Neurophysiological features in relation to clinical signs in clinically diagnosed corticobasal degeneration

D. Monza, C. Ciano, V. Scaioli, P. Soliveri, F. Carella, G. Avanzini, F. Girotti

Research output: Contribution to journalArticlepeer-review


We investigated the association between clinical and neurophysiological characteristics in patients with a clinical diagnosis of probable corticobasal degeneration (CBD), and searched for neurophysiological features supporting the diagnosis in life. Ten patients with clinically probable CBD underwent comprehensive neurological evaluation and brain MRI. Long latency reflexes (LLR), upper limb somatosensory (SEP) and motor evoked (MEP) potentials were recorded. The mini-mental state examination (MMSE), the phonemic verbal fluency test (PVFT) and the De Renzi ideomotor apraxia test were also performed. Polygraphic EEG was performed in the six patients with myoclonus. The SEP N30 frontal component was absent bilaterally in four patients, was absent on the left side in one, and had increased latency in other three. MEPs were abnormal in four patients (three had prolonged central motor conduction time, one of whom also had increased MEP threshold, and one had increased MEP threshold). All six patients with myoclonus had enhanced LLRs at rest, which were also of abnormally increased amplitude during motor activation; latencies were generally shorter than in classic cortical reflex myoclonus. On back-averaging, no EEG spikes time-locked to EMG activity were found in any myoclonus patient. Five patients were demented by MMSE, eight had ideomotor apraxia scores in the ideomotor apraxia range and five had defective verbal fluency. Brain MRI revealed asymmetric cortical atrophy in all patients, particularly evident frontoparietally. Neurophysiological techniques, particularly LLR, can assist CBD diagnosis especially in patients with myoclonus. Patients with evident parkinsonism had greater SEP N30 (frontal) abnormalities, while most patients with marked paresis had slower MEP times.

Original languageEnglish
Pages (from-to)16-23
Number of pages8
JournalNeurological Sciences
Issue number1
Publication statusPublished - Apr 2003


  • Corticobasal degeneration
  • Myoclonus
  • Neurophysiology long latency reflexes

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology


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