Contributed by Luiz Felipe R. Vasconcellos1 ; Alexandre M. Cunha 2,3 ; Victor Hugo R. Marussi 4 ; Nathalie H. Silva Canedo 5 ; Leila M. C. Chimelli 1,5
1 Division of Neurology, Instituto de Neurologia Deolindo Couto, Federal University of Rio de Janeiro, Brazil,
2 Division of Neurosurgery, Department of Surgery, Hospital Universitário Clementino Fraga Filho, Federal University of Rio de Janeiro, Brazil,
3 Division of Neurosurgery, Department of Surgical Specialties, Hospital Universitário Pedro Ernesto, State University of Rio de Janeiro, Brazil,
4 Division of Neuroradiology, Medimagem, Hospital Beneficência Portuguesa, São Paulo, Brazil,
5 Division of Neuropathology, Department of Pathology, Hospital Universitário Clementino Fraga Filho, Federal University of Rio de Janeiro, Brazil
A previously healthy 20-year-old man developed progressive hearing loss for the last 3 years, evolving to tetraparesis with sphincter impairment. There is no history of fever, headache or vision loss. Neurologic examination demonstrated global spastic tetraparesis, more severe in lower limbs, with evident pyramidal signs (tetrahyperreflexia, clonus and bilateral Babinski sign) and severe bilateral hearing loss. Evaluation of sensibility and coordination was not reliable due to motor deficits and hearing loss.
Cranial and spinal MRI showed diffuse thickening and enhancement of pachymeninges throughout the posterior fossa, cervical, thoracic and lumbar spine. It involved cranial nerves and nerve roots and compressed the cervicomedullary transition, cervical and thoracic spinal cord (Figures 1, 2 and 3). Sagittal T2-weighted image (Figure 3) showed marked hypointense signal of dura mater, reflecting high cellularity and an area of subdural hemorrhage. Computed tomography (CT) of the chest, abdomen and pelvis were normal. Spinal fluid analysis revealed: 290 leukocytes (90% of lymphocytes and 10% mononuclear); protein: 3.720 mg/dl; glucose: 17 mg/dl; VDRL, anti-HIV, anti-HTLV 1 and 2, PCR for BK, cultures for bacterial, BK and fungi were all negative.
The patient was submitted to posterior fossa decompression with partial resection of the expansive lesion at the cranio-cervical level. Suboccipital craniotomy with removal of the posterior arch of C1 was performed and showed a thick dura mater. The dura was opened disclosing two major lesions at the level of the cranio-cervical junction, which compressed the cervicomedullary structures and enclosed the spinal roots of the accessory nerves. A partial removal of these lesions was performed followed by a fascia lata duroplasty to expand the volume of posterior fossa. The immediate postoperative course was uneventful and later the patient developed dysautonomia and pulmonary infection that progressed to sepsis and death.
Histopathological examination of the surgical specimen showed a densely inflamed tissue (Figures 4, 5 and 6) intermingled with thick collagen bands. The areas of inflammatory infiltrate were rich in lymphocytes, plasma cells and histiocytes (Figures 5 and 6). Sometimes these cells appeared to form granulomas, but were negative for acid fast bacilli and fungi. Lymphocytes were positives for CD3 and CD20, plasma cells were positives for CD138 (Figures 7 and 8) and histiocytes were positive for CD68 and negative for CD1A. Reactive meningothelial cells simulating giant cell were positive for the anti-EMA antibody. Immunoreaction for IgG4 and Alk were negative. Figures 7 and 8 show that the relative density of CD138 cells, corresponding to plasma cells, does not allow the diagnosis of plasmocytoma or IgG4 related disease. What is your diagnosis?