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OBJECTIVE: Susac syndrome (SuS), multiple sclerosis (MS), and primary angiitis of the central nervous system (PACNS) present diagnostic challenges due to overlapping clinical features. We aimed to enhance diagnostic precision by developing the SPAMS (SuS, PACNS, MS) score, a practical radiological tool. METHODS: This multicenter study included 99 patients (43 SuS, 37 MS, 19 PACNS) from South American countries. Relevant MRI features were identified through an elastic-net model determined key variables. RESULTS: The SPAMS score assigned 2 points for snowball lesions, 1 point for spokes-like lesions, or if there are more than 4 lesions in the corpus callosum, corpus callosum involvement, or cerebellar involvement. It subtracted 1 point if gadolinium-enhancing lesions or 4 points if Dawson's fingers are present. Bootstrapping validated the optimal cutoff at 2 points, exhibiting a diagnostic performance of area under the curve = 0.931, sensitivity = 88%, specificity = 89%, positive predictive value = 88%, negative predictive value = 89%, and accuracy = 88%. INTERPRETATION: When specific MRI findings coexisted, the SPAMS score differentiated SuS from MS and PACNS. Access to MRI and standard protocol sequences makes it a valuable tool for timely diagnosis and treatment, potentially preventing disability progression and severe clinical outcomes. ANN NEUROL 2024.
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BACKGROUND: : Pregabalin (PGB) is an L-type, voltage-dependent, calcium-channel blocker, useful for the treatment of neuropathy pain and some forms of seizures. We report the case of a 64-year-old woman who developed full-blown parkinsonism after PGB administration. CASE REPORT: : A female patient who developed diabetic sensory-motor polyneuropathy began treatment with gabapentin 300 mg plus amitriptyline 25 mg at the age of 56 years, with good tolerance. Eight years later, PGB 150 mg was added, after 3 months she developed a parkinsonian-like syndrome with axial symptoms, bilateral symmetric postural tremor, bradykinesia, and rigidity in both upper and lower limbs. Unified Parkinson's Disease Rating Scale motor score was 27/108 points at this time. Clear link to PGB introduction as well as symmetric limb involvement and symptom intensity made us suspect drug-induced parkinsonism. Pregabalin was withdrawn. Six months later, the patient had almost completely recovered; her Unified Parkinson's Disease Rating Scale motor score dropped to 4/108; and 3 months after that, it was 0. CONCLUSION: : Although some cases of tremor were reported in clinical trials on PGB, to our knowledge, this is the first report of full-blown parkinsonism associated with PGB use. Vigilance of PGB-treated patients is recommended.