86 mg%, and glucose of 71 mg%. CSF VDRL and serum RPR titres were unchanged. At 6 months, no additional improvement in oculomotor nerve functions was seen but fatigue had subsided. Repeat MRI 7 months after hospital admission showed complete resolution of the oculomotor nerve abnormality.

Figure 1

Head MRI showing the 8 mm (antero-posterior) × 6 mm (left to right) × 6 mm (rostro-caudal) tapering spheroid lesion at the base of the right midbrain, tracing the course of the oculomotor nerve forward into the cavernous sinus (panels A–F). The lesion is isointense to adjacent brain on T1 and T2 sequences (panels A and B) and enhances on a T1 sequence after gadolinium contrast (panel C). Pre-treatment (D–F) and post-treatment (G–I) coronal images demonstrate complete resolution at 7 months.

Discussion

Neurosyphilis is known to cause oculomotor nerve palsies either in the meningovascular phase, due to small vessel vasculitis with resultant nerve infarction,2 or in granulomatous basal meningitis, due to inflammation of the nerve or its investiture; however, the literature on syphilitic mass lesions around the oculomotor nerve is sparse. Vogl et al3 reported a case of oculomotor nerve palsy associated with MR findings similar to ours that also resolved with penicillin treatment. Standaert et al4 described an enhancing penicillin-responsive lesion based in the interpeduncular cistern that compressed the ventral midbrain. The oculomotor nerve lesion in our patient was isointense to adjacent brain on T1 and T2 MR sequences, with brisk enhancement after intravenous injection of gadolinium contrast. We believe the lesion was a manifestation of meningeal syphilis in the form of an oculomotor nerve gumma. A gumma is a focally accentuated, exuberant granulomatous response of the meninges, typically with sparse treponemal organisms. Nonetheless, treatment of the underlying infection quiets the inflammatory process and can, as in our patient, lead to significant reversal of neurological deficit. We add our case to the growing literature on MR correlates of neurosyphilis and encourage a search for neurosyphilis when an unexplained mass lesion is present in the basal subarachnoid space. Neurosyphilis, albeit rare, still deserves inclusion among eminently treatable causes of a rapidly developing oculomotor nerve palsy.

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