Cognitive and behavioural neurology Question Bank

A 62-year-old male presents to the cognitive clinic with progressive problems with speech. He has a past medical history of hypertension and hypercholesterolaemia. There is no family history of early dementia, psychosis, or motor neurone disease. He currently works from home as an accountant but is experiencing increasing difficulties with typing and writing.

On direct questioning, his wife reports he can drop words from sentences and use them out of order. His daughter had noticed reversal of binary terms, such as “yes” and “no” or “him” and “her” and difficulty in comprehension of complex sentences. 

On examination, he exhibits anomia and significant agrammatism. Repetition, word meaning, semantic association and writing of irregularly spelled words are relatively spared. A Montreal Cognitive Assessment (MoCA) is 22/30 with impairment of executive function, fluency and visuospatial tasks. The remainder of the neurological examination is normal without frontal release signs. 

An MRI-brain reveals dominant atrophy of Broca areas 44 and 45. 

What is the probable diagnosis?

A 56-year-old female with a history of psychotic depression develops a resting and postural tremor, bradykinesia and a shuffling gait. Drug-induced parkinsonism is suspected. The patient’s medication includes stable doses of risperidone, venlafaxine and mirtazapine. There is a family history of Parkinson’s disease. 

The patient undergoes of DatScan following standard procedures, which demonstrates bilateral asymmetrical reduced radionucleotide uptake within the putamina relative to the caudate. 

The patient’s mental state improves and their antipsychotic and antidepressant medication is withdrawn. On follow up review, 4 months later, you note an improvement in parkinsonism with a mild unilateral rest tremor. 

Which of the following is the most accurate in relation to the above scenario?

Which of the following is correct in relation to Huntington’s disease (HD)?

Which of the following findings on FDG PET is most suggestive of dementia with Lewy bodies?

A 64-year-old female you have seen in clinic with mild cognitive impairment and a family history of dementia undergoes a lumbar puncture for cerebrospinal fluid neurodegenerative biomarkers. 

Which of the following CSF profiles is the strongest predictor of developing Alzheimer’s dementia?

Which of the following may be compatible with a diagnosis of transient global amnesia (TGA)?

A 74-year-old male presents to the outpatient department with an insidiously progressive decline in language. He has a past history of dyslexia. During the consultation, you notice hesitations or false starts with circumlocutions. 

On examination, he is unable to repeat sentences with a reduced verbal rate and phonologic paraphasias. Grammar, motor speech and single-word comprehension is intact. There is no ideomotor apraxia, parkinsonism, oculomotor abnormality or gait impairment. 

An MRI brain reveals left temporo-parietal junction atrophy with thinning of the left superior temporal gyrus. 

What is the likely cause of his presentation?

A 50-year-old female attended alone for review in the cognitive disorders. She reports long-standing issues with memory, which have worsened in the last 2-3 years. She describes episodes of difficulty finding words and being unable to recall details of some recent family events. She was diagnosed with anxiety last year and takes Citalopram 10mg OD. 

She lives with her husband and teenage son. She has worked as a secretary for over 20 years but has recently taken time off due to stress. She had a number of confrontations at work, which is unusual. Her mother was diagnosed with Alzheimer’s dementia at the age of 76. 

She performs highly on neuropsychological testing, although displays relatively poor performance (5^th centile) on an easy test of psychomotor speed and much better (60^th centile) on a more challenging task. She self-rates her memory as low-average but performs above average in most domains of memory assessment.

She scores 13/21 on the anxiety subset and 8/21 on the depression subset of the Hospital Anxiety and Depression Scale. She has normal routine bloods and a normal MRI brain. 

1 year later she reports persistent concerns about her memory. Repeat neuropsychology testing is stable.

What is the most likely diagnosis?

You are called to see an 86-year-old female who has been referred by her GP to the emergency department after she reported seeing images of people and animals. Her family report some decline in her short-term memory but she remains functionally independent. She has a past medical history of macular degeneration. She has had 2 falls in the last month. 

She is alert and oriented, tells you she has longstanding very poor vision and knows what she is seeing is not real. Visual acuity is 6/60 bilaterally. Neurological examination is otherwise normal. 

Which of the following is the most correct diagnosis?

A 44-year-old man presents to the Emergency Department with progressive confusion, skin changes and diarrhoea. He has a past history of alcoholism, recent bariatric surgery and has type 2 diabetes mellitus. His medications include insulin, metformin and atorvastatin. 

On examination, you notice a sunburn-like rash over sun-exposed areas including his hands and his neck – the latter consistent with Casal’s necklace. His GCS is 14 (M5V5E4) and there are no lateralising neurological signs. 

