Bing Neel syndrome presenting as isolated cranial nerve palsies - a case report.

Background and aims: Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the central nervous system. Case report: 64-year-old man, presented with 4 years history of slowly progressive diplopia and ptosis of eyes. Examination showed left oculomotor (internal and external ophthalmoplegia), with trochlear, abducens, and right partial oculomotor and abducens nerve involvement. Evaluation showed anemia of hemoglobin 10.7 g/dL, raised erythrocyte sedimentation rate of 120 mm/h and plasma albumin:globulin reversal. Serum protein electrophoresis showed a paraprotein peak in the early gamma region with elevated IgM level (3810 mg/dL) and elevated free kappa light chain level (70.1 mg/L). Bone marrow aspiration from posterior iliac crest revealed mature small lymphocytes with positive immunohistochemical markers of CD5, CD10 negativity and MYD88 mutation positivity suggestive of WM. Patient was treated with bendamustine and rituximab regimen, with no neurological improvement at the end of one year. Conclusion: This case expands spectrum of paraproteinemic neuropathy to include cranial nerve palsy. Thus, plasma cell dyscrasias have to be considered in patients with isolated ophthalmoparesis especially in elderly patients, even with other comorbidities such as diabetes mellitus.

Cervical and Ocular Vestibular Evoked Myogenic Potential Recovery in Susac Syndrome: A Case Report.

PURPOSE: Susac syndrome (SS) is a rare autoimmune disorder that affects the brain and the retina and causes unilateral or bilateral sensorineural hearing loss. Although vestibular dysfunction is reported in SS, limited information is available underlying the vestibular pathophysiology. METHOD: The diagnosis of SS was established based on symptoms and diagnostic tools such as magnetic resonance imaging and fundus fluorescein angiography. The audiovestibular evaluation was done on the seventh day of admission (Session 1) into the emergency unit, whereas the second and third evaluations were done at 3-month (Session 2) and 8-month (Session 3) follow-ups after discharge, respectively. The audiovestibular test battery consisted of routine audiological tests, auditory brainstem response, and vestibular evoked myogenic potentials (VEMPs; both cervical and ocular). RESULTS: We found unilateral sensorineural hearing loss and absent cervical VEMPs(cVEMPs) when testing the left ear during Session 1. In the following sessions, the unilateral hearing loss did not recover; however, cVEMPs were present bilaterally. The ocular VEMPs showed an increase in amplitude during Sessions 2 and 3. CONCLUSIONS: SS can selectively disrupt auditory and vestibular structures. It may present with unique findings of audiovestibular tests. A detailed audiovestibular evaluation may be essential in patients with SS. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.21513843.

PET-MRI in idiopathic inflammatory myositis: a comparative study of clinical and immunological markers with imaging findings.

BACKGROUND: We sought to determine the utility of PET-MRI in diagnosing Idiopathic Inflammatory Myositis (IIM), and look for association between FDG uptake and clinical, pathological and laboratory parameters. METHODS: A retrospective, observational study was conducted on IIM patients having positive serum autoantibodies and who underwent PET-MRI (3-Tesla SIEMENS Biograph MR scanner) between 2017 and 2021. Thirty patients who underwent PET-MRI to detect systemic metastasis without muscle involvement formed the control group. RESULTS: In the IIM cohort, female: male sex ratio was 1.73, mean age at diagnosis was 40.33 years, and the mean duration of illness was 7 months. 33.33% of patients had severe limb weakness. Mi2B (43.33%), Mi2A (43.33%), PL-7(10%), PL-12(6.67%), SRP (16.67%), Tif1gamma (3.33%), NxP2 (3.33%), Ro-52(40%), PM-Scl, U1-RNP, ANA (26.67%) were the serum autoantibodies identified. Using SUV max Ratio to quantify FDG uptake, PET-MRI showed a sensitivity of 100% with 93.3% specificity in diagnosing IIM.FDG uptake was maximum in proximal lower limb region followed by proximal upper limb. Multivariate regression analysis showed that the severity of muscle weakness, serum Mi2B antibody positivity and serum creatinine kinase levels had a significant positive correlation with FDG uptake (value of 0.005, 0.043, 0.042, respectively for whole-body FDG uptake). FDG uptake also showed good correlation with histopathological features and muscle MRI, but there was no significant association with treatment response. Three female patients in our cohort had primary malignancy involving the breast, uterus, and cervix. CONCLUSIONS: PET-MRI is a promising diagnostic modality for IIM. PET-MRI reflects the severity of muscle inflammation, showing good association with various clinical/laboratory parameters, histopathology, and muscle MRI. Parameters associated with severe muscle inflammation in PET-MRI-clinical severity of muscle weakness, Mi2B positivity, and serum creatine kinase levels-may be used as clinical/laboratory markers of disease severity in IIM. PET-MRI has the added advantage of detection of systemic malignancy.