The Skin as a Window to the Central Nervous System in Frontotempolar Lombar Degeneration

Participant with Frontotemporal Lobar degeneration equally distributed into behavioral variant of frontotemporal dementia (bvFTD), language variant with primary progressive aphasia (PPA) and motor presentations with atypical parkinsonian disorders (corticobasal degeneration-CBD and progressive supranuclear palsy-PSP) and motoneuron disorder (amyotrophic lateral sclerosis -ALS) will be included at Nantes University Hospital during a period of 24 months.

Healthy volunteers will be included as comparative group. A skin biopsy and venous blood samples will be collected for all participants.

Inclusion criteria

(patients):

• Adressed or followed at memory clinic or ALS expert center at Nantes university hospital.
• Aged 50-75 years
• Fulfilling current diagnosis criteria for one of the disorder: vcfFTD, non-Alzheimer PPA (semantic or non fluent), DCB or PSP,ALS
• MMSE ≥ 18
• Membership of social security scheme

Inclusion criteria

(healthy volunteers):

• No history of neurological disease, diabetes, or alteration/damage of peripheral nervous system
• Aged 50-75 years Paired to at least one patient on age (less or more 5 years)
• MOCA ≥ 26
• Membership of social security scheme

Non inclusion Criteria (Patients and healthy volunteers):

• Concomitting conditions affecting the peripheral nervous system such as but not limited to diabetes, renal failure, thyroid disorder, vitamin B12 deficiency, acute and chronic inflammatory diseases HIV, syphilis
• Know allergy to local anesthetic
• Known coagulopathy
• Pregnant women or breastfeeding women
• Person under court protection sous sauvegarde de justice
• Person under guardianship
• Inability to sign an informed consent

Non inclusion criteria (Patients) • Patient with neurological disease other than FTLD

Non inclusion criteria (Healthy volunteers) :

• Evidence of neurological disorder at the inclusion including but not limted to FTLD, Parkinson disease, Alzheimer disease, lewy body dementia, Huntington disease, systemic lupus erythematosus multiple sclerosis; learning disabilities, mental retardation, severe hypoxic brain injuries, brain trauma with permanent cognitive impairments.