Progressive ataxia and palatal tremor (PAPT) is a rare syndrome combining palatal tremor and progressive cerebellar ataxia. PAPT is classified into familial and sporadic forms, but etiopathogenesis remains largely unclear.1 Recent neuropathological and positron emission tomography studies2, 3 revealed tau deposition in brains of patients affected by sporadic PAPT, suggesting it may be a novel tauopathy. Another condition, termed “oculo-palatal tremor and tardive ataxia”4 has been described as a rare complication of large brainstem lesions with onset from months to years following a monophasic lesion.4, 5 It has been suggested that this syndrome could be considered as an acquired or structural form of PAPT (sPAPT).1 Although the progressive course of sPAPT suggests a neurodegenerative phenomenon,4, 5 to date no clear evidence in support of this hypothesis has been presented.
Increased Cerebrospinal Fluid Biomarkers of Neurodegeneration in Acquired Progressive Ataxia and Palatal Tremor Following a Static Lesion: A Case Report
Fazio, Carlo;Comolli, Davide;Calculli, Alessandra;Grillo, Piergiorgio;Cosentino, Giuseppe;Pisani, Antonio
2024-01-01
Abstract
Progressive ataxia and palatal tremor (PAPT) is a rare syndrome combining palatal tremor and progressive cerebellar ataxia. PAPT is classified into familial and sporadic forms, but etiopathogenesis remains largely unclear.1 Recent neuropathological and positron emission tomography studies2, 3 revealed tau deposition in brains of patients affected by sporadic PAPT, suggesting it may be a novel tauopathy. Another condition, termed “oculo-palatal tremor and tardive ataxia”4 has been described as a rare complication of large brainstem lesions with onset from months to years following a monophasic lesion.4, 5 It has been suggested that this syndrome could be considered as an acquired or structural form of PAPT (sPAPT).1 Although the progressive course of sPAPT suggests a neurodegenerative phenomenon,4, 5 to date no clear evidence in support of this hypothesis has been presented.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.