Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy.
OBJECTIVE
In contrast with findings in amyotrophic lateral sclerosis (ALS), cognitive impairments have as yet not been shown in the lower motor neuron variant of motor neuron disease, progressive spinal muscular atrophy (PMA). The objective of this study was to investigate cognitive function in PMA and to compare the cognitive profile with that of ALS. In addition, visuospatial functions were assessed comprehensively; these tests are underrepresented in earlier neuropsychological investigations in ALS.


METHODS
23 PMA and 30 ALS patients (vital capacity >70% of predicted value) underwent a neuropsychological assessment adapted to motor impairments: global cognitive and executive functioning, psychomotor speed, memory, language, attention and visuospatial skills. The results were compared with age, education and sex matched controls and with normative data.


RESULTS
Compared with controls, PMA patients performed worse on attention/working memory (digit span backward), category fluency and the Mini-Mental State Examination. Compared with normative data, PMA patients most frequently showed impairment on three measures: letter-number sequencing, and immediate and delayed story recall. 17% of PMA patients showed cognitive impairment, defined as performance below 2 SDs from the mean of normative data on at least three neuropsychological tests. In ALS, similar but more extensive cognitive deficits were found. Visuospatial dysfunction was not found in PMA and ALS.


CONCLUSIONS
17% of PMA patients have executive and memory impairments. PMA with cognitive impairment adds a formerly unknown phenotype to the existing classification of motor neuron diseases.
Raaphorst
Joost
J
Department of Neurology (H2-235), Academic Medical Centre, Amsterdam, The Netherlands. j.raaphorst@amc.uva.nl
de Visser
Marianne
M
van Tol
Marie-José
MJ
Linssen
Wim H J P
WH
van der Kooi
Anneke J
AJ
de Haan
Rob J
RJ
van den Berg
Leonard H
LH
Schmand
Ben
B
D017677:Age Distribution; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000523:psychology; D001288:Attention / Q000502:physiology; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology*; D004493:Education; D056344:Executive Function / Q000502:physiology*; D005260:Female; D006801:Humans; D007361:Intelligence Tests; D007802:Language; D008297:Male; D008569:Memory Disorders / Q000209:etiology / Q000523:psychology*; D008570:Memory, Short-Term / Q000502:physiology; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000523:psychology*; D009134:Muscular Atrophy, Spinal / Q000150:complications / Q000523:psychology*; D009483:Neuropsychological Tests; D011597:Psychomotor Performance / Q000502:physiology; D017678:Sex Distribution; D013028:Space Perception / Q000502:physiology; D014796:Visual Perception / Q000502:physiology