Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Phase 3a Medicine > Neurology > Flashcards

Neurology Flashcards

1
Q

Syncope

A

Loss of consciousness due to transient global cerebral hypoperfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the commonest cause of transient loss of consciousness?

A

Syncope

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the most common cause of syncope?

A

Vasovagal (faint)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are some triggers of vasovagals?

A

Sudden surprise/fear
Pain
Sight of blood
Prolonged standing
Venesection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the mechanism of vasovagals?

A

Vagus nerve receives a strong stimulus, which stimulates the parasympathetic nervous system, resulting in reflex bradycardia +/- peripheral vasodilation, causing the BP to drop and hypoperfusion of the brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some causes of syncope?

A

Vasovagal
Carotid sinus hypersensitivity
Hypoglycaemia
Anaemia
Hypovolaemia, e.g. haemorrhage, ruptured aneurysm, dehydration
Cardiac
Infection
Anaphylaxis
Epileptic seizures
Postural hypotension
Functional
Hyperventilation
Micturition syncope
Cough syncope

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is carotid sinus hypersensitivity?

A

Mild external pressure on carotid bodies in the neck (shaving, neck turning, tight collar) induces a reflex response resulting in transient loss of consciousness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are some cardiac causes of syncope?

A

Arrhythmias
Valvular heart disease
Hypertrophic cardiomyopathy
Pulmonary embolus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is postural hypotension?

A

Drop in BP of more than 20mmHg or reflex tachycardia of more than 20bpm when a person goes from lying down or sitting to standing, due to delay in constriction of veins of the leg resulting in blood pools and less venous return to the heart

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are some medications that can increase your risk of syncope?

A

Block vasoconstriction: calcium channel blockers, beta blockers, alpha blockers, nitrates
Affect volume status: diuretics
Prolong the QT interval: antipsychotics, antiemetics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the clinical manifestation of syncope?

A

Brief prodrome: dizziness, lightheadedness, nausea, sweating, hot or clammy, blurry vision, pallor, feeling of heaviness, headache
Sudden loss of consciousness and fall to the ground, with unconsciousness lasting for a few to 30 seconds
Rapid recovery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Associated symptoms of syncope

A

Palpitations
Sweats
Pallor
Chest pain
Dyspnoea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Investigations for syncope

A

Bloods
* U&E (arrhythmia, seizure)
* FBC (anaemia)
* Mg and Ca
* Glucose (DM)
* ABG (hyperventilation)

Cardiovascular examination (palpitations, arrhythmias)
* 12-lead ECG +/- 24 hour ECG (arrhythmias, heart block, prolonged QT interval)
* Echocardiogram (structural heart disease

Neurological examination
* CT/MRI brain
* EEG

Lying and standing BP (postural hypotension)
* Tilt-table test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are some differences between syncope and seizures?

A

Triggers
Prodrome
Duration
Convulsions
Colour
Lateral tongue biting
Recovery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the management of vasovagals?

A
  • Avoid triggers
  • Early recognition of prodromal symptoms in order to sit/lie down or use physical counter-pressure manoeuvres (leg and knee crossing, squatting, hand gripping and arm tensing)
  • Lifestyle modifications: adequate fluid intake and regular meals
  • Review medications
  • Fludrocortisone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How to manage postural hypotension

A
  • Sit rather than stand where possible
  • Sit first when moving from lying to standing
  • Eat frequent small meals and adequate fluid intake
  • Head-up sleeping
  • Compression garments (abdominal binders or support stockings)
  • Early recognition of prodromal symptoms in order to sit/lie down or use physical counter-pressure manoeuvres (leg and knee crossing, squatting, hand gripping and arm tensing)
  • Review medications
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is brain death?

A

Irreversible loss of capacity for consciousness and to breathe due to cessation of brainstem function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Clinical manifestation of brain death

A

Apnoeic coma (unresponsive on a ventilator, with no spontaneous respiratory efforts)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the management for brain death?

A
  • Stop mechanical venilation and other life-supporting measures
  • Retrieval of organs while heart is still beating for transplantation (if organ donor)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How is brain death examined?

A

2 senior doctors
* Absent reflexes - oculocephalic, light, corneal, vestibulo-occular on caloric testing, gag or cough
* Fixed, unresponsive pupils
* No motor response within the cranial nerve territory to painful stimuli applied centrally or peripherally
* Absent spontaneous respiration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What must be diagnosed in order to announce brain death?

A

Irremediable structural brain damage due to a disorder that can cause brainstem death, e.g. head injury, intracranial haemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What reversible causes of coma must be excluded before announcing brain death?

