Speech and Higher Mental Function Examination — Clinical Case

DCE Short Case — "Please examine this patient's speech and higher mental function"

Instruction

"Please examine this patient's speech and higher mental function. Her family say her speech changed suddenly an hour ago. You have 7 minutes for examination and 8 minutes for discussion." [1]

Patient brief (provided to examiner / simulated patient)

Patient: Mrs Harpreet Kaur, 72 years old, retired schoolteacher, hypertensive, type 2 diabetes. [1]

Findings the trainee elicits: She is alert and afebrile, blood pressure 156/92, glucose 6.8. Spontaneous speech is fluent — long phrases, normal melody, effortless — but empty, with phonemic and verbal paraphasias and several neologisms; she is not frustrated by this. She manages an occasional one-step command but cannot follow a two-step command. She cannot repeat "no ifs, ands, or buts." She cannot name a watch or a pen. Hearing is intact to a whispered number in each ear. Attention, tested by digit span (six forward, four backward) and months backwards, is preserved. There are no focal limb signs. The cranial nerves, including the facial nerve and the tongue, are normal. [1]

Systematic examination routine (model)

1. Set the stage: introduce, consent, seat the patient upright at eye level, ensure her hearing aids are in and working, exclude pain, fever and hypoxia, and state the plan to examine speech first then higher mental function.
2. Listen to spontaneous speech: ask her to describe why she came in. Note fluent but empty speech with paraphasias and neologisms, and the lack of frustration.
3. Assess fluency: confirm the speech is fluent — full phrase length, normal melody, effortless.
4. Test comprehension: yes/no questions, then one-step commands (she manages), then two-step commands (she fails).
5. Test repetition: "no ifs, ands, or buts" — she fails, with phonemic paraphasias on the attempt.
6. Test naming: a watch, a pen, the fingers — she fails (anomia is present in all aphasias).
7. Test reading and writing: she cannot read a simple command or write a meaningful sentence.
8. Confirm hearing is intact to exclude a peripheral auditory cause.
9. Assess attention (digit span, months backwards) to exclude delirium and confirm this is a focal aphasia.
10. Screen the other cognitive domains noting they are limited by the aphasia, and state this at presentation. [1]

Presentation template (model)

"I examined Mrs Kaur, a 72-year-old woman with hypertension and diabetes, brought in forty minutes after the sudden onset of strange speech. She is alert and afebrile. On examination of the speech, spontaneous speech is fluent but empty, with phonemic and verbal paraphasias and several neologisms, and she is not frustrated by it. Comprehension is impaired — she manages an occasional one-step command but cannot follow a two-step command. Repetition is impaired — she cannot repeat 'no ifs, ands, or buts.' Naming is impaired for objects and fingers. Hearing is intact. Attention, tested by digit span and months backwards, is preserved. [1]

'In summary, this patient has a fluent aphasia with impaired comprehension and impaired repetition — a Wernicke aphasia. The sudden onset in a vasculopathic patient localises to the dominant superior temporal gyrus in the inferior division of the left middle cerebral artery, and this is an acute stroke. Her preserved alertness and attention confirm this is a focal aphasia, not delirium. My immediate priority is to activate the acute stroke pathway for urgent CT to exclude haemorrhage, then assess for thrombolysis and thrombectomy.'" [1]

Discussion questions

Q: "How do you know this is aphasia and not delirium?" [1]

"Because the core features of delirium are absent. Delirium is an acute, fluctuating impairment of attention with altered consciousness. This patient is fully alert, her digit span is preserved at six forward, and she manages one-step commands — her attention is intact. In delirium the speech is globally disorganised — rambling and tangential — whereas here the fluency and melody are normal and only the content is disordered, with specific language errors: paraphasias and neologisms. The onset is sudden and discrete, consistent with a vascular event, not the fluctuating, precipitant-driven course of delirium. The danger of the confusion label is that a patient with an acute dominant-hemisphere stroke is worked up for sepsis while the thrombolysis clock runs out. I confirm attention formally, then activate the stroke pathway." [1]

Q: "Why does she not have limb weakness if it is an MCA stroke?" [1]

"Because Wernicke area lies in the superior temporal gyrus, which is supplied by the inferior division of the middle cerebral artery and sits outside the primary motor cortex. The superior division of the MCA supplies the frontal motor cortex and the face and arm area, so a superior-division or complete-MCA occlusion produces a contralateral hemiparesis, but an isolated inferior-division occlusion can produce a Wernicke aphasia with no limb weakness. The absence of limb signs therefore does not exclude the stroke — it localises it to the inferior division of the MCA. I would still image urgently, because the clinical story of a sudden focal language deficit is an acute stroke until proven otherwise." [1]

