Neuro Part 2

Question 1

What is the pathophysiology of Parkinsons Disease?

Answer 1

• Progressive degenerative disorder characterised by neuronal loss in brainstem and basal ganglia of the brain, leading to disorders of movement.
• Loss of dopaminergic neurones in substantia nigra leads to inadequate dopamine transmission
• Can lead to lewy body formation in affected neurones

Question 2

What is the triad of fx seen in PD?

Answer 2

• Resting tremor - 4-6 Hz resting tremor
• Muscular Rigidity
• Bradykinesia

Question 3

What are some of the signs + sx of PD?

Answer 3

• Unilateral Tremor: Pill rolling tremor: 4-6 Hz (4-6 times a second)
• Bradykinesia: movements get slower and smaller.
• “Cogwheel” Rigidity
• Shuffling gait: they can only take small steps when walking
• Hypomimia: reduced facial movements and facial expressions
• Micrographia
• Autonomic dysfunction: postural hypotension, constipation, urinary frequency/urgency, dribbling of saliva
• Neuropsychiatric complications: depression, dementia, psychosis, sleep disturbance, Cognitive impairment and memory problems

Question 4

What are some of the differences between the PD tremor and benign essential tremor

Answer 4

Question 5

What features form the diagnostic criteria for PD

Answer 5

Three or more required for diagnosis of definite Parkinson’s disease in combination with step one:

• Unilateral onset
• Rest tremor present
• Progressive disorder
• Persistent asymmetry affecting side of onset most
• Excellent response (70-100%) to levodopa
• Severe levodopa-induced chorea
• Levodopa response for 5 years or more
• Clinical course of ten years or more

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Question 6

What is parkinsonism?

Answer 6

Parkinsonism – bradykinesia + rigidity NO tremor

Question 7

What medications are used for the mx of PD?

Answer 7

• Levodopa - synthetic dopamine (SE: dyskinesias. dystonia, chorea, athetosis **, nausea, hallucinations, psychosis, low BP)
• Peripheral decarboxylase inhibitors. (stops levodopa being broken down in the body before entering brain): carbidopa and benserazide
• Combination:
  
  • Co-benyldopa (levodopa and benserazide)
  • Co-careldopa (levodopa and carbidopa)
• COMT Inhibitors: entacapone: Inhibitors of catechol-o-methyltransferase (COMT) which metabolises levodopa in both the body and brain.
  
  • Taken with levodopa to slow its breakdown (extend duration)
• Dopamine Agonists: Bromocryptine., pergolide, Carbergoline - mimic dopamine in the basal ganglia and stimulate the dopamine receptors
• Monoamine Oxidase-B Inhibitors: Selegiline, Rasagiline - Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline.

Question 8

What are the fx of benign essential tremor

Answer 8

• Fine tremor
• Symmetrical
• More prominent on voluntary movement
• Worse when tired, stressed or after caffeine
• Improved by alcohol
• Absent during sleep

Question 9

How is benign essential tremor mx’d?

Answer 9

There is no definitive treatment for benign essential tremor

Medications:

• Propranolol
• Primidone (a barbiturate anti-epileptic medication)

Question 10

What muscles does CNVII innervate?

Answer 10

• Face: Muscles of facial expression
• Ear: nerve to stapedius in the inner ear and the posterior digastric, stylohyoid and platysma muscles in the neck.
• Taste: Sensory: anterior 2/3 of the tongue.
• Tear: Parasympathetic: it provides the PS to the submandibular and sublingual salivary glands and the lacrimal gland (stimulating tear production).

Question 11

What are the different causes of LMN and UMN facial nerve lesions?

Answer 11

Question 12

What is Bell’s Palsy? How is it mx’d?

Answer 12

• Idiopathic unilateral LMN facial nerve palsy.
• The peak incidence is 20-40 years and the condition is more common in pregnant women.
• Mx: if in 72h of presenting symptoms: prednisolone
  
  • 50mg for 10 days
  • 60mg for 5 days followed by a 5-day reducing regime of 10mg a day
  • Other: lubricating eye drops
    
    • Exposure keratopathy: urgent opthalmological review

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Question 13

What are the sx of Bell’s palsy?

Answer 13

• LMN facial nerve palsy - forehead affected
• Post-auricular pain (may precede paralysis)
• Altered taste
• Dry eyes
• Hyperacusis

Question 14

What is Ramsay Hunt Syndrome? What is its mx?

