Idiopathic late onset cerebellar Ataxia (iloca), and cerebellar plus syndrome

Abstract

Spinocerebellar Ataxias (SCAs), also called spinocerebellar degenerations, comprise a large group of slowly progressive neurodegenerative diseases characterized by truncal and limb Ataxias as the cardinal clinical features. Sporadic degenerative Ataxias include idiopathic late-onset cerebellar Ataxia (ILOCA), ILOCA with cerebellar-plus syndrome, and multiple system atrophy (MSA). The clinical presentations of ILOCA (CCA) are characterized by late ages at onset with slow progression and pure cerebellar Ataxia with markedly ataxic gait with relative preservation of coordination in the upper limbs. ILOCA with cerebellar-plus syndrome is characterized by cerebellar Ataxia together with additional neurological features including pyramidal signs, mild dementia, supranuclear ophthalmoplegia, optic atrophy, dysphagia, parkinsonism, sphincter disturbances, hypopallaesthesia, or chorea. MSA is a sporadic neurodegenerative disorder characterized by various combinations of autonomic dysfunction, cerebellar symptoms, parkinsonism, and pyramidal signs. MSA has recently been classified into two subtypes: MSA-C (characterized by cerebellar Ataxia) and MSA-P (characterized predominantly by parkinsonism). Among these sporadic neurodegenerative Ataxias, ILOCA (CCA) is characterized by slow progression rate, while patients with MSA-c show faster progression compared to other neurodegenerative Ataxias. Although there are no curative treatments available to prevent disease progression, continuous physiotherapeutic interventions in Ataxia patients are encouraged.