A
  • Sedative drugs, poisoning or neuromuscular blocking agents
  • Hypothermia
  • Metabolic disturbances
  • Endocrine disturbances
  • Abnormal plasma electrolytes
  • Abnormal acid-base balance
  • Abnormal blood glucose levels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Define essential tremor

A

Fine tremor during voluntary muscle contraction that typically involves the hands and is brought about by anti-gravity positions, e.g. outstretched hands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are the characteristics of an essential tremor?

A
  • Fine tremor (6-12Hz)
  • Symmetrical
  • More prominent with voluntary movement or voluntarily maintained in a certain position (drinking from a cup)
  • Improved by alcohol
  • Absent during sleep
  • Progressive disorder that worsens over time
  • Most notable in hands but can affect other areas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What are risk factors for an essential tremor?
Older Family history (autosomal dominant)
26
How is essential tremor diagnosed?
Clinically: upper limb action tremor +/- tremor in other sites for over 3 years with no other neurological features
27
What investigation rules out other potential diagnoses of essential tremor?
MRI/dopamine transporter CT scan
28
What is the management of an essential tremor?
* Stop triggers * Propranolol (beta blocker) * Primidone (antiepileptic) Only if causing problems!
29
Motor neurone disease
Progressive, degenerative and eventually fatal disease where the motor neurones stop functioning
30
What are the different types of motor neurone disease and which is the most common?
* **Amyotrophic lateral sclerosis**: UMN + LMN * Progressive bulbar palsy: LMN of cranial nerves 9, 10, 11 and 12 * Progressive muscular atrophy: LMN * Primary lateral sclerosis: UMN
31
What are the risk factors for motor neurone disease?
* Advancing age (60) * Male * Family history * Smoking
32
What genetics are associated with amyotrophic lateral sclerosis?
* Mutations in superoxide dismutase (SOD1) * Excess hexanucleotide repeats (GGGGCC)
33
What is the classic presentation of motor neurone disease?
Insidious, progressive weakness of muscles throughout the body, affecting limbs, trunk, face and speech, often first noticed in the upper limbs * Hand weakness, with loss of dexterity and dropping things * Wrist drop * Foot drop, with difficulties with balance, particularly when running, leading to tripping over
34
What are signs of LMNs being affected in MND?
* Muscle wasting * Reduced tone * Fasciculation, especially the tongue * Reduced reflexes * Muscle cramps
35
What are signs of UMNs being affects in MND?
* Increased tone or spasticity * Brisk reflexes * Upgoing plantar reflex * Jaw clenching * Exaggerated jaw jerk
36
What are signs of progressive bulbar palsy?
* Dysarthria * Dysphasia * Nasal regurgitation of fluids and choking * Absent jaw jerk reflex * Flaccid and fasciculating tongue * Jaw spasms * Cognitive problems
37
How is motor neurone disease diagnosed?
Clinically * Definite: LMN + UMN signs in 3 region * Probable: LMN + UMN signs in 2 regions * Probable: LMN + UMN signs in 1 region or UMN sign in more than 1 region + electromyography showing acute denervation in more than 2 limbs * Possible: LMN + UMN signs in 1 region * Suspected: LMN or UMN sign in 1 or more regions
38
What investigations can rule out other potential diagnoses of MND?
* MRI spine (cervical cord compression, myelopathy) * Lumbar puncture (inflammatory causes)
39
What is the management for MND?
* Oral riluzole * If reduced FVC: non-invasive ventilation to support breathing at night * Antispasmodics * Antimuscarinics (saliva) * Analgesia * PEG tube * Therapy: SALT, physio * Advanced care plan and end of life care planning
40
What are complications of MND?
* Aspiration pneumonia * Respiratory failure * Nocturnal respiratory insufficiency - nocturnal awakening, unrefreshed sleep, morning headaches
41
What is the prognosis for MND?
Most die within 3 years of onset of symptoms 80% die within 5 years
42
What is an acoustic neuroma?
Benign slow-growing tumour of Schwann cells surrounding the auditory nerve that innervates the inner ear
43
What are risk factors for acoustic neuroma?
* Neurofibromatosis type 2 (suspect in bilateral acoustic neuromas) * Exposure to low-dose radiation in childhood * Noise
44
Where do acoustic neuromas occur?
Cerebellopontine angle (between cerebellum and pons) on the auditory nerve
45
