A 62-year-old man is admitted to the Emergency Department with a left hemiplegia. His symptoms started around 5 hours but he initially thought he had slept in an awkward position. He has no past medical history of note but on examination is found to have and irregular pulse of 150 / min. The ECG confirms atrial fibrillation. A CT head is immediately arranged and reported as normal.
What is the most appropriate initial management?
Correct Answer A: Rate control should also be initiated. He is outside the thrombolysis window so alteplase is not an option. The 2004 RCP guidelines recommend that anti-coagulation should be commenced 14 days after an ischaemic stroke.
Earlier anti-coagulation may exacerbate any secondary haemorrhage. Dipyridamole should not be used in the acute phase.
Stroke: management:
The Royal College of Physicians (RCP) published guidelines on the diagnosis and management of patients following a stroke in 2004. NICE also issued stroke guidelines in 2008, although they modified their guidance with respect to antiplatelet therapy in 2010.
Selected points relating to the management of acute stroke include:
Thrombolysis:
Thrombolysis should only be given if:
Secondary prevention: NICE also published a technology appraisal in 2010 on the use of clopidogrel and dipyridamole Recommendations from NICE include:
With regards to carotid artery endarterectomy:
*the 2009 Controlling hypertension and hypotension immediately post-stroke (CHHIPS) trial may change thinking on this but guidelines have yet to change to reflect this
**SIGN recommend a window of 4.5 hours
***European Carotid Surgery Trialists' Collaborative Group
****North American Symptomatic Carotid Endarterectomy Trial
A 27-year-old female presents complaining of generalized weakness. Examination of her face reveals bilateral ptosis, dysarthric speech and a slow-relaxing grip.
What is the most likely diagnosis?
Correct Answer A:
Dystrophia myotonica - DM1:
The slow-relaxing grip may be noticed on initial hand-shake with the patient and is typical of myotonic dystrophy.
Dysarthric speech is secondary to myotonia of the tongue and pharynx.
Myotonic dystrophy:
Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.
Genetics:
The key differences are listed in table below:
General features:
Other features:
A 63-year-old woman with motor neuron disease is reviewed in clinic.
Which one of the following interventions will have the greatest effect on survival?
Correct Answer E: Motor neuron disease - treatment: NIV is better than riluzole.
Motor neuron disease: management:
Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognized including amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy.
Riluzole:
Respiratory care:
Prognosis:
A 70-year-old man is investigated for involuntary, jerking movements of his arms. His symptoms seem to resolve when he is asleep.
Damage to which one of the following structures may lead to hemiballism?
Correct Answer C: Hemiballism is caused by damage to the subthalamic nucleus.
Hemiballism:
Hemiballism occurs following damage to the subthalamic nucleus. Ballistic movements are involuntary, sudden, jerking movements which occur contralateral to the side of the lesion. The ballistic movements primarily affect the proximal limb musculature whilst the distal muscles may display more choreiform-like movements Symptoms may decrease whilst the patient is asleep.
Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment.
A 63-year-old man is diagnosed as having restless legs syndrome.
What is the most relevant blood test to perform?
Correct Answer B: A case could be made for all the above tests but a low serum ferritin is most likely to be a cause of secondary restless legs syndrome.
Restless legs syndrome:
Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present.
Clinical features:
Causes and associations:
The diagnosis is clinical although bloods to exclude iron deficiency anaemia may be appropriate.
Management:
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