Primary Central Nervous System Vasculitis Mimicking Susac Syndrome And Multiple Sclerosis With Long-term Remission And Spontaneous Resolution Of Lesions: A Case Report
Koseki, A., Suzuki, Y., Uchida, S., Morishita, N., Hokazono, Y., Kuriki, K., Yamamura, Y., Yoshida, M., & Sakai, N. (2024). Primary Central Nervous System Vasculitis Mimicking Susac Syndrome and Multiple Sclerosis With Long-Term Remission and Spontaneous Resolution of Lesions: A Case Report. Cureus, 16(7), e64358. https://doi.org/10.7759/cureus.64358
View Original Source →Abstract
Primary central nervous system vasculitis (PCNSV) is an angiitis localized to the central nervous system (CNS), with various manifestations and no specific biomarkers. Herein, we report a case of PCNSV that presented with an unusual course. A 40-year-old Japanese male developed inner ear symptoms and visual field disturbances. Later, at 42 years of age, the patient developed right hemiparesis and was diagnosed with multiple sclerosis (MS). He received methylprednisolone pulse therapy, which improved his symptoms and resolved most brain lesions. Subsequently, he did not visit the hospital for 13 years, during which time he experienced no relapse. At 55 years of age, he presented to our hospital with fatigue and dizziness. Susac syndrome was suspected because of sensorineural hearing loss and snowball lesions in the corpus callosum. Some of the brain lesions resolved spontaneously. A biopsy was performed on a right frontal lobe lesion, which revealed vasculitis with fibrinoid necrosis, no demyelinating lesions, no amyloid positivity, and no infiltration of atypical lymphocytes. With no evidence of vasculitis in other organs, the patient was diagnosed with PCNSV. The patient was treated with methylprednisolone pulse therapy, followed by oral prednisolone (1 mg/kg/day). The prednisolone was tapered off, and no relapse of symptoms or new lesions on magnetic resonance imaging (MRI) were noted. As observed in this case, even in a scenario suggestive of Susac syndrome or multiple sclerosis, PCNSV should be considered a differential diagnosis and confirmed via brain biopsy.
Case Details
Disease Location
Brain/cns
Personal Characteristics
40-year-old japanese male. History of atopic dermatitis and bronchial asthma
Clinical Characteristics
- presented with vertigo, nausea, tinnitus, hearing loss in the left ear, left nasolateral visual field defect, and occipital headache without specific triggers. - csf showed pleocytosis with 31 cells/μl (98% mononuclear cells) and a protein level of 89 mg/dl, positive for oligoclonal bands. - MRI showed multiple t2 hyperintense lesions in the dorsal c3/4 cervical spinal cord and cerebral white matter with contrast enhancement of the cervical spinal cord lesions and some cerebral lesions - he was considered to have ms with atypical findings. At age 42. - the patient developed mild right upper and lower extremity paralysis, without any specific triggers - csf analysis showed pleocytosis with 50 cells/μl (89% mononuclear cells), a protein level of 95 mg/dl, and positive oligoclonal band - flair images showed hyperintense lesions without contrast enhancement in the right medulla oblongata, left cerebellum, ventral side of the pons, right optic chiasm, bilateral putamen, left cerebral peduncle, right caudate nucleus, right globus pallidus, left thalamus, and right splenium of the corpus callosum; subcortical white matter in the bilateral occipital, right frontal, and left parietal lobes; and a 20-mm-diameter rounded lesion in the subcortical white matter in the left parietal lobe. - methylprednisolone pulse therapy was started. His symptoms improved, and all hyperintense lesions on flair images, except for the lesions in the right caudate nucleus and the left cerebral peduncle, had resolved. At age 55: - consulted for continuous malaise and dizziness for three months without any specific trigger. - pe: atopic dermatitis rash - cerebrospinal fluid showed an initial pressure of 90 mmh2o, pleocytosis with 33 cells/μl (89% mononuclear cells), a protein level of 89 mg/dl, glucose level of 48 mg/dl, immunoglobulin g index of 0.71, and positive oligoclonal bands - MRI of the head performed on day showed a 30-mm-diameter lesion with edematous lesions in the right frontal lobe, which were t1 and t2 hyperintense lesions without contrast enhancement. Four small lesions with the same intensities as the 30-mm lesion were located anterior and superior to this lesion. Additionally, snowball lesions in the corpus callosum and t2 and flair hyperintense lesions in the white matter of the right occipital lobe and ventral side of the right cerebellar hemisphere were noted. T2*-weighted images revealed scattered microhemorrhagic changes in the subcortex - at day 21 of hospitalization, MRI showed that some of the snowball lesions in the corpus callosum and the right cerebellar lesion had resolved, whereas some of the remaining snowball lesions had decreased in size. The t1 and t2 hyperintense lesions in the right frontal lobe remained unchanged. However, the surrounding edematous lesions were enlarged. - at day 23 of hospitalization, a biopsy was performed on a lesion in the right frontal lobe. Hemorrhagic necrotic nests surrounded by granulation tissue with hemosiderin deposits and small vessels with fibrinoid necrosis were scattered were found. - he was finally diagnosed with pcnsv. - (after image remission) the patient was treated with methylprednisolone pulse therapy, followed by oral prednisolone (1 mg/kg/day). The prednisolone was tapered off to 5 mg in combination with oral azathioprine (50 mg/day).
Remission Characteristics
- at age 40 his symptoms improved without immunotherapy. - at age 55 at day 21 of hospitalization, MRI showed that some of the snowball lesions in the corpus callosum and the right cerebellar lesion had resolved, whereas some of the remaining snowball lesions had decreased in size. - at age 55 at day 76 of hospitalization MRI (after biopsy and pretreatment) revealed that the edematous lesions around the right frontal lobe lesion, which had enlarged before the brain biopsy, had decreased in size, and the remaining snowball lesions had resolved
Treatment & Mechanisms
Clinical Treatment
Methylprednisolone pulse therapy (at age 42) methylprednisolone pulse therapy, followed by oral prednisolone (1 mg/kg/day). The prednisolone was tapered off to 5 mg in combination with oral azathioprine (50 mg/day). (at age 55 after image remission)