Bilateral Facial Palsy in Lymphomatous Meningitis.

A 9-year-old boy presented with bilateral lower-motor-neuron facial-palsy, progressive hearing-loss, and radiating-pains in legs for 2 months. Neuroimaging revealed bilateral VII and VIII cranial-nerve enhancement with arachnoiditis [Figure 1]. Cerebrospinal fluid (CSF) was meningitic [70 cells (60% lymphocytes, no blasts), protein: 194 mg/dl, sugar: 59 mg/dl (RBS: 120 mg/dl)]. Etiological workup for chronic meningitis—bacterial, fungal, viral, tuberculous, sarcoid, and lupus—was noncontributory. CSF serology was positive for Lymes disease (IgM). The child received ceftriaxone and doxycycline for a month and methylprednisolone pulse for arachnoiditis with marginal improvement. While on antibiotics, he developed paraparesis and bowel-bladder incontinence. Repeat CSF was meningitic (without malignant cells). Serum lactate dehydrogenase (LDH) was elevated (2234 IU/l). A whole-body PET scan revealed a presacral and left paravertebral mass with increased uptake [Figure 2]. Also noted were multifocal uptake in meninges (at D1, D2, D12, L1, L2 level) and multiple areas of skeletal involvement. Ultrasound-guided biopsy of the mass revealed lymphoid blast cells with immunohistochemistry corroborating with T-cell anaplastic lymphoma. The child received chemotherapy but succumbed to febrile neutropenia. Parents refused autopsy.

Figure 1

MRI brain and spine of the index patient. (a) Contrast-enhanced axial T1 weighted MRI brain at the level of posterior fossa shows enhancement of bilateral 7th and 8th nerve complexes (arrows in a). (b) Contrast-enhanced sagittal T1 weighted MRI spine shows dense enhancement of the CSF arachnoid space in the lumbar region (arrow in b) consistent with arachnoiditis

Figure 2

FDG-PET (whole body) of the index patient. (a) FDG PET-CT maximum intensity projection image showing increased FDG uptake in the pelvic region. (b and c) Coronal and axial fused PET-CT images showing presacral soft tissue mass in the left paravertebral region (arrowhead in b) and pelvis (arrow in c) with increased FDG uptake

Progressive cranial nerve involvement and arachnoiditis necessitates investigation for malignancy (primary or systemic) besides infectious, inflammatory, and connective-tissue disorders.[1] T-cell lymphoma is notorious for neurological involvement.[2] It may present as tumoral masses at unusual sites.[34] Elevated LDH levels may be a diagnostic clue; hence, they should be a part of the etiological workup for arachnoiditis.

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