What is the presentation of acoustic neuroma?
Gradual onset of: * Unilateral sensorineural hearing loss * Unilateral tinnitus * Dizziness or imbalance * A sensation of fullness in the ear
46
What is used to diagnose acoustic neuroma?
Brain imaging (CT/MRI)
47
How is acoustic neuroma classified?
Koos classification
48
What is the management of acoustic neuroma?
ENT * Conservative monitoring if no symptoms * Surgery to remove the tumour (suboccipital retrosigmoid approach, translabyrinthine approach, middle fossa approach) * Radiotherapy to reduce growth
49
What are complications of acoustic neuroma?
* Cranial nerve palsies * Hydrocephalus * Cerebellar dysfunction * Intracranial tumour (of cerebellopontine angle)
50
What are complications of acoustic neuroma treatment?
* Vestibulocochlear nerve injury, with permanent hearing loss or dizziness * Facial nerve injury, with facial weakness * Persistent headaches * Bleeding * Infection * CSF leaks
51
Neurofibromatosis
Genetic condition causing benign nerve tumours (neuromas)
52
What is the cause of neurofibromatosis?
* Type 1 (more common): autosomal dominant mutation on chromosome 17, which codes for a protein called neurofibromin 1, a tumour suppressor protein * Type 2: autosomal dominant mutation on chromosome 22, which codes for a protein called merlin, a tumour suppressor protein important in Schwann cells
53
What are the diagnostic criteria for neurofibromatosis type 1?
* Cafe-au-lait spots * Relative with NF1 * Axillary or inguinal freckling * Bony dysplasia - bowing of long bone or sphenoid wing dysplasia * Iris hamartomas * Neurofibromas (2 or more skin-coloured raised nodules or papules with a smooth regular surface, or a single larger irregular complex neurofibroma containing multiple cell types) * Glioma of the optic pathway
54
How is neurofibromatosis managed?
Surgical removal if symptoms develop
55
What are complications of neurofibromatosis type 1?
* Migraines * Epilepsy * Renal artery stenosis (HTN) * Learning disability * ADHD * Scoliosis of the spine * Vision loss (optic nerve glioma) * **Malignant peripheral nerve sheath tumours** * **GI stromal tumour** * Brain tumour * Spinal cord tumour
56
What is chronic fatigue syndrome?
Persistent disabling fatigue lasting more than 6 months
57
What are the criteria for chronic fatigue syndrome?
* Lasting more than 6 months * Affects mental and physical function * Present over 50% of the time * Plus 4 more of: myalgia, polyarthralgia, decreased memory, unrefreshing sleep, fatigue after exertion which lasts over 24 hours, persistent sore throat or tender cervical/axillary lymph nodes
58
What are some predisposing risk factors for chronic fatigue syndrome?
* Perfectionist and introspective personality traits * Childhood trauma * Similar illnesses in first-degree relatives
59
What are some precipitating risk factors for chronic fatigue syndrome?
* Infections (infectious mononucleosis, viral hepatitis) * Traumatic events, especially accidents * Life events that precipitate changed behaviour, e.g. taking time off sick * Incidents where they believe others are responsible
60
Risk factors for perpetuating chronic fatigue syndrome?
* Inactivity with consequent physiological adaption * Avoidant behaviour * Maladaptive illness beliefs * Excessive dietary restrictions * Stimulant drugs, e.g. caffeine * Sleep disturbance * Mood disorders * Somatization disorder * Unresolved anger or guilt * Disputed compensation claim
61
What is the management of chronic fatigue syndrome?
* Explanation of ill health, including diagnosis and causes, and education about management * Stop drugs, e.g. caffeine and analgesics * CBT to challenge unhelpful beliefs and change coping strategies * Graded exercise therapies to reduce inactivity and improve fitness * Antidepressants for mood disorders and sleep disturbance
62
Wernicke's encephalopathy
Acute encephalopathy characterised by a triad of confusion, ataxia and oculomotor dysfunction
63
What causes Wernicke's encephalopathy?
Thiamine (vitamin B1) deficiency
64
What are risk factors for Wernicke's encephalopathy?
* Chronic alcoholism (thiamine poorly absorbed in presence of alcohol) * Poor dietary intake - anorexia nervosa, prolonged fasting, starvation * Systemic malignancy * End-stage renal failure * GI disease and malabsorption
65
What are the clinical manifestations of Wernicke's encephalopathy?
* Confusion * Oculomotor dysfunction - nystagmus, conjugate gaze palsies, lateral rectus palsy * Ataxia * Hypothermia
66
