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Status:

COMPLETED

Early Diagnosis of TTR Amyloidosis by Use of Molecular Biology

Lead Sponsor:

Assistance Publique - Hôpitaux de Paris

Conditions:

Polyneuropathy

Diagnosis

Eligibility:

All Genders

51+ years

Brief Summary

Peripheral neuropathies are diseases that affect the nervous system outside the brain and spinal cord, their prevalence is 1% in the general population, the causes are extremely varied with more than ...

Detailed Description

Study of the TTR gene by complete sequencing; search for amyloidogenic mutations of the TTR gene (according to the site http: // amyloidosismutations.com / mut-attr.php) in the laboratory of molecular...

Eligibility Criteria

Inclusion

  • The initial criteria before amendment
  • Inclusion Criteria:
  • A. Adult (\>50 years old)
  • Chronic Peripheral neuropathies (progressing since 12 months),
  • Peripheral neuropathies documentated by ENMG.
  • Chronic polyneuropathy with dysautonomia (orthostatic hypotension) without diabetes
  • Atypical CIDP (situations C, D (even with high protid content on CSF) \& E as defined by the French group for study of CIDP).
  • Disabling neuropathy (gait or balance disorder)
  • Neuropathies with upper limb onset who underwent previously CTS surgery without success.
  • SLA-like syndrome : areflexia with sensory alterations on ENMG. 6: Deterioration of SNAPs' amplitudes on NCS \> 30% in less than6 months by the same NCS laboratory Mandatory : A+B+C one of 1 to 6
  • Exclusion criteria :
  • Amyloid deposit characterized on biopsy
  • Causes of chronic polyneuropathy : Diabetes mellitus, Chronic alcoholic intoxication
  • CIDP responding to IVIg or corticosteroids (improvement by 1 point of ONLS).
  • Neuropathy associated with monoclonal gammapathy and i) anti-MAG activity or ii) POEMS syndrome or) CANOMAD syndrome.
  • Ataxic Neuropathy due to vitamine B12 deficiency
  • Ataxic Neuropathy due to IgM anti-MAG,
  • CANOMAD syndrome,
  • Ganglionopathy by Sjögren's syndrome, or paraneoplastic syndrome with Anti- Hu Antibodies, chemotherapy induced (cis-platine, oxaliplatine).
  • Positive family history of FAP or FAC
  • Proven AL amyloidosis
  • The new criteria after amendment New eligibility criteria from the 351st patient:
  • A. Adults \> 50 years old B1. Progressive axonal polyneuropathy
  • Has:
  • Deterioration of EMG sensory potentials \>30% in less than 6 months by the same electrophysiology team Where -. Clinical worsening over 6 months, i.e. + 1 ONLS point, or extension of sensory disorders (subjective, objective), or reduction in walking distance, or JAMAR -10% OR B2. Atypical chronic polyradiculoneuritis (CIDP)
  • with pure sensitive
  • pure motor
  • . Asymmetrical sensorimotor impairment predominantly in the upper limbs
  • Situations C, D, E -even at high protein content on CSF- and as defined by the French group for the study of CIDP C. Peripheral neuropathy evolving for ≥ 12 months and \< 10 years. D. Peripheral neuropathy documented by abnormal ENMG (Electroneuromyography).
  • And
  • At least one of the following criteria:
  • Chronic polyneuropathy with dysautonomia (orthostatic hypotension)
  • Disabling neuropathy (walking or balance disorders, functional impairment of the hands)
  • Unintentional weight loss of \> 5 kg in the last 5 years
  • History of operated carpal tunnel syndrome
  • Exclusion criteria A. Amyloid deposit characterized by biopsy B. Causes of chronic polyneuropathy: sensory neuropathy typical of diabetes mellitus, chronic alcohol intoxication (women: \>14 drinks/week; men \>21 drinks/week), vitamin B12 deficiency, chemotherapy (cis-Platin, oxaliplatin) C. CIDP responding to IVIG or corticosteroids (1 point improvement in ONLS) D. Peripheral neuropathy evolving for \>10 years E. Dysimmune neuropathy defined by
  • with Ac anti-MAG,
  • POEMS, CANOMAD,
  • Ganglionopathy linked to Gougerot Sjögren's syndrome, to a paraneoplastic syndrome with Anti-Hu antibodies), F. Family history of FAP or FAC (familial amyloid neuropathy or cardiomyopathy) G. AL Amyloidosis

Exclusion

    Key Trial Info

    Start Date :

    March 17 2017

    Trial Type :

    OBSERVATIONAL

    Allocation :

    ACTUAL

    End Date :

    December 10 2021

    Estimated Enrollment :

    560 Patients enrolled

    Trial Details

    Trial ID

    NCT03373370

    Start Date

    March 17 2017

    End Date

    December 10 2021

    Last Update

    December 8 2022

    Active Locations (10)

    Enter a location and click search to find clinical trials sorted by distance.

    Page 1 of 3 (10 locations)

    1

    Chu Bicetre

    Le Kremlin-Bicêtre, Kremlin Bicetre, France, 94270

    2

    CHU Martinique

    Martigues, Martinique, France, 97200

    3

    CHU Saint Etienne

    Saint-Etienne, Saint Etienne, France

    4

    CHU Grenoble

    Grenoble, France, 38043