Medicine 9 Flashcards by Akash Srinivasan

Outline the investigations that may be used in patients with suspected Charcot-Marie-Tooth disease.

Nerve conduction studies (demyelination will cause reduced velocity, axonal degeneration will cause reduced amplitude) 
Genetic testing (PMP22 gene)

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Outline the management options for patients with Charcot-Marie-Tooth disease.

MDT: GP, neurologist, specialist nurses, physiotherapist, OT, orthotics
Foot care and careful shoe choice
Orthotics

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Describe the pathway of the direct and consensual light reflex.

Retina –> Optic Nerve –> Pre-Tectal Nucleus –> Edinger-Westphal Nucleus (both) –> Oculomotor Nerve –> Ciliary Ganglion –> Pupillary Sphincter

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Lesions in which parts of the nervous system could cause symmetrical weakness?

Cord lesions
Neuromuscular junction pathology (MG, LEMS)
Muscle pathology (e.g. myositis)

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What are the different patterns of sensory loss and which lesions are they typically associated with?

Distal sensory loss - peripheral neuropathy (e.g. DM, B12, alcohol)
Sensory level - cord lesion (e.g. cord compression)
Dissociated sensory loss (e.g. loss of pain/temp with conservation of light touch) - cervical cord lesions (e.g. syringomyelia

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Describe the main features of an anterior cerebral artery stroke.

Contralateral motor/sensory loss in LEGS > arms

Face is spared

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Describe the main features of a middle cerebral artery stroke.

Contralateral motor/sensory loss in FACE/ARMS > legs
Contralateral homonymous hemianopia
Cognitive changes (dominant = aphasia; non-dominant = neglect/apraxia)

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Describe the main features of a posterior cerebral artery stroke.

Contralateral homonymous hemianopia with macula sparing

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What are the different anatomical levels at which pathology can cause muscle weakness?

Cerebrum/Brainstem - stroke, SOL, MS
Cord - MS, injury
Anterior Horn - MND, Polio
Nerve Roots/Plexus - cauda equina, cord compression
Motor Nerve - compression, mononeuritis multiplex, CMT
NMJ - Myasthenia, LEMS
Muscle - myositis, muscular dystrophy

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List some causes of bilateral foot drop.

Charcot-Marie-Tooth disease
Guillain Barre syndrome
Cauda equina

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List some causes of unilateral lower limb spasticity.

Stroke
MS
SOL

NOTE: it will cause a circumducting gait

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List some causes of bilateral lower limb spasticity.

Spinal Cord (compression, trauma, transverse myelitis) 
Cerebral palsy (bihemispheric)
MS (bihemispheric)

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List some causes of mixed UMN and LMN symptoms.

Motor neurone disease
Friedreich ataxia
SACD
Taboparesis (syphylis)

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List some causes of sensory ataxia.

Vestibular - Meniere’s disease, viral labyrinthitis

Proprioceptive Loss - B12 deficiency, DM, alcohol

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List some causes of a postural tremor.

Benign essential tremor 
Endocrine (thyrotoxicosis, hypoglycaemia)
Alcohol withdrawal
Beta-agonists 
Anxiety (physiological)

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What is idiopathic focal dystonia?

Most common form of dystonia
Confined to one body part
E.g. spasmodic torticollis, blepharospasm, Writer’s cramp

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List some causes of chorea.

Huntington’s chorea
Sydenham’s chorea (rheumatic fever)
Wilson’s disease
L-DOPA

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Define dementia.

Chronically impaired cognition that affects multiple domains (memory, attention, language) without impairment of consciousness

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List some causes of delirium.

Infection (UTI, sepsis)
Dehydration
Constipation
Drugs (e.g. opioids)
Metabolic (e.g. hyponatraemia, hypoglycaemia)
Intracranial pathology (e.g. subdural haemorrhage)

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List some complications of subarachnoid haemorrhage.

Rebleeding
Cerebral ischaemia (vasospasm)
Hydrocephalus
Hyponatraemia

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Which members of the MDT would be involved in the management of patients who have had a stroke?

Neurologist 
Physiotherapist 
SALT 
Dietician 
OT 
Specialist nurse

Which investigations should be done to find a potential source of an embolus in a patient with a TIA?

ECG
Echocardiogram
Carotid artery doppler

Check Risk Factors: FBC, U&E, ESR, glucose, lipids

Which scoring system is used to assess the risk of future stroke in a patient who has had a TIA?

ABCD2
4 or more = TIA clinic within 24 hours
< 4 = TIA clinic within 1 week

What are the typical features of cerebral venous sinus thrombosis?

