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A 62-year-old male with Parkinson’s disease has attended the local neurology clinic after prompting by his wife due to sleep disturbances. He has no other past medical history and is self-caring. On closer questioning, his wife reports that he has been thrashing out in his sleep and that he occasionally shouts out at night. The patient is unaware of the events himself.
He is maintained on ropinirole. He denies taking any new medications and has been otherwise well in himself lately.
On examination, you note typical features of parkinsonism with no unexpected neurological findings.
What is next step in management to treat his symptoms?
A 29-year-old Cuban gentleman who is an office worker attends outpatient clinic with progressive walking difficulties over 8 years.
On examination, he has a broad-based gait. Ocular movements reveal saccadic slowing with an increased latency and saccade dysmetria. Pursuit it broken and the vestibulo-ocular reflex is impaired bilaterally. There is no nystagmus and eye movements are full. Limb examination reveals a rest tremor, bradykinesia and limb ataxia. Plantar responses are extensor.
An MRI scan is shown below.
What is the likely cause?
A 31-year-old Japanese female presents to outpatient clinic with progressive 2-year history of unsteadiness. She has been using a stick to mobilise more recently.
On examination, you notice limb choreiform movements and evidence of limb rigidity, dysmetria, dysdiadochokinesia and heel-shin ataxia bilaterally. Cranial nerves revealed intersaccadic intrusions with multi-directional gaze-evoked nystagmus. MRI demonstrated cerebellar atrophy. A Montreal Cognitive Assessment score was 19/30 with delayed recall, attention and executive function most affected.
An Atrophin-1 gene mutation is identified.
What is the cause of her condition?
A 72-year-old female presents to the outpatient clinic with unsteadiness on her feet. She has noticed these symptoms after prolonged standing at church, which causes her legs to “freeze up” and give way.
What is the first line medication typically used to treat this condition?
A 30-year-old male is admitted to the Neurology ward with worsening gait and dementia. His symptoms have been present since his mid-twenties but have reached a nadir precipitating admission.
Examination reveals dysarthria and truncal ataxia. He has brisk reflexes with extensor plantar responses. There is bilateral rigidity and bradykinesia. His eye movements reveal multi-directional nystagmus.
His mother died of a similar condition but was undiagnosed.
He unfortunately passes away whilst in hospital and post-mortem analysis reveals amyloid clusters within the cortex and basal ganglia.
What is the likely cause of his presentation?
A 50-year-old female is seen in the outpatient neurology clinic due gait problems and “noisy” breathing at night. Her husband has a recording with noises heard on inspiration.
On examination, she has a broad-based gait with limb dysmetria and dysdiadochokinesia. Her speech is dysarthric, limb reflexes brisk with extensor plantar responses. There is bradykinesia with evidence of a jerky tremor of the outstretched hands.
An MRI-brain is performed and shown below.
What is the likely diagnosis?
A 64-year-old man presents with a 3-year history of gradually progressive action tremor. The tremor has failed to respond to primidone and topiramate.
Past medical history includes mild depression and asthma.
Neurological examination is notable for an action tremor with intention component and a slightly broad-based gait. He undergoes MR imaging which is diagnostic.
Which of the following is most accurate in relation to this gentleman’s diagnosis?
A 72-year-old male is admitted to hospital with severe delirium in the context of a UTI. He is noted to have florid visual hallucinations. Collateral history from his wife reveals low mood, some difficulty with planning and carrying out certain tasks for the past year. He has had some brief episodes of disorientation at night, during which he has described seeing people and animals that are not present.
Which of the following is most accurate in relation to the likely diagnosis?
A 20-year-old female with a longstanding history of fluctuating predominantly lower limb dystonia and walking difficulties since childhood has a dramatic improvement after taking levodopa.
Which of the following statements is true of her diagnosis?
A 53-year-old man undergoes a CT head after a suspected first seizure (shown below). Past medical history includes anxiety and depression.
On examination you note mild symmetrical parkinsonism and mild gait ataxia.
CSF evaluation is unremarkable, including negative infectious serological work-up. A quantiferon test is outstanding.
Which of the following best explains these imaging abnormalities in this patient?
A 75-year-old female with a 7-year history of manifest Parkinson’s disease attends for a routine outpatient review. She reports that her motor symptoms had been well controlled until recently, but things have deteriorated over the last 2 months. Her deterioration coincides with stopping entacapone on the advice of her Parkinson’s nurse due to diarrhoea. Currently she describes a good quality ‘ON’ state but with re-emergence of tremor and bradykinesia 30 minutes before her next dose of Sinemet is due. She has no concerning non-motor symptoms at present, but she has in the past had mild impulsive spending when taking pramipexole.
You examine the patient 1 hour after taking her morning medications. She has minimal evidence of parkinsonism and no dyskinesia.
Her medication for Parkinson’s includes Sinemet 25/100mg plus Sinemet 12.5/50mg QDS, Rasagiline 1mg OD and Sinemet CR 50/200mg at night.
What would be the most appropriate alteration to this patient’s management?
