2023
DOI: 10.1016/j.nrleng.2022.08.003
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Cerebellar hypermetabolism: an alternative marker for diagnosis of paraneoplastic cerebellar degeneration

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“…36 It is estimated that up to 20% of patients with cerebellar ataxia who have an otherwise negative workup may have an autoimmune syndrome and lack a confirmatory diagnostic test, but the characteristic development of subacutely progressive gait ataxia (ie, developing over weeks, reaching a nadir at 6 months, with initially normal MRI but developing midline vermian atrophy) is very typical of all of these syndromes, and initial cerebellar hypermetabolism on PET followed by later hypometabolism has been reported. 11,54 Downbeat nystagmus (especially vibration-induced nystagmus) is a recently described manifestation that appears more often in paraneoplastic cerebellar syndromes compared with nonparaneoplastic syndromes. 11,55 When atrophy is already present on initial imaging, a nonimmune cause is much more likely (eg, toxic, genetic, metabolic) (TABLE 7-6) (CASE 7-3).…”
Section: Iglon5-associated Syndromesmentioning
confidence: 99%
“…36 It is estimated that up to 20% of patients with cerebellar ataxia who have an otherwise negative workup may have an autoimmune syndrome and lack a confirmatory diagnostic test, but the characteristic development of subacutely progressive gait ataxia (ie, developing over weeks, reaching a nadir at 6 months, with initially normal MRI but developing midline vermian atrophy) is very typical of all of these syndromes, and initial cerebellar hypermetabolism on PET followed by later hypometabolism has been reported. 11,54 Downbeat nystagmus (especially vibration-induced nystagmus) is a recently described manifestation that appears more often in paraneoplastic cerebellar syndromes compared with nonparaneoplastic syndromes. 11,55 When atrophy is already present on initial imaging, a nonimmune cause is much more likely (eg, toxic, genetic, metabolic) (TABLE 7-6) (CASE 7-3).…”
Section: Iglon5-associated Syndromesmentioning
confidence: 99%