What criteria is used for Wernicke's encephalopathy?
Cane criteria: presence of more than 1 of the following criteria in patients with chronic alcoholism * Dietary deficiency * Oculomotor abnormalities * Cerebellar dysfunction * Altered mental status or mild memory impairment
67
What is the management for Wernicke's encephalopathy?
* Stabilisation and resuscitation * IV thiamine 250mg * Magnesium * Folic acid * Multivitamins * Abstain from alcohol
68
What are complications of Wernicke's encephalopathy?
* Coma and death * Inability to walk * Deficits in learning and memory * Korsakoff's syndrome
69
What is Korsakoff's syndrome?
Late neuropsychiatric manifestation of Wernicke's encephalopathy with chronic amnesic syndrome characterised by defects in both anterograde and retrograde memory
70
What are characteristics of Korsakoff's syndrome?
* Memory impairment * Behavioural changes * Confabulation (stories made up to fill gaps in memory) * Disorientation to time * Poor insight * Global cognitive dysfunction
71
What is the management of Korsakoff's syndrome?
* IV thiamine * Consider magnesium, potassium and/or phosphate replacement Often irreversible requiring care
72
Coma
Unrousable unresponsiveness
73
A coma is a person with a GCS score less than or equal to what?
8
74
What does consciousness depend on?
* Arousal (level of consciousness) - ascending reticular activating system projecting from brainstem to thalamus * Content of consciousness - cerebral cortex
75
Causes of coma
* Drug overdose - alcohol, sedatives * CO poisoning * Traumatic brain injury * Hypoglycaemia * Hepatic encephalopathy * Respiratory failure with CO2 retention * Metabolic acidosis * Infections - encephalitis, meningitis, cerebral malaria * Subarachnoid haemorrhage * Brainstem neoplasm * Wernick-Korsakoff syndrome * Brain tumour
76
Initial examinations for coma
* ABCDE * Vital signs * GCS
77
Parameters for GCS score
* Eye opening - spontaneous (4), to speech (3), to pain (2), no response (1) * Motor response - obeys (6), localises (5), withdraws (4), flexion (3), extension (2), no response (1) * Verbal response - orientated (5), confused conversation (4), inappropriate words (3), incomprehensible sounds (2), no response (1)
78
What system examinations would you complete for someone in a coma?
* General - signs of trauma, stigmata of other illnesses, skin, meningism * Cardiovascular * Respiratory * Abdominal (and rectal?) * Neurological
79
Neurological aspects of examining someone in a coma
* Papilloedema and subhyaloid retinal haemorrhage * Pupils size and reaction to light * Eye movements and position - lack of vestibulo-ocular reflex (deep coma), slow side-to-side eye movements (light or deep coma) * Corneal and gag/cough reflex * Lateralisation of pathology - asymmetry of response to visual threat, drooping or dribbling on one side, unilateral flaccidity or spasticity, asymmetrical response to painful stimuli or tendon reflexes and plantar responses
80
Investigations for coma
* Blood glucose * Drug screen - alcohol, salicylates, urine toxicology (benzodiazepines, narcotics, amfetamines) * U&E * CT brain * Lumbar puncture after risk assessment (!intracranial mass lesion!) * EEG * Calcium * LFT * TFT * Cortisol * ABG * Cerebral malaria and porphyria
81
Locked-in syndrome
Complete paralysis, except vertical eye movements/blinking in ventral pontine infarction (fully aware but unable to communicate)
82
Immediate management of coma
* Consider intubation if GCS <8 * Support circulation if required with IV fluids * Oxygen * Protect cervical spine unless trauma known not to be the cause * Treat cause - glucose for hypoglycaemia, buccal midazolam for seizure, IV cefotaxime for fever and meningism, aciclovir for encephalitis, IV naloxone for opiate intoxication, IV flumazenil for benzodiazpine intoxication, IV thiamine for Wernicke's encephalopathy, artemether/quinine for malaria
83
Long-term management of coma
* Turn to avoid pressure ulcers * Oral hygiene - mouthwashes, suction * Eye care - lid taping, irrigation * NG or IV fluids * Feeding via a fine bore-NG tube or PEG * Catheterisation and rectal evacuation when essential
84
Causes of Horner's syndrome
* Space-occupying lesion * MS * Cervical cord trauma * Stroke * Pancoast tumour * Cervical rib * Brachial plexus injury * Carotid artery dissection * Cavernous sinus pathology * Neck mass * Following thyroid/laryngeal/carotid surgery
85
Horner's syndrome is caused by a lesion along which pathway?
