Cognitive and Sleep Features of Multiple System Atrophy: Review and Prospective Study

Background: The profile and degree of cognitive impairment in Multiple System Atrophy (MSA) and the impact of sleep disorders, REM sleep behavior disorder (RBD) in particular, in parkinsonism-related cognitive deficits are currently being debated. Summary: We reviewed the cognitive, affective and sleep findings in MSA and also carried out a longitudinal investigation of 10 MSA patients. At the first evaluation, 3 patients showed isolated cognitive deficits. After a mean of 16 months, these patients remained unchanged, while 1 patient worsened from a normal condition. No significant differences emerged when the cognitive, affective and video-polysomnographic findings of MSA-P and MSA-C were compared. Depression was present in half of the patients, although it did not influence their cognitive performance. Comparisons between the first and second evaluation data showed significant worsening in visual attention and in ADL/IADL and UMSARS. Key Messages: Isolated cognitive deficits are evidenced in a minority of MSA patients with the absence of a clear-cut diagnosis of dementia in the early stages of the disease. Attention and executive functions are often impaired. This study with a short follow-up period showed that RBD, although present in almost all patients affected by MSA, does not appear a clear early marker of cognitive impairment. Future longer-term studies with a larger patient sample are thus encouraged.

[1]  S. Spaccavento,et al.  Multiple system atrophy with early cognitive deficits: A case report , 2013, Neurocase.

[2]  Young Eun Kim,et al.  Clinical and imaging characteristics of dementia in multiple system atrophy. , 2013, Parkinsonism & related disorders.

[3]  J. Molinuevo,et al.  Neurodegenerative disease status and post-mortem pathology in idiopathic rapid-eye-movement sleep behaviour disorder: an observational cohort study , 2013, The Lancet Neurology.

[4]  E. Tolosa,et al.  A cross-sectional multicenter study of cognitive and behavioural features in multiple system atrophy patients of the parkinsonian and cerebellar type , 2013, Journal of Neural Transmission.

[5]  P. Cortelli,et al.  Standing worsens cognitive functions in patients with neurogenic orthostatic hypotension , 2012, Neurological Sciences.

[6]  M. T. Pellecchia,et al.  Excessive daytime sleepiness in multiple system atrophy (SLEEMSA study). , 2011, Archives of neurology.

[7]  T. Shimohata,et al.  Factors influencing the cognitive function in patients with multiple system atrophy , 2010, Movement disorders : official journal of the Movement Disorder Society.

[8]  M. Silber,et al.  REM sleep behavior disorder preceding other aspects of synucleinopathies by up to half a century , 2010, Neurology.

[9]  Y. Agid,et al.  Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. , 2010, Brain : a journal of neurology.

[10]  M. Jahanshahi,et al.  A comparison of depression, anxiety, and health status in patients with progressive supranuclear palsy and multiple system atrophy , 2010, Movement disorders : official journal of the Movement Disorder Society.

[11]  L. Sambati,et al.  Are subjective cognitive complaints a risk factor for dementia? , 2010, Neurological Sciences.

[12]  Nir Giladi,et al.  Cognition in multiple system atrophy: neuropsychological profile and interaction with mood , 2010, Journal of Neural Transmission.

[13]  C. Christine,et al.  Cognitive and Neuropsychiatric Profile of the Synucleinopathies: Parkinson Disease, Dementia With Lewy Bodies, and Multiple System Atrophy , 2009, Alzheimer disease and associated disorders.

[14]  Yen-Hsiang Chang,et al.  Cognitive deficits in multiple system atrophy correlate with frontal atrophy and disease duration , 2009, European journal of neurology.

[15]  Yoon-Ho Hong,et al.  Does Multiple System Atrophy Itself Affect Sleep Structure? , 2009, The neurologist.

[16]  K. Nakashima,et al.  Assessment of dementia in patients with multiple system atrophy , 2009, European journal of neurology.

[17]  G. Plazzi,et al.  Status dissociatus evolving from REM sleep behaviour disorder in multiple system atrophy. , 2009, Sleep medicine.

[18]  P Sandroni,et al.  Second consensus statement on the diagnosis of multiple system atrophy , 2008, Neurology.