Q: "What does her clock-drawing test tell you, and what are its limits?" [1]

"In the acute phase, a clock-drawing test would be confounded by her aphasia — she may not understand the instruction, and even if she does, the language of the task (numbers, the concept of ten past eleven) is precisely what she cannot access. So I would not over-interpret a clock drawing in an acute aphasic patient; the speech examination is the primary tool here. In a non-aphasic patient, clock drawing is a high-yield visuospatial and executive screen whose error patterns localise: neglect of one side to a non-dominant parietal lesion, perseveration to a frontal executive deficit, and a conceptual breakdown to a generalised dementia. A systematic review reports a pooled sensitivity of about 80 per cent and specificity of about 75 per cent under the Shulman system, though it is less sensitive for very mild impairment [5]."

DCE Long Case — Motor Neuron Disease with Bulbar and Pseudobulbar Features

Patient brief (provided to trainee)

Patient: Mr Robert Chen, 64 years old, former carpenter. [1]

Presenting complaint: Progressive slurring of speech, difficulty swallowing, and muscle wasting with twitching over nine months. [1]

History of presenting complaint: His speech became slurred and slow around nine months ago and has steadily worsened; his wife now struggles to understand him on the phone. He coughs when drinking water and has lost six kilograms. His arms have become weak and wasted, with visible twitching under the skin. He has noticed stiffness and cramps in his legs. He is emotionally labile — he laughs and cries inappropriately, which distresses him. There is no sensory loss, no visual change, and no sphincter disturbance. [1]

Past history: hypertension, no diabetes, no family history of neurological disease. [1]

Current medications: perindopril, amlodipine. Riluzole was recently commenced by his neurologist. [1]

Examination findings (trainee elicits):

• Alert, oriented, cognition intact (MMSE 28/30, though the speech difficulty makes this hard).
• Speech: slow, strained, spastic-sounding dysarthria with poor articulation; the language is intact (he understands, names objects, follows commands, and when asked to write writes a clear, grammatical sentence). There is no aphasia.
• Tongue: wasted and fasciculating at rest; on protrusion it is slow and spastic but moves fully.
• Jaw jerk: brisk. Gag reflex: brisk.
• Palate: elevates poorly and symmetrically.
• Limbs: widespread wasting and fasciculations in both arms, more marked distally, with UMN signs — brisk reflexes, sustained clonus at both ankles, and extensor plantar responses. Tone is increased in the legs. Power is reduced symmetrically, 4/5 in the arms and 4+/5 in the legs. Sensation is normal. Coordination is normal.
• Respiratory: FVC 55 per cent of predicted; he is breathless on lying flat. [1]

Candidate's structured presentation (model)

Opening statement: [1]

"Mr Chen is a 64-year-old former carpenter who presents with a nine-month history of progressive slurred speech, difficulty swallowing, weight loss, arm wasting with twitching, leg stiffness, and emotional lability. On examination he has a spastic dysarthria with intact language — he writes a clear sentence — a wasted and fasciculating tongue that is also spastic, a brisk jaw jerk and gag, poor palatal elevation, and in the limbs a combination of lower motor neuron signs (wasting, fasciculations) and upper motor neuron signs (brisk reflexes, clonus, extensor plantars), with preserved sensation and coordination. His FVC is 55 per cent of predicted and he is orthopnoeic. [1]

His main problems are:

1. Amyotrophic lateral sclerosis (motor neuron disease) with combined UMN and LMN signs in a bulbar and limb distribution
2. A spastic and flaccid (mixed) dysarthria from bulbar and pseudobulbar involvement
3. Dysphagia with aspiration risk and a six-kilogram weight loss
4. Respiratory muscle weakness with an FVC of 55 per cent and orthopnoea — a prognostic red flag
5. Pseudobulbar affect (emotional lability)
6. The psychological and advance-care-planning impact of a life-shortening neurodegenerative diagnosis [1]

This is motor neuron disease. My priorities are to confirm the diagnosis, optimise function and safety — particularly swallowing and breathing — commence or continue disease-modifying and symptomatic treatment, and begin advance care planning with Mr Chen and his family." [1]

Management plan: [1]