Oculosympathetic
86
Classic triad of Horner's syndrome
Ipsilateral * Miosis (small pupil) * Ptosis (drooping eyelid) * Anhidrosis (lack of sweat)
87
How is Horner's syndrome diagnosed?
Clinically
88
What tests confirm Horner's syndrome?
* Cocaine drops (block reuptake of noradrenaline leading to activation of pupillary dilator and subsequent dilatation) * Apraclonidine (pupil dilates due to alpha-1 receptor denervation supersensitivity)
89
Investigations for cause of Horner's syndrome
* CT angiography (carotid artery dissection) * MRI spine (spinal cord lesions) * MRI head (cavernous sinus) * CT chest (pancoast tumour)
90
How to localise where the lesion is in Horner's syndrome
Hydroxyamphetamine eye drops (stimulate release of noradrenaline, enabling differentiation between first or second order lesions (pupillary dilatation) and third order lesions (no effect))
91
Management of acute painful Horner's syndrome
Urgent assessment for carotid dissection, with urgent neuroimaging and discussion with a hyperacute stroke unit (HASU)
92
What is raised intracranial pressure?
Increase in volume of cranium >15mmHg
93
Causes of raised intracranial pressure
* Head injury * Haemorrhage * Brain tumour * Infection - meningitis, encephalitis, brain abscess * Hydrocephalus * Cerebral oedema * Status epilepticus
94
What is Cushing's reflex?
Nervous system response to raised intracranial pressure, resulting in Cushing's triad 1. Bradycardia 2. Irregular respirations 3. Widened pulse pressure (large difference between systolic and diastolic BP)
95
Pathophysiology of Cushing's reflex
* Initial increase in systemic BP and heart rate (decreased cerebral perfusion causes brain ischaemia which activates the sympathetic NS) * Heart rate decreases (carotid and aortic baroreceptors detect increased BP and activates the parasympathetic NS) * Irregular respirations followed by periods where breathing ceases completely (as pressure in the brain continues to rise, brainstem dysfunctions)
96
Presentation of raised intracranial pressure
* Headache, worse on coughing and learning forward * Vomiting * Altered GCS - drowsiness, irritability, coma * Constriction and then dilatation of pupils * Blurred vision and peripheral visual field loss * Papilloedema
97
Investigations for raised intracranial pressure
* CT head * Lumbar puncture if safe (measure opening pressure) * Bloods - U&E, FBC, LFT, glucose, serum osmolality, clotting, blood culture * Toxicology screen * Chest x-ray (infection)
98
Management of raised intracranial pressure
* ABC * Correct hypotension (MAP > 90mmHg) and treat seizures * **Elevate head of the bed to 35** * If intubated, **hyperventilate** (cerebral vasoconstriction) * **IV** **mannitol** (**osmotic** **diuretic**) * **Corticosteroids** (**methylprednisolone**) if oedema surrounding tumour * Consider **sedation** (**propofol**), anti-epileptics and therapeutic hypothermia * Restrict fluid to < 1.5L / day * Monitor neuro-obs and ICP * Treat cause and exacerbating factors (e.g. hyperglycaemia and hyponatraemia) * **Decompressive craniotomy or burrhole** * **CSF drainage**
99
Complications of raised intracranial pressure
* Herniation (brain tissue shifts to opposite side or down towards brainstem) * Infarction of brain tissue
100
What is multiple sclerosis?
Progressive autoimmune condition causing demyelination of oligodendrocytes of the CNS
101
What is the commonest cause of chronic neurological disability in young adults in the UK?
MS
102
Pathophysiology of MS
Immune system attacks and destroys the myelin sheath of oligodendrocytes of the CNS * Myelin-reactive T lymphocytes enter brain though disruption in BBB and activated by myelin * Activated T lymphocytes: change BBB so it expresses more receptors allowing immune cell infiltration and releases cytokines that recruit more immune cells (B lymphocytes, macrophages and microglia) and directly damage myelin * B lymphocytes make antibodies and macrhages use these antibodies to attack and destroy myelin * MS plaques form (T and B lymphocytes and macrophages)
103
Why do symptoms resolve in early MS?
Regulatory T lymphocytes inhibit type 4 hypersensitivity reaction (release of cytokines by T lymphocytes and recruitment of immune cells) allowing remyelination
104
Why do symptoms change over time in MS?
Lesions vary in location over time
105
Why are symptoms permanent in later stages of MS?
Remyelination is incomplete
106
If someone with MS presents with optic neuritis, where is the lesion?
