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Case Reports
. 2019 Jul-Aug;10(4):444-446.
doi: 10.4103/idoj.IDOJ_388_18.

A Rare Case of Possible Vacuolar Degeneration of Leprosy in Brain with Segmental Necrotizing Granulomatous Neuritis and Horner's Syndrome

Sukriti Baveja  1 , Sunmeet Sandhu  1 , Deepak Vashisht  1
Affiliations
Case Reports

A Rare Case of Possible Vacuolar Degeneration of Leprosy in Brain with Segmental Necrotizing Granulomatous Neuritis and Horner's Syndrome

Sukriti Baveja et al. Indian Dermatol Online J. 2019 Jul-Aug.

Abstract

Leprosy has a predilection for peripheral nerves, but rarely does it involve the central nervous system (CNS). There is a single study of CNS involvement in leprosy showing vacuolar changes of motor neurons in medulla oblongata and spinal cord in autopsy findings. Besides this, there has been only one case report providing direct histopathological and molecular evidence of CNS involvement by leprosy in a living patient. Segmental necrotizing granulomatous neuritis (SNGN) is a rare condition affecting the peripheral nerves in leprosy usually seen as a complication of tuberculoid (TT) and borderline tuberculoid (BT) leprosy. We report the case of a 23-year-old male patient, a case of Hansen's disease (BT) who developed CNS involvement in the form of partial Horner's syndrome (right) and SNGN while on treatment. Magnetic resonance imaging of the brain revealed T2 hyperintense lesion on the dorsal aspect of left pontomedullary junction, suggestive of vacuolar degeneration of leprosy. Histopathology of greater auricular nerve (right) revealed SNGN.

Keywords: Granulomatous neuritis; Horner's syndrome; leprosy; segmental necrotizing; vacuolar degeneration.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Malar region (Rt) and ear showing well-defined erythematous edematous plaque, Grade 3 thickened (Rt) supraorbital (red line), and greater auricular nerve (yellow line)
Figure 2
Figure 2
Magnetic resonance imaging of the brain revealing T2 hyperintense lesion on the dorsal aspect of left pontomedullary junction measuring 6.6 × 8 × 8.7 mm with no post contrast enhancement (yellow arrow)
Figure 3
Figure 3
(a) Nerve biopsy of greater auricular nerve (Rt) showing intact nerve within perineurium with multiple ill-formed granulomas bordered by epithelioid cells, lymphocytes, and giant cell reaction (10×, H and E stain). (b) Nerve biopsy revealing large areas of caseous necrosis surrounded by granuloma characteristic of segmental necrotizing granulomatous neuritis (10×, H and E stain)
See this image and copyright information in PMC

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