Psychotic and Emotional Phenomena Associated With Amyotrophic Lateral Sclerosis

Comparative analysis of C9orf72 and sporadic disease in an ALS clinic population

“…The link between ALS and dementia has been established for some time, with reports many decades ago of patients with sporadic and familial ALS with comorbid FTD. 33,34 The discovery in recent years of genetic mutations underlying the coexistence of ALS and FTD in families, the most common of which is the C9orf72 repeat expansion, has advanced our knowledge of ALS as a multisystem disease. 35 Yet our clinical definitions for family history of disease have changed very little, and clearly should include questions about dementia, especially with behavioral changes, as well as other neurodegenerative phenotypes.…”

Pseudobulbar affect: an under-recognized and under-treated neurological disorder

“…1,6 Clinical case and study data have shown that PBA may cause severe distress, embarrassment, and social disability among patients. 3,[7][8][9][10] In addition, studies have shown that patients with PBA or similar syndromes experience an increased incidence of depression, 11 impairments in executive function 12 and sexual function, 13 and ability to perform activities of daily living 14 10,11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32] ). This variability is probably due to differences in the populations studied and in the criteria and methods used for identifying PBA.…”

“…Ranges of estimated prevalence for PBA and similar syndromes, such as emotional lability as variously defined and identified in published reports are indicated by the vertical gray arrows. 5,10,11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32] Prevalence rates determined in the present study with PLACS≥13 and CNS-LS≥13 are indicated by the transverse plot points. The prevalence rates determined with CNS-LS≥21 in the present study were very similar to those for PLACS≥13, and are not shown here to enhance visual clarity.…”

Manifestaciones neuropsiquiátricas y cognitivas en demencia frontotemporal y esclerosis lateral amiotrófica: dos polos de una entidad común

“…Los trastornos conductuales son similares a aquellos encontrados en DFTvc [96][97][98] . Análisis de series pequeñas sugieren la presencia de síntomas psicóticos, como ideas delirantes y alucinaciones en casos con DFT-ELA 96,99,100 . Las ideas delirantes se presentan como psicosis de aparición tardía, que precede los trastornos conductuales 101-104 y remiten espontáneamente en el curso de la enfermedad.…”