Language Case Files in Neurological Disorders

Louise Cummings

• Publisher: Routledge
• Year: 2021
• ISBN:
  9781000427004
• Edition: 1
• Topic: Languages & Linguistics
• Subtopic: Linguistics
• Index: Languages & Linguistics

This book features case studies of ten individuals with acquired neurological disorders. These disorders have implications for speech, language, and communication, but to date they have not been the focus of research in speech-language pathology.

Chapters present a brief medical overview of each condition, followed by detailed linguistic analysis. A carefully assembled narrative captures the impact of each neurological disorder on an individual's daily life and social activities. This structured approach, supported by further reading and exercises, gives readers a nuanced understanding of each disorder's clinical presentation and language and communication features, and the complex interrelationship between language, communication, and cognitive and motor symptoms.

The book will be of interest to students of all levels, researchers, and clinicians in speech-language pathology and related disciplines, including neurology, psychiatry, and psychology.

Case Study 1

Corticobasal Degeneration

Key facts and figures:

• Corticobasal degeneration (CBD) is a tauopathy in which there is deposition of abnormal tau protein in the brain. Corticobasal syndrome (CBS), the most common phenotype, is characterised by asymmetric rigidity and apraxia, cortical sensory deficits, dystonia and myoclonus. Patients with clinical CBD can have non-CBD pathology such as Alzheimer’s pathology.¹
• The prevalence of CBD is 10.84 per 100,000 and the incidence is 1.61 per 100,000. These figures are based on a population of 1.69 million in Cambridgeshire and Norfolk in England. A crude prevalence of 9 per 100,000 is reported in a rural district in Japan. The age-standardised incidence rate is 0.02 per 100,000/year in a Russian population-based study.^2–4
• Age at onset and survival time in CBD have been reported in several studies. In a review of 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks, mean age at symptom onset was 63.7 years (range 45–77.2 years) and mean disease duration was 6.6 years (range 2.0–12.5 years).⁵
• Motor, behavioural, cognitive and language symptoms are common in CBD. In one clinical sample of 48 patients with CBD, apraxia (93.8%) was the most common symptom followed by behavioural changes (75%), rigidity (68.8%), postural instability (66.7%), language impairment (66.7%), akinesia (62.5%), dystonia (54.2%) and supranuclear gaze paresis (45.8%).²
• Speech, language and cognitive impairments are common in CBD. In a clinical sample of 33 patients with CBS, motor speech disorder was present in 33%, agrammatism in 48% and sentence comprehension problems in 60%. Impairments of executive functions were reported in 81% of the sample.⁶ Language disturbances in some patients with CBD manifest as an aphasia syndrome.⁷
• CBD is a challenging disorder to diagnose. It can present with multiple phenotypes, and other neurodegenerative disorders with a different underlying pathology can mimic its clinical course.⁸

Background

Bob (not his real name) is a man of 66; 4 years who was diagnosed with corticobasal degeneration (CBD) in September 2017. [Bob passed away on 25 March 2021. He was last seen in clinic by his neurologist on 10 February 2021, following which his diagnosis was revised to “progressive non-fluent aphasia – corticobasal syndrome”.] He is a former joiner. His wife is a retired science teacher. Both have children from previous relationships. Bob’s wife noticed some changes in him a few years ago that “started alarm bells ringing”. In August 2014, some guests at a family wedding expressed concerns for Bob as he did not seem to be himself. Shortly afterwards, his wife noticed that he was experiencing difficulties with coordination. In the third week of August 2014, Bob and his wife were walking along the promenade in their hometown. Bob went to put his mobile phone in his pocket but completely missed his pocket and ended up kicking the phone ahead of him. A couple of weeks after this incident, Bob’s wife noticed that he was unable to help her change the duvet on the bed, a task he had completed many times before. Bob was unable to bring the duvet and cover together and eventually had to abandon the task. Not long after this, Bob’s wife noticed that he was unable to coordinate actions such as getting dressed and putting on his shoes. Bob sat and looked at his shoes as if he were lost. By August 2016, Bob was unable to measure and cut pieces of wood to a required length, something he had done since he was 14 years old. Bob has no family history of dementia or mental illness.

There ensued a long-running series of medical and cognitive investigations to establish the cause of Bob’s difficulties. In November 2014, Bob was referred for an MRI of the head. He had experienced right-sided facial weakness and speech difficulty about two months earlier. His general practitioner had referred him for a CT scan, but no abnormality was detected. Bob presented with bilateral upgoing plantars without any sensory loss. He had expressionless facies but no other signs of Parkinson’s disease. The MRI was undertaken to investigate if there wa...

Table of contents

• Cover
• Half Title
• Series Page
• Title Page
• Copyright Page
• Table of Contents
• List of Figures and Tables
• Acknowledgements
• Introduction
• Case Study 1: Corticobasal Degeneration
• Case Study 2: Progressive Supranuclear Palsy
• Case Study 3: Huntington’s Disease
• Case Study 4: Lewy Body Disease
• Case Study 5: Multiple Sclerosis
• Case Study 6: Parkinson’s Disease
• Case Study 7: Motor Neurone Disease
• Case Study 8: Alcohol-Related Brain Damage
• Case Study 9: Covid-19 Infection
• Case Study 10: Guillain-Barré Syndrome
• Conclusion
• Glossary
• Appendix
• Index