The effects of motor neurone disease on language: Further evidence

doi:10.1016/S0093-934X(03)00357-2

Brain and Language

Volume 89, Issue 2, May 2004, Pages 354-361

https://doi.org/10.1016/S0093-934X(03)00357-2 Get rights and content

Abstract

It might sound surprising that Motor Neurone Disease (MND), regarded still by many as the very example of a neurodegenerative disease affecting selectively the motor system and sparing the sensory functions as well as cognition, can have a significant influence on language. In this article we hope to demonstrate that language dysfunction is not only a pronounced and well documented symptom in some MND patients but also that the study of language in MND can address interesting theoretical questions about the representation of language and conceptual knowledge in the brain. After a brief introduction delineating clinical and pathological features of the disease we discuss the evidence available in the literature for language dysfunction in MND. We then present linguistic data from our own study of seven patients with MND/dementia/aphasia syndrome focusing on the dissociation between noun and verb processing. To illustrate the clinical, neuropsychological and linguistic aspects of MND we describe in more detail the patient E.N., a pathologically confirmed case of MND/dementia. Finally, we attempt to characterise the nature of the linguistic impairment in MND in the light of current debates about the mechanisms underlying noun/verb dissociation.

Section snippets

Language involvement in motor neurone disease: Past evidence and future perspectives

One fundamental limitation to neuropsychological lesion studies in humans is the fact that, in many traditionally investigated conditions, the type and extent of brain damage is determined primarily by the lesion location (e.g., arterial territories in stroke, the site of the injury in trauma or the site of the tumour in neoplasms), which bears often little relation to the functional architecture of the underlying brain regions. Neighbouring areas of different embryological origin, with

The Cambridge MND/aphasia series

In the years 1996–2002 we had the opportunity to examine seven patients presenting, in a fairly uniform manner, with an insidious onset of predominantly neuropsychiatric symptoms such as obsession with food, overeating, hoarding, “childlike behaviour,” irritability, disinhibition, socially inappropriate behaviour, and, in some cases, paranoid ideas and hallucinations (one of the patients, N.N., believed that her childhood boyfriend, whom she has not seen for decades, moved into her house).

E.N.—Case description

The patient E.N. (initials changed to protect privacy) was admitted to Addenbrooke’s Hospital, Cambridge with a clinical picture similar to that of other MND/dementia cases. The history of his illness began one year earlier, when his wife noticed persecutory delusions and a change in food preference (towards chocolate and peanuts). Over the following four to five months the patient became slower, his speech slurred and his verbal output extremely limited (usually “yes” and “no”). His cognitive

Discussion: Towards a better characterisation of the linguistic deficit in MND

Our findings confirm that aphasia can be an early and prominent feature of MND/dementia. Pronounced language dysfunction was documented in all seven examined patients. In all cases it preceded the development of motor symptoms and was, therefore, unlikely to be caused by them. It is equally unlikely that the language dysfunction was a result of generalised dementia: the level of language impairment was much higher than would be expected on the basis of the results on cognitive screening tests.

Acknowledgments

We thank Tina Emery and Angela O’Sullivan for their dedicated work and their support for our patients and their families in general and E.N. in particular. We are indebted to John Xuereb and the Cambridge Brain Bank for the pathological examination of the brains of MND patients, to Simon Boniface for the neurophysiological assessment, to Karalyn Patterson and Friedemann Pulvermüller for many fruitful discussions and to Marion Wilkinson and Ashley Muir for their help in editing the manuscript.

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The effects of motor neurone disease on language: Further evidence

Abstract

Section snippets

Language involvement in motor neurone disease: Past evidence and future perspectives

The Cambridge MND/aphasia series

E.N.—Case description

Discussion: Towards a better characterisation of the linguistic deficit in MND

Acknowledgments

Journal of Neurolinguistics

Brain and Language

Brain and Language

Neuropsychologia

Cortex

Journal of Psychiatric Research

Single-photon emission computed tomographic investigation of patients with motor neuron disease

Neurology

Noun–verb dissociation in three patients with motor neuron disease and aphasia

Brain and Language

Cognition, language and behaviour in motor neurone disease: Evidence of frontotemporal dementia

Dementia and Geriatric Cognitive Disorders

Motor neurone disease, dementia and aphasia coincidence, co-occurrence or continuum?

Journal of Neurology

Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease–dementia–aphasia syndrome

Brain

L’association maladie de Pick et sclerose laterale amyotraphique

Encephale

Object and action naming in Alzheimer’s disease and frontotemporal dementia

Neurology

Rapidly progressive aphasic dementia and motor neuron disease

Annals of Neurology

Syndrome familial: Association de Maladie de Pick at sclerose laterale amyotrophique

Encephale

Thalamic dementia and motor neuron disease

Neurology

Rapidly progressive aphasia with bulbar motor neurone disease: A clinical and neuropsychological study

Behavioural Neurology

Amyotrophic Lateral Sclerosis: A synthesis of research and clinical practice

Dementia of frontal lobe type and motor neuron disease. A Golgi study of the frontal cortex

Journal of Neurology, Neurosurgery, and Psychiatry

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Pyramids and palm trees: A test of semantic access from pictures and words