Headache 
Vomiting 
Seizures 
Reduced visual acuity 
Papilloedema 
Proptosis (cavernous sinus thrombosis)

NOTE: DDx - SAH, meningitis, managed with LMWH/warfarin, fibrinolytics

List some risk factors for cerebral venous sinus thrombosis.

``` Pregnancy OCP Head injury Dehydration Malignancy Thrombophilia ```

List some contraindications for lumbar puncture.

Raised ICP Thrombocytopaenia Coagulopathy Infection at LP site

List some causes of provoked seizures.

Withdrawal (alcohol, opiates) Metabolic (hypoglycaemia, hypocalcaemia) Raised ICP (trauma, haemorrhage, SOL) Infection (encephalitis)

Which investigations should be requested in a patient who has had a seizure?

``` FBC, U&E, Glucose AED levels Urine toxicology ECG EEG MRI (if adult and risk of SOL) ```

What are the types of cerebral oedema?

Vasogenic (increased capillary permeability) - trauma, tumour, ischaemia, infection Cytotoxic - e.g. hypoxia Interstitial - e.g. obstructive hydrocephalus, hyponatraemia

Which sensory and motor signs might you see in a patient with multiple sclerosis?

MOTOR: spastic weakness, transverse myelitis (weakness + numbness) SENSORY: paraesthesia, reduced vibration sense NOTE: patients may also have cerebellar signs (e.g. ataxia, dysarthria)

What are some differentials for multiple sclerosis?

CNS sarcoidosis | SLE

Which signs might you see in a patient with spinal cord compression?

Back pain Progressive UMN weakness and sensory loss below the lesion (e.g. bilateral spasticity in lower limbs) Radicular pain in dermatomal distribution NOTE: tone and reflexes are initially REDUCED in acute cord compression

Describe the clinical features of conus medullaris lesions.

Early constipation/retention Mixed UMN/LMN weakness Back pain Sacral sensory disturbance

``` Which muscles are weak in the following nerve lesions? C5fac C6 C7 C8 ```

C5 - deltoid, supraspinatus C6 - biceps, brachioradialis (reduced biceps jerk) C7 - triceps, finger extension (reduced triceps jerk) C8 - finger flexors, intrinsic hand muscles

How does L5 radiculopathy manifest?

Weak hallux extension Foot drop Reduced sensation on inner dorsum of foot NOTE: MRI lumbar spine would be useful to check for compression

How does S1 radiculopathy manifest?

Weak plantarflexion and eversion Loss of ankle jerk Reduced sensation over sole of foot and back of calf NOTE: MRI lumbar spine would be useful to check for compression

What are some complications of Bell's palsy?

``` Synkinesis (e.g. blinking causes upturning of mouth) Crocodile tears (eating stimulates unilateral lacrimation) ```

What are the features of radial nerve palsy?

``` Finger drop (low) Wrist drop (high) Triceps paralysis (very high) Loss of sensation of first dorsal web space ```

What are the features of sciatic nerve palsy?

Weakness of knee flexion Weakness of all muscles below the knee Loss of sensation below knee laterally and foot

What are the features of common peroneal nerve palsy?

Foot drop Week dorsiflexion and eversion Loss of sensation below knee laterally

What are the features of tibial nerve injury?

Weak plantarflexion (can't stand on toes) Weak toe flexion Loss of sensation to sole of foot

List some causes of peripheral motor neuropathy.

Guillain Barre syndrome Botulism Charcot-Marie-Tooth Lead poisoning

List some causes of autonomic neuropathy.

Diabetes mellitus HIV SLE Guillain-barre syndrome

Outline the management of motor neurone disease.

MDT: neurologist, physio, OT, dietician, specialist nurse, GP Medical: riluzole (antiglutamatergic) Symptomatic: NG/PEG feeding, NIV for respiratory failure, analgesia, baclofen for spasticity NOTE: most die of respiratory failure or infection

List some causes of bulbar and pseudobulbar palsy.

Bulbar: MND, GBS, myasthenia, central pontine myelinolysis Pseudobulbar: MS, stroke, MND

Describe the typical clinical features of polio.

Asymmetrical LMN paralysis | No sensory involvement

What are the clinical features of fascioscapulohumeral muscular dystrophy?

Weakness of face, shoulders and upper arms (often asymmetrical) Winging of the scapula Foot drop

List some causes of cafe au lait spots.

NF1 McCune Albright syndrome Noonan syndrome

Outline the management of NF1.

MDT approach Epilepsy management Excise some neurofibromas (cosmetic/sarcomatous) Genetic counselling NOTE: it is autosomal dominant

Outline the management of benign essential tremor.

``` Avoidance of exacerbating factors (coffee, stress) Beta-blockers (e.g. propanolol) Anti-epileptics (e.g. topiramate) Benzodiazepines (e.g. clonazepam) Botulinum injections MRI-guided high intensity ultrasound ```