Non-motor symptoms in ALS

Mental decline
About 50% of the pALS develop symptoms of mental decline.
There are 3 different groups:
• 49% develops no mental decline
• 45% develops a mild to moderate mental decline
• 6% develops FTLD

Mild mental decline
This mild decline is so subtle that it can only be demonstrated through neurological tests or when it causes small frustrations to the patient.

Symptoms
- bad planning
- difficulties in solving problems
- inattention or confusion
- problems with the short memory
- speech difficulties
- difficulties in finding the right words

Consequences
This mental decline raises the question to what extent the patient is still capable of taking medical decisions, or decisions regarding his end of life.

Neuropsychological evaluations can be useful to assist the family in making a plan for the future.

Medical and financial representatives have to conduct discussions and should consider whether the wishes of the patient are followed.

FTLD (Fronto-temporal-lobar-degeneration)
When such a mental decline prevents the patient from executing daily actions we talk about FTLD.

FTLD is often associated with shrinkage of the brain in the frontal and temporary lobes of the brain.  In order to prove FTLD no lab tests exist.  This can only be done by questioning the patient and the family about the symptoms, the family history and on the basis of neuro-psychological tests.

FTLD behavioral changes
-lack of interest or reservedness
-reduced spontaneity
-reduced emotions or increased emotional reactions
-socially inhibited or inappropriate social behavior
-change in sleeping or eating patterns
-recurring behaviors such as storing food or constant food cravings

FTLD mental changes
-inattention
-bad planning and organization
-amnesia
-lack of language
-problem solving difficulties and poor understanding

Treatment of FTLD
There is no specific treatment for FTLD.  Doctors can only try to alleviate the symptoms and learn the family how to cope with these changes
-Rilutek and Neurontin are medicines that are often used to treat ALS, but it has never been demonstrated that they improve the cognitive decline.
-With Aricept, Exelon and Reminyl, medication commonly used in Alzheimer’s disease, it has been demonstrated that it aggravates the irritability characteristic of FTLD
-SSRI’s such as Prozac, Zoloft and Celexa are occasionally used to treat behavioral disorders in FTLD.


Pseudo Bulbar Affect (PBA)
PBA manifests itself in unwanted and uncontrollable moments of laughing and crying at inappropriate moments.  PBA can cause a lot of misery in the social field, in the labor market or within the family.  The emotion is often triggered by something totally insignificant that is somewhat sad or funny.  For example a sad commercial may lead to uncontrolled weeping.  The emotion can also bring the opposite emotion of what the person feels at that moment.  As such uncontrolled laughing can be the consequence of feelings of anger.

Prevalence
43% to 49% of the pALS shows PBA.  PBA has also been discovered with people with head injuries, multiple sclerosis, Parkinson’s disease and Alzheimer’s disease.

Treatments
Pharmaceutical
At this moment Nuedexta is the only medicine that is recommended for PBA.

Antidepressants, such as amitriptyline, fluoxetine and citalopram can also be used to treat PBA.

Behavioral
Instigating the opposite emotion can help to control the emotions. During moments of uncontrolled weeping, someone can for example tell a funny joke to stop the weeping.

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