[19]  E. Sinforiani,et al.  Cognitive performance in REM sleep behaviour disorder: a possible early marker of neurodegenerative disease? , 2008, Sleep medicine.

[20]  P. Kempster,et al.  Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. , 2008, Brain : a journal of neurology.

[21]  K. Ito,et al.  Cognitive impairments in multiple system atrophy , 2008, Neurology.

[22]  M. S. Lee,et al.  Effects of disease duration on the clinical features and brain glucose metabolism in patients with mixed type multiple system atrophy. , 2008, Brain : a journal of neurology.

[23]  G. Plazzi,et al.  Impaired cortical and autonomic arousal during sleep in multiple system atrophy , 2007, Clinical Neurophysiology.

[24]  E. Wolf,et al.  Orthostatic hypotension and attention in Parkinson’s disease with and without dementia , 2007, Journal of Neural Transmission.

[25]  U. Mosimann,et al.  Orthostatic hypotension in Parkinson's disease: association with cognitive decline? , 2006, International journal of geriatric psychiatry.

[26]  A. Kishore,et al.  Neuropsychological functions in progressive supranuclear palsy, multiple system atrophy and Parkinson's disease. , 2006, Neurology India.

[27]  I. Daum,et al.  Cognitive function in multiple system atrophy of the cerebellar type , 2006, Movement disorders : official journal of the Movement Disorder Society.

[28]  J. Hodges,et al.  Visuospatial functions in atypical parkinsonian syndromes , 2006, Journal of Neurology, Neurosurgery & Psychiatry.

[29]  B. Bioulac,et al.  Sleep disorders in multiple system atrophy , 2005, Journal of Neural Transmission.

[30]  Nick C Fox,et al.  Can the frontal assessment battery (FAB) differentiate bradykinetic rigid syndromes? Relation of the FAB to formal neuropsychological testing , 2005, Neurocase.

[31]  P. S. Mathuranath,et al.  Subcortical dementia revisited: Similarities and differences in cognitive function between progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA) , 2005, Neurocase.

[32]  E. Tolosa,et al.  Characteristics of idiopathic REM sleep behavior disorder and that associated with MSA and PD , 2005, Neurology.

[33]  T. Rogers,et al.  Cognitive bedside assessment in atypical parkinsonian syndromes , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[34]  Sid Gilman,et al.  Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS) , 2004, Movement disorders : official journal of the Movement Disorder Society.

[35]  S. Cappa,et al.  Neuropsychological assessment in idiopathic REM sleep behavior disorder (RBD) , 2004, Neurology.

[36]  Gesine L. Alders,et al.  Differentiation of parkinsonian syndromes according to differences in executive functions , 2003, Journal of Neural Transmission.

[37]  K. Arai,et al.  Laryngeal Stridor in Multiple System Atrophy , 2003, European Neurology.

[38]  G. Plazzi,et al.  Italian version of the Epworth sleepiness scale: external validity , 2003, Neurological Sciences.

[39]  L Junck,et al.  Patterns of neuropsychological performance in multiple system atrophy compared to sporadic and hereditary olivopontocerebellar atrophy , 2002, Brain and Cognition.

[40]  Paolo Caffarra,et al.  Una versione abbreviata del test di Stroop: dati normativi nella popolazione italiana , 2002 .

[41]  B. Bioulac,et al.  Sleep disorders and their determinants in multiple system atrophy , 2002, Journal of neurology, neurosurgery, and psychiatry.

[42]  P. Caffarra,et al.  Rey-Osterrieth complex figure: normative values in an Italian population sample , 2002, Neurological Sciences.

[43]  E. Capitani,et al.  Wisconsin card sorting test: a new global score, with Italian norms, and its relationship with the Weigl sorting test , 2000, Neurological Sciences.

[44]  F. Carella,et al.  Neuropsychological follow up in patients with Parkinson's disease, striatonigral degeneration-type multisystem atrophy, and progressive supranuclear palsy , 2000, Journal of neurology, neurosurgery, and psychiatry.

[45]  T. Wetter,et al.  Sleep and periodic leg movement patterns in drug-free patients with Parkinson's disease and multiple system atrophy. , 2000, Sleep.

[46]  N P Quinn,et al.  What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson's disease? , 2000, Journal of neurology, neurosurgery, and psychiatry.

