Central pontine myelinolysis can occasionally enhance, but there were no precipitating factors, and the lesion appearances are atypical. 1Īlternative diagnoses included autoimmune or parainfectious disorders, neoplasia (particularly primary CNS lymphoma), vasculitis, and infection. The initial presentation of a steroid-responsive brainstem encephalitis with curvilinear and nodular pontocerebellar enhancement and T-cell-predominant CSF leukocytosis suggested CLIPPERS syndrome. One month later, her myelopathic symptoms had fully resolved. Our patient received further pulsed and maintenance steroids. Oligoclonal bands remained negative.Īquaporin-4 (AQP4) antibodies were negative, but serum anti-MOG immunoglobulin G1 antibodies were positive using a cell-based assay using full-length human MOG. MRI showed residual pontine changes and a new long cord lesion involving the conus ( figure, F).ĬSF studies revealed 18 lymphocytes, elevated protein (554 mg/L), and again a reactive picture without clonality on flow cytometry. Upper limb, cranial nerve, and cerebellar examination results were normal. Left leg pinprick and temperature sensation were reduced. She had a spastic paraparesis, pyramidal weakness, brisk 4-limb reflexes, crossed adductor jerks, and bilateral patellar and ankle clonus. Two weeks later, the patient developed progressive painful tightness in both legs, altered perineal sensation, difficulty climbing stairs, and transient urinary retention requiring catheterization. With full symptom resolution, steroids were slowly weaned and discontinued 5 months after initial admission. Subsequently, the clinical findings and imaging appearances improved markedly ( figure, E). (F) Five months after initial presentation, following steroid withdrawal, MRI spine shows a new longitudinally extensive lesion with intrinsic enhancement and edema within the conus medullaris, spanning 3 vertebral segments (T11–L1, arrows indicate top and bottom extent of lesion).Īs symptoms were progressing, we commenced treatment with high-dose steroids (3 days IV methylprednisolone thereafter 1 mg/kg/day prednisolone). (E) Nine weeks later, the pontine lesion no longer demonstrates contrast enhancement and there was a reduction in the extent of abnormal high T2 signal in the pons and middle cerebellar peduncles. There was no restricted diffusion and no supratentorial lesions. (C, D) T1-weighted MRI shows curvilinear, punctate, and nodular gadolinium contrast enhancement most severe adjacent to the surface but extending into the center of the pons (C, white arrow) and cerebellar peduncles (D, white arrow). There is diffuse but patchy signal change in pons (A, dark arrow), extending into the cerebellar peduncles (B, white arrow) with some associated swelling but without substantial mass effect or hydrocephalus. (A, B) T2-weighted MRI shows axial slices through pons. Angiotensin converting enzyme levels were normal, and antinuclear antibody screening was negative. Flow cytometry identified reactive T cells (CD4:CD8 ratio 3:1). Microscopy, culture, and viral PCR were negative. CSF showed 2 lymphocytes/µL and elevated protein (686 mg/L). 1 CT of the chest/abdomen/pelvis was normal. Her gait was ataxic.īrain MRI showed patchy pontocerebellar signal change ( figure, A–D), consistent with CLIPPERS. Limb reflexes were brisk, without clonus. Visual acuities and fundal appearances were normal, as were tone, power, sensation, and sphincter function. There were no constitutional symptoms suggesting systemic illness.Įxamination revealed diplopia, horizontal nystagmus on left gaze, dysarthria, and left-sided facial weakness. Shortly before admission, she developed slurred speech, gait ataxia, and double vision. Although neuromyelitis optica spectrum disorders (NMOSD) with brainstem involvement may feature in the broad differential diagnosis of CLIPPERS, this is the first report describing an overlap with the anti-MOG phenotype of NMOSD, and highlights that CLIPPERS may not be a distinct nosologic entity.Case report.Ī 36-year-old woman presented with a 2-week history of dizziness, left facial paresthesia, allodynia, and altered intraoral sensation. Five months later, she developed a longitudinally extensive spinal cord inflammatory lesion affecting mainly the conus, and had antibodies to myelin-oligodendrocyte glycoprotein (MOG). Our patient initially presented with classical clinicoradiologic features of CLIPPERS. 1 Autoimmunity has been postulated, although specific CNS antibodies have not been reported. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory brainstem syndrome of uncertain etiology, with distinct radiologic features.
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