Optic nerve and often others throughout the CNS, most of which are not causing symptoms
107
What are the different courses of MS?
* Clinically isolated syndrome * Relapse-remitting MS * Secondary progressive MS * Primary progressive MS * Progressive relapsing MS
108
Which is the most common course of MS?
Relapse-remitting Episodes of disease and neurological symptoms followed by recovery, tending to occur in different areas with each episode
109
What is the difference between secondary progressive and primary progressive MS?
Primary progressive worsens from point of diagnosis without relapses and remissions Secondary progressive starts as relapse-remitting, but progressively worsens
110
What is progressive relapsing MS?
One constant attack but bout superimposed, during which disability increases even faster
111
Risk factors for MS
* 20-40 * Female * White * Family history * Viral infections (EBV) * Low vitamin D * Smoking * Obesity * Other autoimmune diseases
112
Uhthoff's phenomenon
In MS, symptoms worsen with heat, e.g. hot bath
113
Presentation of MS
* Optic neuritis * Abnormal eye movements * Focal weakness * Focal sensory loss * Cerebellar dysfunction
114
Symptoms of optic neuritis in MS
* Reduced vision in one eye, developing over hours to days * Central scrotoma (enlarged central blind spot, pale optic disc) * Impaired colour vision (greying, particularly of reds) * Blurring * Relative afferent pupillary defect (constricts more when shining light in contralateral eye)
115
Symptoms of eye movement abnormalities in MS
* Double vision * Oscillopsia (environment moving and unable to create stable image) * Internuclear ophthalmoplegia (INO; slowed adduction) * Conjugate lateral gaze disorder
116
If there is internuclear ophthalmoplegia, where is the lesion?
Medial longitudinal fasciculus
117
If there is a conjugate lateral gaze disorder, where is the lesion?
CN 6
118
Examples of focal weakness in MS
* Incontinence * Horner's syndrome * Facial nerve palsy * Limb paralysis
119
Exames of focal sensory deficit in MS
* Trigeminal neuralgia * Numbness * Paraesthesia * Lhermitte's sign (electric shock sensation down spine and into limbs when flexing neck)
120
What lesion causes Lhermitte's sign?
Cervical spinal cord in dorsal column
121
Examples of symptoms of cerebellar lesions in MS
* Sensory ataxia (positive Romberg's test) * Dysarthria * Intention tremor * Nystagmus * Vertigo * Clumsiness
122
How is MS diagnosed?
McDonald criteria: 2 independent clinical attacks (time) and 2 separate lesions on MRI (space)
123
Investigations to support MS diagnosis
* MRI of brain and spine (plaques) * Lumbar puncture for CSF (oligoclonal bands and increased IgG) * Evoked potentials: somatosensory, visual and motor (slow)
124
What is in the CSF of someone with MS?
Oligoclonal bands and increased IgG
125
Management of MS
* MDT - neurologists, specialist nurses, PT/OT * Steroids: **methylprednisolone** + PPI gastroprotection * Disease-modifying drugs: **interferon beta**, **monoclonal antibodies** (alemtuzumab, natalizumab), latiramer acetate, fingolimod
126
Management of MS flare ups with optic neuritis
High-dose IV methylprednisolone
127
Managements to help with spasticity in MS
* Stretching, PT, splinting * Baclofen * Gabapentin * Botox * Cannabis
128
Management of urinary symptoms in MS
* Antimuscarinics - oxybutynin, solifenacin * Intermittent self-catheterisation or long-term catheters * Botox * Bladder training exercises
129
Management of pain in MS
* Amitriptyline * Gabapentin * Pregabalin
130
Management of pain with trigeminal neuralgia or Lhermitte's (MS)
Carbamazepine
131
Drug to improve walking speed in adults with MS
Fampridine
132
Which course of MS has the best and worst prognosis?
Best = relapse remitting MS Worst = primary progressive MS
133
Difference beween a head injury and traumatic brain injury
Head injury is any trauma to the head TBI needs evidence of damage to the brain as a result of trauma to the head, presenting as a reduced GCS or presence of focal neurological deficit All TBIs are head injuries, but not all head injuries are TBIs
134
Mechanisms of traumatic brain injuries
* Shearing and rotational stresses on decelerating brain * Direct trauma to neurones and axons * Brain oedema and raised intracranial pressure * Brain hypoxia * Brain ischaemia
135
Risk factors for TBI
* Road traffic accidents * Excessive alcohol use
136
GCS for minimal, mild, moderate and severe head injury
