Occurrence of the "applause sign" in patients with amyotrophic lateral sclerosis.

The applause sign was originally described as a quick bedside test to discriminate progressive supranuclear palsy (PSP) (positive applause sign, PAS) from Parkinson's disease (PD) and frontotemporal dementia (FTD) (negative applause sign). However, recent research demonstrated that the test is positive not only in a subset of patients with PD and FTD, but also in other neurodegenerative diseases. We tested 22 patients with amyotrophic lateral sclerosis (ALS) together with 22 healthy sex- and age-matched controls for the occurrence of PAS.

Inappropriate sexual behaviour in a case of ALS and FTD: successful treatment with sertraline.

In a subset of patients, ALS is associated with frontotemporal dysfunction (ALS-FTD). Clinically, ALS-FTD may present with a variable spectrum of cognitive and behavioural deficits. We report a 53-year-old ALS-FTD patient developing inappropriate sexual behaviour (ISB) manifesting as a vigorous sexual drive with exhausting demands for sexual intercourse and physical aggressions against his wife.

Glial proliferation and metabotropic glutamate receptor expression in amyotrophic lateral sclerosis.

Accumulating evidence indicates that alterations in glial activation and disturbances in glial glutamate metabolism may contribute to the pathogenesis of amyotrophic lateral sclerosis (ALS). Metabotropic glutamate receptors (mGluRs) are involved in glutamate homeostasis as well as in glial proliferation. Using in situ hybridization and immunohistochemistry we found a strong upregulation of group I and group II mGluR mRNA and protein in ALS spinal cord as compared to controls (mGluR5 > mGluR1 > mGluR2/3).

Differential expression of mGluR5 in human lumbosacral motoneurons.

Glutamatergic excitotoxicity is one of the main hypotheses to explain motoneuronal degeneration in amyotrophic lateral sclerosis (ALS). Interestingly, autonomic motoneurons remain almost unaffected, even in late stages of the disease. Since glutamate receptors may mediate neurotoxic as well as neuroprotective effects, different expression patterns may contribute to neuronal vulnerability.