Neurosyphilis

Case contributed by Damien Charles
Diagnosis almost certain

Presentation

History of unknown cause left deep MCA/ACA infarct. Presenting with new left deep infarct and multiple enhancing brain lesions.

Patient Data

Age: 45 years
Gender: Male

MRI without contrast

mri

History of left deep MCA/ACA infarct of unknown etiology. The patient presents with a new left deep infarct (high signal on DWI and T2/FLAIR).

MRI with contrast, same day

mri

There are three intra-axial enhancing lesions: two are located in the right frontal lobe, while the third one is in the right occipital lobe. All of them are surrounded by a significant amount of vasogenic edema. The right frontal lesions show low rCBV (relative cerebral blood volume). Additionally, some areas of these lesions show hypoT2 signal. DWI is not shown, but no reduced diffusion was observed.

Case Discussion

This patient has been diagnosed with two left deep infarcts in the territory of lenticulostriate arteries ACA/MCA, one old and one recent. Additionally, the patient's brain MRI reveals three intra-axial enhancing lesions with low rCBV, extensive vasogenic edema, as well as areas of low T2 signal. No signs of meningitis were found on MRI. A CT scan of the chest, abdomen, and pelvis was normal.

Although lenticulostriate infarcts and "granulomatous" lesions are often associated with tuberculosis, after conducting a thorough clinical examination, reviewing the patient's medical history, and performing a lumbar puncture, the final diagnosis was determined to be neurosyphilis. Specifically, the patient had syphilitic gummas and vascular complications. Gummas are traditionally cortical-based, more frequent in the convexities, and can be associated with adjacent meningeal enhancement. These gummas are a manifestation of tertiary syphilis.

Syphilis is a systemic sexually transmitted disease caused by a bacterium called Treponema pallidum. It is known as the "great mimicker" because it can imitate many other infectious or inflammatory conditions. Neurosyphilis can occur 1 to 25 years after the initial infection and may occur in up to 40% of untreated patients. Three major clinical phenotypes are described:

  1. neuropsychiatric: diffuse cerebral atrophy (sometimes more pronounced in fronto-temporal regions), white matter lesions, and infarcts (secondary to arteritis).

  2. meningovascular: infarcts, cerebral atrophy, and meningeal enhancement.

  3. myelopathic: long segment "myelopathy" (sometimes with preferential dorsal column involvement); contrast enhancement (patchy or nodular) might occur.

Other imaging manifestations include cranial nerve enhancement and optic neuritis (with or without associated perineuritis), basilar meningitis, gummas (pseudo-tumoral lesions), and bi-temporal encephalitis.

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