Answer 14

• Varicella zoster virus (VZV).
• Unilateral LMN facial palsy (like Bells)
• Presentation: painful and tender vesicular rash in the ear canal, pinna and around the ear on the affected side. This rash can extend to the anterior 2/3 of the tongue and hard palate
• Mx: in 72h: Prednisolone and Aciclovir

Question 15

What are some of the cause of bilateral facial nerve palsies?

Answer 15

• Sarcoidosis
• Guillain-Barre syndrome
• Lyme disease
• Bilateral acoustic neuromas (neurofibromatosis T2)

Question 16

What is MS?

Answer 16

• Chronic and progressive condition that involves demyelination (plaques) of the myelinated neurones in the CNS. This is caused by an inflammatory process involving the activation of immune cells against the myelin
• Typically presents in young adults (under 50 years) and is more common in women.
• Symptoms tend to improve in pregnancy and in the postpartum period.

Question 17

Describe the pathophysiology of MS?

Answer 17

• Inflammation around myelin and infiltration of immune cells that cause damage to the myelin. This affects the way electrical signals travel along the nerve leading to the symptoms of multiple sclerosis.
• Early: re-myelination can occur and sx can resolve.
• Later: re-myelination is incomplete and sx gradually become more permanent.
• MS lesions “disseminated in time and space”.

Question 18

What are some of the causes of MS?

Answer 18

Multiple genes, Epstein–Barr virus (EBV). Low vitamin D, Smoking, Obesity

Question 19

What are some of the signs + sx of MS?

Answer 19

• Eye movement abnormalities: Abducens nerve palsy: diplopia and internuclear ophthalmoplegia and conjugate lateral gaze disorder.
• Focal weakness: Bells palsy. Horners syndrome, Limb paralysis, Incontinence
• Focal sensory symptoms: Trigeminal neuralgia, Numbness, Paraesthesia (pins and needles)
• Lhermitte’s sign: an electric shock sensation that travels down the spine and into the limbs when flexing the neck.
• Optic neuritis: unilateral reduced vision developing over hours to days
• Ataxia: sensory or cerebellar

Question 20

What are some of the features of optic neuritis and its mx’d?

Answer 20

• Central scotoma
• Pain on eye movement
• Impaired colour vision
• RAPD
• Mx: urgent ophthalmologist referral for assessment.
• Medical: steroids and recovery takes 2-6 weeks

Question 21

What criteria is used for the diagnosis of MS?

Answer 21

McDonald Criteria for diagnosing MS

1. Lesions consistent with an inflammatory process
2. No alternative diagnosis
3. Multiple lesions in time and space (remitting MS)
4. Progressive neurological deterioration for 1 y (primary progressive MS)

Question 22

How is MS Ix’d + mx’d?

Answer 22

Ix:

• MRI: typical lesions – demyelination plaques
• LP: “oligoclonal bands” in CSF

Management

• MDT: Medical: DMARDs (Beta-interferon has been shown to reduce the relapse rate), biological therapies
  
  • Monoclonal antibodies: alemtyzumab, matalizunab
  • Non-immunosuppresives: glatiramer, mitoxantrone
  • Other: azathioprine
• Relapses: methylprednisolone:
  
  • 500mg orally OD for 5 days
  • 1g IV daily for 3–5 days where oral treatment has failed previously or where relapses are severe

Question 23

How is MS mx’d symptomatically?

Answer 23

• Neuropathic pain: amitriptyline or gabapentin
• Depression: SSRIs
• Urge incontinence: anticholinergic medications such as tolterodine or oxybutynin (although be aware these can cause or worsen cognitive impairment)
• Spasticity: baclofen, gabapentin, diazepam
• Tremor: botulinium toxin type a
• Fatigue: amantadine, cbt, and exercise

Question 24

What are some of the SEs of epilepsy mx medication?

Answer 24

• 1st line: Sodium Valproate. SE: teratogenic, liver damage and hepatitis, hair loss, tremor.
  
  • induces metabolism of vitamin D and she should have her Vitamin D levels measured routinely.
• Carbamazepine. SE: Agranulocytosis, Aplastic anaemia, Induces the P450 system so there are many drug interactions
• Phenytoin. SE: Folate and vitamin D deficiency; Megaloblastic anaemia (folate deficiency); Osteomalacia (vitamin D deficiency)
• Ethosuximide. SE: night terrors, rashes
• Lamotrigine. SE: stevens Johnson/ DRESS syndrome, leukopenia

Question 25

What fx describe a generalised tonic clonic seizure and how is the mx'd

Answer 25

* LOC and tonic (muscle tensing) and clonic (muscle jerking) episodes * Possible associated *tongue biting, incontinence, groaning and irregular breathing.* * **Post-ictal period:** occurs after the seizure there is prolonged confused, drowsy and irritability, depression. Confusion and myalgia are also common * Mx: 1st: ***sodium valproate; 2nd: lamotrigine or carbamazepine***

Question 26

What are some of the sx of absence seizures and how are they managed?