1. Confirm the diagnosis with the El Escorial criteria. The combination of UMN and LMN signs in bulbar and multiple spinal regions, with progressive spread and no sensory loss, meets the criteria for clinically probable or definite ALS. I would arrange nerve conduction studies and EMG to document the widespread active denervation, and an MRI brain and cervical spine to exclude a structural mimic (a foramen magnum lesion, cervical myelopathy with radiculopathy).
2. Disease-modifying therapy. Riluzole, a glutamate antagonist, has been commenced and should be continued — it modestly prolongs survival (by about three months in the original trial) and is the standard of care. Newer agents (edaravone) may be considered with the neurologist. There is no cure.
3. Swallowing and nutrition. Refer to speech and language therapy and dietetics for a swallowing assessment and dietary modification. Given the weight loss and aspiration risk, I would discuss early gastrostomy (PEG or RIG) for nutritional support and medication access, preferably placed before the FVC falls below 50 per cent of predicted to reduce procedural risk.
4. Respiratory management — the key prognostic domain. An FVC of 55 per cent with orthopnoea indicates diaphragmatic weakness and predicts respiratory failure. I would arrange formal respiratory function tests including sniff nasal inspiratory pressure, discuss non-invasive ventilation (NIV) early — which improves survival and quality of life in ALS — and screen for symptoms of nocturnal hypoventilation (morning headache, daytime somnolence, orthopnoea).
5. Symptomatic therapy. A low-dose SSRI or amitriptyline for pseudobulbar affect and sialorrhoea; antispasticity agents (baclofen) for leg stiffness; and a communication aid referral for the dysarthria, as speech deteriorates.
6. Multidisciplinary care and advance care planning. Refer to a specialist MND clinic for coordinated multidisciplinary input (neurology, respiratory, speech therapy, dietetics, physiotherapy, occupational therapy, social work, palliative care). Initiate advance care planning early — discuss the trajectory, the role of NIV and gastrostomy, and the patient's wishes regarding future ventilation and end-of-life care, because cognitive involvement (though classically absent) can occur and decision-making capacity should be established while it is intact. [1]

Examiner discussion questions

Q: "How do you classify his speech disorder, and how does it differ from an aphasia?" [1]

"He has a dysarthria — a disorder of articulation — not an aphasia. The decisive test is that his language is intact: he understands, he names objects, he follows commands, and when I ask him to write, he writes a clear, grammatical sentence. An aphasic patient cannot do that, because aphasia is a disorder of the language system itself. His dysarthria is mixed — partly spastic (strained, effortful, from the bilateral UMN involvement of the corticobulbar tracts, the pseudobulbar component) and partly flaccid (from the wasted, fasciculating tongue and the LMN involvement of the brainstem motor nuclei, the bulbar component). The brisk jaw jerk and gag and the poor palatal elevation confirm the pseudobulbar (UMN) side, and the tongue wasting and fasciculation confirm the bulbar (LMN) side. The combination of both in the same patient is characteristic of motor neuron disease, which uniquely affects the upper and lower motor neurons together. So the speech examination alone, done systematically, points strongly to the diagnosis before I even examine the limbs." [1]

Q: "Why does his clock-drawing and writing remain intact when his speech is so impaired?" [1]

"Because his language and his visuospatial and executive cortex are spared — motor neuron disease classically spares cognition and the sensory system, affecting only the motor pathways. His dysarthria is a pure motor articulatory problem: the muscles of speech are weak and spastic, but the brain that selects and orders the words is intact. That is exactly why he can write a perfect sentence — writing bypasses the impaired articulatory muscles and uses the intact language output through a different (hand) motor pathway. This is the cleanest demonstration of the aphasia-versus-dysarthria distinction: a severely dysarthric patient with intact language and perfect writing, as opposed to an aphasic patient whose writing is as disordered as their speech. It also tells me his cognitive screen (the MMSE) is valid — the speech difficulty made it hard to administer, but the underlying cognition is intact." [1]

Q: "His FVC is 55 per cent and he is orthopnoeic. Why does this change your management?" [1]

"Because respiratory muscle weakness is the single most important prognostic factor and the commonest cause of death in motor neuron disease. An FVC below 50 per cent of predicted predicts respiratory failure within months and is also the threshold at which procedural risk (for a gastrostomy) rises, so it is the trigger for several simultaneous actions. First, I arrange formal respiratory assessment including sniff nasal inspiratory pressure, which detects diaphragmatic weakness earlier than FVC. Second, I discuss non-invasive ventilation, which in ALS has been shown to improve both survival and quality of life when introduced once symptoms or objective respiratory impairment appear. Third, because his FVC is right at the gastrostomy threshold, I would move the PEG or RIG discussion forward and place it promptly, before the FVC falls further and the procedure becomes hazardous. Fourth, the orthopnoea is a symptom of nocturnal hypoventilation, so I would ask specifically about morning headaches and daytime somnolence, and arrange overnight oximetry. The respiratory domain is where timely intervention changes the trajectory, so it is the priority once the diagnosis is secure." [1]