* Minimal = GCS 15 with no LOC * Mild = 13-15 with concussion * Moderate = 9-12 * Severe = 3-8
137
Presentation of mild TBI
* Stunned or dazed for seconds to minutes * Post-traumatic amnesia * Headache
138
Presentation of more serious TBIs
* Unconscious (GCS <5 at 24 hours) * Post-traumatic amnesia lasting longer * Unable to lay down any continuous memory * Intermittent restlessness or lethargy during first few weeks * Focal deficits, e.g. hemiparesis or aphasia
139
What is Parkinson's disease?
Neurodegenerative disorder characterised by loss of dopaminergic neurones (and so reduction of dopamine) within the substantia nigra pars compacta of the basal ganglia, leading to disorders of movement
140
Pathophysiology of Parkinson's disease
Loss of dopaminergic neurones in the substantia nigra pars compacta leads to progressive reduction of dopamine in the basal ganglia Lack of dopamine causes loss of communication between the basal ganglia, thalamus and motor cortex and impaired control of voluntary movement Lewy bodies in dopaminergic neurones of SNPC
141
Risk factors for Parkinson's disease
* Increasing age * Male * Family history
142
Prodromal pre-motor symptoms of Parkinson's disease
* Anosmia * REM sleep behaviour disturbance * Insomnia * Daytime sleepiness * Depression and anxiety * Aches, pains and cramping * Autonomic: orthostatic hypotension, constipation, urinary frequency and incontinence, sweating, hypersalivation and drooling
143
4 core symptoms of Parkinsonism
* Bradykinesia * Resting tremor * Rigidity * Postural instability
144
Examples of bradykinesia in Parkinson's disease
* Reduced dexterity - problems doing up buttons and typing * Festinant gait (shuffling, pitched forward), reduced arm swing, freezing at obstacles or doors * Micrographia * Hypomimia * Serpentine stare
145
What type of tremor is seen in Parkinson's?
Resting tremor Pill-rolling Improves on voluntary movement
146
Types of rigidity in Parkinson's
* Cogwheel (resistance to passive movement) * Lead-pipe (stiffness through entire movement)
147
What is the progression of symptoms in Parkinson's usually?
* Prodromal pre-motor symptoms * Motor symptoms starting unilaterally and becoming bilateral
148
How is Parkinson's disease diagnosed?
Clinical diagnois - UK PDS Brain Bank Criteria
149
What imaging can be useful in Parkinson's?
Dopamine transporter (DAT) imaging using single-photon emission CT (SPECT) or PET scan
150
Differentials for Parkinson's disease
* Vascular Parkinsonism * Normal pressure hydrocephalus * Pressure on substantia nigra from tumour/bleed/abscess * Antipsychotics * Cerebellar ataxia * Wilson's disease * Encephalitis and neurosyphilis * Parkinson's plus
151
4 Parkinson's plus diseases
* Multiple system atrophy * Progressive supranuclear palsy * Lewy body dementia * Corticobasal degeneration
152
How is Parkinson's tremor different from benign essential tremor?
* Asymmetrical * Slower * Worse at rest * Improves with intentional movement * Other Parkinson's features * No change with alcohol
153
Drugs for Parkinson's disease
* Synthetic dopamine * Dopamine agonist * Monoamine oxidase B (MAO-B) inhibitor * Catechol-O-methyltransferase (COMT) inhibitor * Amantadine
154
2 synthetic dopamines
* Levodopa * Carbidopa
155
Ways of taking synthetic dopamine
* Dispersible (rescue/morning) * Standard release (daytime) * Slow release (night)
156
What can be taken with levodopa to prevent breakdown before it reaches the brain?
Peripheral decarboxylase inhibitor: benserazide and carbidopa (co-beneldopa and co-careldopa respectively)
157
What is Stalevo?
* Levodopa (synthetic dopamine) * Carbidopa (symthetic dopamine and peripheral decarboxylase inhibitor) * Entacapone (catechol-O-methyltransferase inhibitor)
158
4 non-ergot derived dopamine agonists
* Pramipexole * Ropinirole * Rotigotine * Apomorphine
159
Important side effect of dopamine agonists
Dopamine dysregulation syndrome - impulse control disorder and obsessions
160
How can apomorphine be taken?
* Subcutaneous * Infusion * Rescue treatment (pen)
161
3 ergot-derived dopamine agonists
* Bromocriptine * Pergolide * Cabergoline
162
2 monoamine oxidase B (MAO-B) inhibitors
* Selegiline * Rasagiline
163
Dangers and contraindications with monoamine oxidase B (MAO-B) inhibitor
* Serotonin syndrome with SSRI and tricyclics * Tramadol, pethidine and methadone contraindicated
164
2 catetchol-O-methyltransferase (COMT) inhibitors
* Entacapone * Opicapone
165
Non-pharmacological management of Parkinson's