[47]  I Litvan,et al.  Consensus statement on the diagnosis of multiple system atrophy , 1998, Journal of the Neurological Sciences.

[48]  G. E. Smith,et al.  REM sleep behavior disorder and degenerative dementia , 1998, Neurology.

[49]  D. Radice,et al.  Cognitive dysfunction and impaired organization of complex motility in degenerative parkinsonian syndromes. , 1998, Archives of neurology.

[50]  H. Shibasaki,et al.  REM sleep motor dysfunction in multiple system atrophy: with special emphasis on sleep talk as its early clinical manifestation , 1997, Journal of neurology, neurosurgery, and psychiatry.

[51]  G. Plazzi,et al.  REM sleep behavior disorders in multiple system atrophy , 1997, Neurology.

[52]  N. Quinn,et al.  Multiple system atrophy: A review of 203 pathologically proven cases , 1997, Movement disorders : official journal of the Movement Disorder Society.

[53]  E. Capitani,et al.  Trail making test: normative values from 287 normal adult controls , 1996, The Italian Journal of Neurological Sciences.

[54]  F. Doricchi,et al.  Attentional functions in multiple system atrophy and Parkinson's disease. , 1996, Journal of neurology, neurosurgery, and psychiatry.

[55]  N. Quinn,et al.  REM sleep behaviour disorder as the presenting symptom of multiple system atrophy. , 1995, Journal of neurology, neurosurgery, and psychiatry.

[56]  Y. Agid,et al.  Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy. , 1995, Journal of neurology, neurosurgery, and psychiatry.

[57]  P N Leigh,et al.  Cognitive deficits in progressive supranuclear palsy, Parkinson's disease, and multiple system atrophy in tests sensitive to frontal lobe dysfunction. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[58]  R. Manni,et al.  Nocturnal sleep in multisystem atrophy with autonomic failure: polygraphic findings in ten patients , 1993, Journal of Neurology.

[59]  Giuseppe Zappalà,et al.  The mini‐mental state examination: Normative study of an Italian random sample , 1993 .

[60]  M. Musicco,et al.  Cognitive and motor performance in multiple system atrophy and Parkinson's disease compared , 1993, Neuropsychologia.

[61]  C. Marsden,et al.  Cognitive performance in multiple system atrophy. , 1992, Brain : a journal of neurology.

[62]  A. Pfefferbaum,et al.  Neuropsychological deficits accompanying striatonigral degeneration. , 1991, Journal of clinical and experimental neuropsychology.

[63]  N. Quinn,et al.  Multiple system atrophy--the nature of the beast. , 1989, Journal of neurology, neurosurgery, and psychiatry.

[64]  C. Papagno,et al.  Verbal and spatial immediate memory span: Normative data from 1355 adults and 1112 children , 1987, The Italian Journal of Neurological Sciences.

[65]  Marcella Laiacona,et al.  Tre test clinici di ricerca e produzione lessicale , 1986 .

[66]  A. Stracciari,et al.  Neuropsychological assessment of mental deterioration: purpose of a brief battery and a probabilistic definition of “normality” and “non‐normality” , 1986, Acta psychiatrica Scandinavica.

[67]  F. D. Naylor,et al.  Success and failure experiences and the factor structure of the state-trait anxiety inventory , 1978 .

[68]  S. Folstein,et al.  "Mini-mental state". A practical method for grading the cognitive state of patients for the clinician. , 1975, Journal of psychiatric research.

[69]  A. Beck,et al.  An inventory for measuring depression. , 1961, Archives of general psychiatry.

[70]  M. Mahowald,et al.  Delayed emergence of a parkinsonian disorder or dementia in 81% of older males initially diagnosed with idiopathic REM sleep behavior disorder (RBD): 16 year update on a previously reported series , 2013 .

[71]  A. Chesson,et al.  The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology, and Techinical Specifications , 2007 .

[72]  A. Chesson,et al.  The American Academy of Sleep Medicine (AASM) Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications , 2007 .

[73]  G. Plazzi,et al.  Sleep disorders in multiple system atrophy: a correlative video-polysomnographic study. , 2004, Sleep medicine.

[74]  L. Colombo,et al.  Stima del quoziente intellettivo tramite l'applicazione del TIB (Test Breve di Intelligenza) , 2002 .