Table 1.

Clinical characteristics of pathologically proven cortical–basal ganglionic degeneration (CBGD)

PatientAge at death, ySexDuration, yPresentationExtrapyramidal signsCognitive impairmentOther featuresClinical diagnosisOriginal neuropathological diagnosis
* Patients reported by Riley et al.11
† Patients reported by Bergeron et al.2
169F4Confusion Disorientation Memory lossRigidity, bradykinesia L > R side after 1 yearEarlyMute after 1 yearADDementia unclassified vs atypical Pick’s
278M5Bizarre behavior Memory loss DisorientationLate difficulty walkingEarlyADDementia unclassified
376M6Memory loss Speech disorder progressing rapidly to mutismLate rigidity and bradykinesiaEarlyADAtypical Pick’s
470F6Memory loss Disorientation Confusion Loss of interestsBilateral rigidity 1 year after onsetEarlyADDementia unclassified
5*62F10Action tremor L hand progressing to alien limbL-sided bradykinesia, rigidity 4 years after onsetNoCortical sensory loss Hyperreflexia L side L Babinski Ocular and limb apraxia Dysarthria Stimulus sensitive myoclonusCBGDCBGD
664M7Withdrawn, forgetful ApathyNone specifiedEarlyADDementia unclassified
7*68M7Difficulty controlling L hand progressing to alien limb Tripping L footL > R-sided bradykinesia, rigidity L arm action tremorLateStimulus sensitive myoclonus L-sided cortical sensory loss and apraxia Ocular apraxia DysarthriaCBGDCBGD
873F7Withdrawn Loss of interests Decreased speech output Early preserved memoryNone specifiedYesAtypical AD (Frontotemporal dementia)Atypical Pick’s disease
977M5Memory loss Speech difficultyShuffling gait 2 years before deathEarlyADCBGD
1072M9Speech disorder progressing to anarthria6 years after onset right-sided bradykinesia and rigidityLateStimulus sensitive facial myoclonus Buccolingual apraxia Apraxia of gaze and limbs DysphagiaCBGDCBGD
1171F4Behavior changes Episodic confusion Disorientation1 year after onset bradykinesia, rigidity, shuffling gaitEarlyAlcoholic with hepatic cirrhosisDementia with parkinsonismPick’s + Wernicke’s encephalopathy
1268M6Personality changes Hypersexual Irritability4 years after onset bradykinesia, rigidity, and shuffling gaitEarlyFrontotemporal dementiaCBGD
1364F3Difficulty controlling L arm and leg Alien limbMarked bradykinesia, rigidity L > R sideEarlyEmotional liability Stimulus sensitive Myoclonus L > R armCBGDCBGD