Table 1.

Summary of clinical presentations

	Case 1	Case 2	Case 3
Age of onset	72	49	64
Duration (months)	18	25	29
Clinical appearance (syndrome)	MND/PSP/FTLD	FTLD	FTLD/PSP
Central motoneuron dysfunction	Hyperreflexia	Generalized spasticity	Right-sided spasticity
Periferal motoneuron dysfunction	Yes, EMG proven	Interosseal amyotrophy	No
Dysarthria/dysphagia	Yes, prominent	Yes, later	Yes-early/prominent
Early falls	Yes	Yes	Yes
Eyelid opening apraxia	Early	Late	Present
Oculomotoricity, conjugate ocular pursuit movements	Abnormal/Voluntar movements reduced both horisontally and vertically	Initially normal, reduced mainly vertically, but after one year	Reduced mainly vertically
Saccades	Slow, hypometric	Slow, hypometric	Slow, hypometric
Bradykinesia	Axial predominance	Axial and limbs	Axial predominance
Rigidity	Axial predominance	Axial and limbs	Axial predominance
Tremor	No	No	No
Dystonia	No	Facial movements, neck anteflexion and lateroflexion	Late - retrocollis
Response to L-dopa	Poor	Poor	Non
Executive dysfunction	Prominent	Prominent	Prominent
Insight	Lacking	Present at the beginning	Lacking
Frontal release signs	Grasping/spastic laughter/disinhibited behavior	Behavioral changes, dysexecutive syndrome	Behavioral changes
Memory problems	Minimal	Minimal, effect of cueing	Impaired
Visuospatial dysfunction	Impaired	Impaired	Impaired
Gait apraxia	Early feature	Present early	Early
Autonomic function	N/A	Not present	Not present
	Case 1	Case 2	Case 3
MRI	Frontotemporal atrophy with left predominance	Generalized atrophy with mild predominance periventriculary and frontal	Frontotemporal and brainstem atrophy
Cerebrospinal fluid	Normal	Not done	Normal
EMG	Probable ALS according to El Escorial criteria, normal conduction	Not done	Normal conduction, no denervation
Pathological diagnosis	FTLD-TDP/MND	FTLD-TDP/MND	FTLD-TDP/MND