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Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy
  1. Joost Raaphorst1,2,
  2. Marianne de Visser1,
  3. Marie-José van Tol3,
  4. Wim H J P Linssen2,
  5. Anneke J van der Kooi1,
  6. Rob J de Haan4,
  7. Leonard H van den Berg5,
  8. Ben Schmand1,6
  1. 1Departments of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
  2. 2Department of Neurology, Sint Lucas Andreas Hospital, Amsterdam, The Netherlands
  3. 3Department of Psychiatry, Leiden University Medical Centre, Leiden, The Netherlands
  4. 4Clinical Epidemiology and Biostatistics, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
  5. 5Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Centre Utrecht, Utrecht, The Netherlands
  6. 6Department of Psychonomics, University of Amsterdam, Amsterdam, The Netherlands
  1. Correspondence to J Raaphorst, Department of Neurology (H2-235), Academic Medical Centre, PO Box 22660, 1100 DD Amsterdam, The Netherlands; j.raaphorst{at}


Aim 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.


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  • Funding The study was supported by a grant from The Netherlands ALS Foundation (JR).

  • Competing interests None.

  • Ethics approval The study was conducted with the approval of the medical ethics committees of the hospitals.

  • Provenance and peer review Not commissioned; externally peer reviewed.