Main objective: - Obtain long-term clinical follow-up data in PLS patients to investigate the reliability of the diagnosis PLS. - Obtain data on cognitive functioning to investigate the possible relation between PLS and FTD.Secondary objectives: -…
ID
Source
Brief title
Condition
- Neuromuscular disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Reliability of the diagnose will be tested by use of anamnesis, neurological
examination and genetic research.
Cognitive and behavioural functioning will be assessed and explored using the
ECAS, ALS-FTD-Q, FAB and HADS rating scales. Neuropsychological evaluation will
be performed.
Secondary outcome
Physical deterioration and functioning of the patient will be measured by use
of the MRC-scores used during the neurological investigation and the ALS-FRS
scale.
Background summary
We know that the reliability of the diagnosis PLS(primary lateral sclerosis)
increases with the duration of the symptoms. As PLS can develop into ALS
(amyotrophic lateral sclerosis), which has a worse outcome, it is beneficial
for the patient to have more certainty about the diagnose. Possibly long term
follow-up data can create insight in long-term conversion to ALS (or other
diagnoses).
Also, recently multiple cases of FTD (frontotemporal dementia) and PLS have
been identified. As a relation between ALS and FTD is already well established,
we need to further examine the relation between PLS and FTD.
Study objective
Main objective:
- Obtain long-term clinical follow-up data in PLS patients to investigate the
reliability of the diagnosis PLS.
- Obtain data on cognitive functioning to investigate the possible relation
between PLS and FTD.
Secondary objectives:
- Investigate whether the ALS and FTD related genetic mutation C9orf72, is also
prevalent in a PLS population (with or without cognitive deficits).
- Save samples for future research.
Study design
The study has an observational character and consists of a single follow-up of
PLS patients who were studied in the period of 2003-2006.
Study burden and risks
Participation is limited to one visit with a duration of approximately 2 *
hours. Patients will be visited at their residential location, or in the UMC
Utrecht if preferred. Neurological investigation, the FVC (a short pulmonary
functioning test) and multiple interviews and a neuropsychological evaluation
will be performed. Two tubes of 10-ml blood will be drawn in total.
Heidelberglaan 100
Utrecht 3584CX
NL
Heidelberglaan 100
Utrecht 3584CX
NL
Listed location countries
Age
Inclusion criteria
Participation of the former PLS related study of 2003-2006
Age 18-years or above.
Exclusion criteria
None.
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL57082.041.16 |