Table 1.

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

Patient	Age at death, y	Sex	Duration, y	Presentation	Extrapyramidal signs	Cognitive impairment	Other features	Clinical diagnosis	Original neuropathological diagnosis
* Patients reported by Riley et al.11
† Patients reported by Bergeron et al.2
1	69	F	4	Confusion Disorientation Memory loss	Rigidity, bradykinesia L > R side after 1 year	Early	Mute after 1 year	AD	Dementia unclassified vs atypical Pick’s
2	78	M	5	Bizarre behavior Memory loss Disorientation	Late difficulty walking	Early		AD	Dementia unclassified
3	76	M	6	Memory loss Speech disorder progressing rapidly to mutism	Late rigidity and bradykinesia	Early		AD	Atypical Pick’s
4	70	F	6	Memory loss Disorientation Confusion Loss of interests	Bilateral rigidity 1 year after onset	Early		AD	Dementia unclassified
5^*	62	F	10	Action tremor L hand progressing to alien limb	L-sided bradykinesia, rigidity 4 years after onset	No	Cortical sensory loss Hyperreflexia L side L Babinski Ocular and limb apraxia Dysarthria Stimulus sensitive myoclonus	CBGD	CBGD
6	64	M	7	Withdrawn, forgetful Apathy	None specified	Early		AD	Dementia unclassified
7^*	68	M	7	Difficulty controlling L hand progressing to alien limb Tripping L foot	L > R-sided bradykinesia, rigidity L arm action tremor	Late	Stimulus sensitive myoclonus L-sided cortical sensory loss and apraxia Ocular apraxia Dysarthria	CBGD	CBGD
8	73	F	7	Withdrawn Loss of interests Decreased speech output Early preserved memory	None specified	Yes		Atypical AD (Frontotemporal dementia)	Atypical Pick’s disease
9^†	77	M	5	Memory loss Speech difficulty	Shuffling gait 2 years before death	Early		AD	CBGD
10^†	72	M	9	Speech disorder progressing to anarthria	6 years after onset right-sided bradykinesia and rigidity	Late	Stimulus sensitive facial myoclonus Buccolingual apraxia Apraxia of gaze and limbs Dysphagia	CBGD	CBGD
11	71	F	4	Behavior changes Episodic confusion Disorientation	1 year after onset bradykinesia, rigidity, shuffling gait	Early	Alcoholic with hepatic cirrhosis	Dementia with parkinsonism	Pick’s + Wernicke’s encephalopathy
12^†	68	M	6	Personality changes Hypersexual Irritability	4 years after onset bradykinesia, rigidity, and shuffling gait	Early		Frontotemporal dementia	CBGD
13	64	F	3	Difficulty controlling L arm and leg Alien limb	Marked bradykinesia, rigidity L > R side	Early	Emotional liability Stimulus sensitive Myoclonus L > R arm	CBGD	CBGD