Answer 26

* Ytypically happen in children (90% stop as they get older) * Pt becomes blank, stares into space and then abruptly returns to normal. During the episode they are unaware of their surroundings and won’t respond. * These typically only lasts 10-20 seconds. * Mx: First line: sodium valproate or ethosuximide

Question 27

What are atonic seizures and how are they mx'd

Answer 27

* Brief lapses in muscle tone * Don’t usually last more than 3 minutes. * They typically begin in childhood. * Sudden weakness of legs - cause the patient to fall to the ground. No warning, no LOC and no confusion afterwards * Causes: hydrocephalus, narcolepsy * May be indicative of ***Lennox-Gastaut syndrome*** * Mx: 1st line: ***sodium valproate; 2nd:*** ***lamotrigine***

Question 28

What are myoclonic seizures?

Answer 28

* The patient usually remains awake during the episode * They occur in various forms of epilepsy but typically happen in children as part of ***juvenile myoclonic epilepsy***. * Mx: 1st ***sodium valproate;*** Other options: ***lamotrigine***, ***levetiracetam*** or ***topiramate***

Question 29

What are some the features of focal seizures?

Answer 29

They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present: * Hallucinations * Memory flashbacks * Déjà vu * Doing strange things on autopilot Mx (reverse of tonic-clonic) * First line: ***carbamazepine*** or ***lamotrigine*** * Second line: ***sodium valproate*** or ***levetiracetam (safe in pregnancy)***

Question 30

What are some the common paediatric and adult intracranial tumours found\>

Answer 30

Question 31

What are the two common types of adult tumour and how do they present\>

Answer 31

Question 32

What features faour pseudoseizures over seizures?

Answer 32

* pelvic thrusting * family member with epilepsy * much more common in females * crying after seizure * don't occur when alone * gradual onset

Question 33

How are intracranial tumours investigated?

Answer 33

Question 34

How are paediatric brain tumours managed?

Answer 34

Question 35

How are adult brain tumours mx'd?

Answer 35

Question 36

How do SOL present?

Answer 36

* **Seizures:** common px, with aura (funny smells or taste) - temporal * Focal neurology and gait disturbance * **Raised ICP** (actually rare): headaches, vomiting, papilledema * **Neuropsychiatric effects:** personality, mental state and memory and cognition * **Endocrine**: pituitary * Incidental finding on imaging

Question 37

How do the two common paediatric brain tumours present?

Question 38

How should pts for diabetic neuropathy be screened?

Answer 38

* All pts should be screened annually using **urinary albumin:creatinine ratio (ACR)** * should be an early morning specimen * ACR \> 2.5 = microalbuminuria

Question 39

How is diabetic nephropathy mx'd?

Answer 39

* Conservative: Dietary protein restriction; tight glycaemic control; BP control: aim for \< 130/80 mmHg * Benefits independent of blood pressure control have been demonstrated for ACE inhibitors (ACE-i) and angiotensin II receptor blockers (A2RB). Combinations of ACE-i and A2RB are not commonly used anymore following the ON-TARGET trial which showed worse outcomes for patients on dual blockade * Control dyslipidaemia e.g. Statins

Question 40

How is neuropathic pain mx'd?

Answer 40

* 1^st line: \*: amitriptyline, duloxetine, gabapentin or pregabalin * if the first-line drug treatment does not work try one of the other 3 drugs * in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added * tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain * topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia) * pain management clinics may be useful in patients with resistant problems

Question 41

What are the sx of Horners Syndrome?

Answer 41

Features * miosis (small pupil) * ptosis * enophthalmos\* (sunken eye) * anhidrosis (loss of sweating one side) Distinguishing between causes * heterochromia (difference in iris colour) is seen in congenital Horner's * anhidrosis: see pic

Question 42

What are some sx + ix of wernickes?

Answer 42

Features * nystagmus (the most common ocular sign) * ophthalmoplegia * ataxia * confusion, altered GCS * peripheral sensory neuropathy Investigations * decreased red cell transketolase * MRI