Corticobasal degeneration: A clinical study of 36 cases

Abstract

The presenting features and their subsequent evolution in 36 patients with pathologically proven or clinically probable corticobasal degeneration are described. The most common initial complaint was of a unilateral 'clumsy, stiff or jerky arm' (n = 20). Typically the arm was akinetic, rigid and apraxic. In about a third of these the arm was held in a striking and characteristic fixed dystonic posture. Jerking of the arm, due to action and stimulus-sensitive myoclonus accompanied these symptoms in about a third of the cases. The next most common presentation (n = 10) was difficulty walking due to clumsiness and loss of fine motor control of one leg due to apraxia or dysequilibrium, or a combination of both. Sensory symptoms in the affected arm heralded the onset of illness in three and accompanied a motor disturbance in two cases. Presentation with dysarthria or a behavioural syndrome were rare. The symptoms progressed slowly, usually involving first the ipsilateral arm and leg, but gradually spread to affect all four limbs. After a mean follow-up of 5.2 years (range 2-8 years) gait difficulties and a supranuclear ophthalmoplegia had emerged in most patients and dysarthria and pyramidal signs were common. Higher mental function was relatively preserved in most and a cortical sensory loss was evident in a quarter of cases.

Imaging-Pathologic Correlation in Corticobasal Degeneration

“…In some patients, CBD presents with dementia, behavioral abnormalities, and attention deficit in the early stage, but patients may be misdiagnosed due to a lack of cortical symptoms. [10][11][12][13][14] The above patient (case 2) may be an example of such a clinical presentation. Clinical evaluation showed little evidence of asymmetric cortical dysfunction, and neuropathology revealed only minimal cortical asymmetry.…”

Ocular motor abnormalities in neurodegenerative disorders

“…A supranuclear gaze palsy may be seen in CBD, but usually only when the disease is advanced. 76 Eye signs are rarely an early feature of MSA, but in some cases may mimic PD.…”

Clinical, Cognitive and Anatomical Evolution from Nonfluent Progressive Aphasia to Corticobasal Syndrome: A Case Report

“…In contrast, clinical definitions of corticobasal degeneration (CBD), also referred to as corticobasal syndrome (CBS; Boeve et al, 2003), emphasized motor manifestations (Rebeiz et al, 1968;Gibb et al, 1989;Riley et al, 1990;Rinne et al, 1994). Typical motor features include asymmetrical, non-L-dopa responsive extrapyramidal syndrome, myoclonus and dystonia.…”