A 58-year-old male is assessed in general neurology outpatients with a 1-year history of right-hand tremor and reduced finger dexterity. He reports stiffness of the right shoulder. Further questioning reveals sleep disturbance suggestive of REM sleep behaviour disorder and mild anxiety with no symptoms of autonomic disturbance or cognitive change.
Examination findings include reduced facial expression, normal speech, a unilateral thumb-involving rest tremor and mild-moderate cogwheel rigidity of the right upper limb. Gait assessment reveals a reduced arm swing on the right with otherwise normal initiation, speed and stride length and a negative pull test.
Which of the following is most accurate based on the above symptoms and signs?
A 66-year-old female presents with a decline in walking speed and reports having generally “slowed down”. She has recently undergone a CT brain arranged by her general practitioner.
Which of the following clinical features would raise concern about proceeding to neurosurgical intervention in this case?
A 70-year-old female with a previous diagnosis of restless leg syndrome attends the outpatient clinic. Her symptoms were well controlled with pramipexole MR for several years but have gradually returned. In the last 12 months she has noticed symptoms start earlier in the day and the uncomfortable sensation now involves her arms. Symptoms are temporarily relieved by moving but return shortly after. She reports being unable to sleep on several nights of the week due to her symptoms. Increasing the dose of pramipexole MR with her GP from 520mcg OD up to 1.05mg OD has not improved the situation.
Her neurological examination is normal. A recent serum ferritin is at the upper end of the normal range.
Which of the following would be the most appropriate next step in her management?
A 29-year-old male undergoes a CT brain as part of a research study. This is reported to show bilateral calcification in the basal ganglia. She is referred to the neurology clinic for further evaluation.
She is estranged from her family but reports that her father developed tremor, slow unsteady walking and depression/anxiety in mid-life which got progressively worse. She denies any neurological symptoms and her only medical history is of mild anxiety and obsessive-compulsive disorder. There is no history of toxic/ chemical exposure and no regular medications.
Serum calcium, thyroid function and parathyroid hormone levels are all normal.
Which if the following is most accurate in relation to likely underlying condition?
A 58-year-old man is admitted with acute onset ataxia and vertigo. His symptoms improve over the course of 2 days.
An MRI brain with diffusion weighted imaging is negative for a stroke.
He tells you he has previous episodes that have presented similarly, one of which was diagnosed as a transient ischemic attack. He recalls his father having similar episodes.
He has a history of hypertension. He feels well between attacks.
Examination is notable for downbeat nystagmus and mild gait ataxia.
Reviewing the MRI scan with the neuroradiology consultant, subtle midline cerebellar atrophy is noted alongside mild-moderate small vessel ischemic disease.
What is the correct diagnosis?
A 78 year-old-female is referred to the outpatient clinic. She was diagnosed with Parkinson’s disease 18 months ago and currently takes co-careldopa 25/100mg TDS, which has been gradually up-titrated but unhelpful to date. She has had 4 completed falls in the last 6 months, resulting in head injury on one occasion. She describes some difficulty swallowing, a change in her voice and difficulty opening her eyes, particularly in the morning. She has a tendency to be emotionally labile, which is out of character.
On examination she has moderate dysarthria, slowing of vertical saccades, no tremor, moderate axial and symmetrical limb rigidity and moderate bradykinesia. Her gait is relatively upright and she takes 4 steps to correct her balance on a pull test. Her MRI is shown below.
What is the most likely diagnosis?
Regarding device-assisted therapy for Parkinson’s disease (PD), which of the following is correct:
Regarding surgical treatment of idiopathic Parkinson’s disease (PD), which of the following is correct?
Which of the following drugs used to treat Parkinson’s is an antiviral?
With respect to chorea, which of the following statements is correct?
A 62-year-old gentleman presents to the outpatient Neurology department with bilateral hand tremors. His past medical history includes asthma and chronic migraine.
Regular medications are ventolin, beclomethasone inhaler, and amitriptyline.
On examination, you note a rhythmic 4-12Hz tremor of the outstretched hands on action and during isometric hold but not during rest. Archimedes spirals and handwriting are not micrographic. There is no evidence of an extrapyramidal disorder.
Which of the following medications would be optimal to start?
A 24-year-old male is being seen in The Outpatient Department with a progressive difficulty mobilising and clumsiness. He has a past medical history of depression and anxiety for which he takes sertraline.
On examination, he has a festinant gait, jerky irregular dystonic tremor and dysarthria with slow tongue movements and risus sardonicus. His sensory examination is normal. Deep tendon reflexes are globally brisk.
A diagnosis of Wilson disease is suspected. Which of the following copper test results would be compatible with this diagnosis?
A 62-year-old man is reviewed in the outpatient movement disorder clinic. He was diagnosed with parkinsonism 3 years ago. He reports no tremor but troublesome stiffness and slowness which has not improved with Co-careldopa which he is taking at dose of 50/200mg QDS. He reports 3 falls in the last year. He reports dizziness on standing and postural blood pressure testing reveals a systolic drop of 28mmHg.
You suspect this could be multiple system atrophy (MSA) and arrange an MRI as part of further investigation which is shown below.
Which of the following additional features would be necessary to make a diagnosis of clinically established MSA?