You commence pabrinex and monitor for alcohol withdrawal using local hospital protocols. Brain imaging is unrevealing. 

What is the most likely underlying cause?

A 45-year-old female is admitted with headache, fever and seizures. She is subsequently diagnosed with herpes simplex encephalitis. 

Which main cognitive measure is most often affected in this condition?

You see a 73-year-old female patient in the acute neurology clinic. She presented to the Emergency Department the day before with acute onset predominantly anterograde amnesia which had largely resolved by the time she was assessed 4 hours later. She was discharged with a provisional diagnosis of transient global amnesia. 

Which of the following would lead you to consider an alternative diagnosis?

A 72-year-old male is admitted with acute encephalitis, later found to be caused by herpes simplex virus type 1. 

Whilst an inpatient you notice alterations in his facial expressions and speech. He becomes aphasic and cannot chew, protrude his tongue or smile. However, spontaneous and automatic facial movements associated with laughter and crying are preserved. 

Hearing and comprehension are normal. 

What is the likely cause?

A 62-year-old retired engineer was referred to the general neurology clinic with a 12-month history of personality change. His family had noticed socially inappropriate behaviour such as laughing inappropriately, swearing and making offensive comments to friends in social settings. He had lost interest in his social activities and had stopped attending his weekly choir practice. His family felt he had become “indifferent”, participated less in family conversations, and spent most of the day watching television. He regularly became frustrated with his wife and was verbally aggressive, which was completely out of character with his normally placid demeanour. His wife explained that his appetite had changed over the past 6 months, stating that he would only eat desserts and chocolate bars. He had become very rigid in his behaviours and would repeatedly empty the cupboards and rearrange items. His memory was felt to be normal, and there were no changes in language.

^99mTc–hexamethylpropyleneamine oxime (HMPAO) brain single-photon emission CT (SPECT) imaging of his brain was performed.

Variants in which of the following genes are associated an increased risk of this gentleman’s diagnosis?

What medication is recommended as a first-line treatment for the management of psychosis in patients with dementia with Lewy bodies? 

A 70-year-old male presents with progressive memory loss and difficulty with language. 

A neurological examination reveals deficits in episodic memory and executive deficits. There are no motor deficits or abnormal movements. 

Imaging studies show mild hippocampal atrophy. CSF reveals a normal amyloid-β 1–42/1–40 ratio (Aβ42/40), p-tau and total tau.

He later passes away and neuropathological evaluation shows TDP-43 protein inclusions in the hippocampal, amygdala and middle frontal gyrus.

Which of the following conditions is the most likely diagnosis?

A 52-year-old female passes away with rapidly progressive dementia. Her post-mortem brain biopsy reveals spongiform vacuolation throughout the grey matter, reactive astrocyte, microglial proliferation, neuronal loss and amyloid plaques.

What is her underlying neurological diagnosis?

A 72-year-old gentleman is seen in cognitive clinic after his wife has noticed a progressive decline in his memory over the past 2 years. His symptoms have now progressed to the point where he requires help with certain activities of daily living.

On examination, he is afebrile, pulse is 42 beats/min, blood pressure 126/84mmHg, oxygen saturation 98% on room air and respiratory rate 16/min. There are no focal neurological deficits and no extrapyramidal signs. His mini-mental state examination reveals a score of 19, highlighting weaknesses in episodic anterograde memory, attention and visuospatial and executive domains. 

An MRI reveals mesial-temporal and temporoparietal lobe atrophy without significant white matter changes. Cerebrospinal fluid reveals reduced amyloid-β 1–42/1–40 ratio (Aβ42/40), as well as increased p-tau and total tau.

Give the likely diagnosis, what medication could be started to help reduce the rate of decline in function?

A 60-year-old female is referred to the outpatient clinic with progressive language impairment. There is a family history of motor neurone disease. During the consultation, you identify difficulties with word retrieval, noting frequent use of generic categories to illustrate the object in question. The patient seems to be relatively at ease with these deficits. Close family members have noted her experiencing problems with word meanings and she has been progressively more irritable and aggressive. 

On examination, you note anomia, which does not improve with verbal cues. Surface dyslexia is evident with difficulty reading words such as “yacht”, “colonel”, and “debt”. Speech, grammar and repetition are spared and sentence comprehension is broadly better than single-word comprehension. 

Limb examination reveals no frontal release or upper motor neurone signs. There is no ideomotor apraxia, parkinsonism, oculomotor abnormalities or gait impairment. 

What is the likely cause?