* MDT - PD nurse specialist, OT, PT, SALT and dietician * Palliative care and end of life planning
166
Treatment of REM sleep behavioural disorder in PD
* Clonazepam * Melatonin
167
Treatment of daytime sleepiness in PD
* Medication review * Modafinil
168
Treatment of nighttime akinesia in PD
* Medication review * Levodopa * Rotigotine patch (dopamine agonist)
169
Treatment of postural hypotension in PD
* Medication review and stop antihypertensives * Midodrine * Fludrocortisone * Ephedrine
170
Treatment of urinary frequency in PD
Anticholinergics
171
Treatment of constipation in PD
Trospium
172
Treatment of hypersalivation in PD
* Hyoscine * Glycopyrrolate * Botox
173
Treatment of dementia in PD
* Acetylcholinesterase inhibitor * Memantine
174
Treatment of psychosis in PD
* Assess for provoking factors (medical illness, infection, metabolic disturbance) * Refer to neurology * Discontinue drugs (anticholinergics, amantadine, MOA inhibitors, dopamine agonist, levodopa) * Quetiapine * Clozapine * Acetycholinesterase inhibitors
175
Complications of PD
* Freezing (sudden stoppage of movement) * Dyskinesia (levodopa) - dystonia, chorea and athetosis * Impulse control disorders (dopamine agonist) * Depression and anxiety * Depression
176
Complications of head injury
* Death * Cognitive impairment * Hemipareis * Epilepsy (depressed skull fracture) * Post-traumatic syndrome: headache, dizziness and malaise * Benign paroxysmal positional vertigo * Chronic subdural haematoma * Hydrocephalus
177
Immediate management of head injury
* Airway * If coma, depressed fracture or suspicion of intracranial haematoma: CT imaging and discussion with neurosurgical unit * Assisted ventilation in severe TBI * Intracranial pressure monitoring * Consider referral to neurosurgery
178
When should you refer a head injury to neurosurgery?
* Significant abnormality on imaging * GCS 8 or less after resuscitation or drop in GCS after admission * Unexplained confusion for over 4 hours * Focal neurological signs or seizures without full recovery * Suspected penetrating injury or evidence of CSF leak
179
Management of depressed skull fracture
Surgical elevation and debridement
180
Management of linear fractures of vault or base
Heal spontaneously
181
Investigation for head injury
CT head +/- cervical spine
182
Indications for a CT head within an hour following head injury
* GCS < 13 on first assessment or < 15 at 2 hours after injury * Signs of basal skull fracture or open or depressed skull fracture * Seizure or > 1 episode of vomiting * Focal neurological deficit
183
Indications for a CT head within 8 hours following head injury
On anti-coagulant or suffered loss of conscioussness/memory loss AND: * Aged over 65 * Previous bleeding disorder * Dangerous mechanism of injury, e.g. cyclist v vehicle or fall from height > 1m * More than 30 minutes of retrograde amnesia of events before the head injury
184
Red flags of head injury
* Impaired consciousness level * Dilated pupils which do not respond to light * Signs of basal skull fracture * Focal neurological deficit or visual disturbances * Seizures or amnesia * Significant headache or N+V
185
How soon upon arrival should a person with head injury be examined? Why?
15 minutes Determine if they have serious brain or spinal injury
186
What should you do if someone is suspected to have a cervical spine injury?
Immobilisation of cerbical spine via semi-rigid collar, blocks and tape
187
Signs of basal skull fracture
* Racoon eyes (bruising around eyes) * Battle's sign (bruising behind ears) * CSF rhinorrhoa or otorrhoea (clear discharge from nose or ear) * Blood bulging from middle (haemotympanum) * Obvious penetrating injury
188
What is a brain abscess?
Focal bacterial infection
189
How common is a brain abscess?
10x rarer than a brain tumour in the UK
190
Causes of brain abscess
* Bacteria: streptococcus anginosus, bacteroides species and staphylococci * HIV * Fungi * Parameningeal infective focus or distant source (lung, heart, abdo)
191
Presentation of brain abscess
* Headache * Focal signs: hemiparesis, aphasia, hemianopia * Epilepsy * Fever
192
Management of brain abscess
* High-dose antibiotics * Surgical resection or decompression
193
Investigations for brain abscess
* Urgent MRI * Neurosurgical aspiration with stereotactic guidance * Bloods (raised ESR, leucocytosis)
194
What would a brain abscess look like on MRI?
Ring-enhancing mass, with considerable surrounding oedema
195
What is shingles caused by?
Viral infection caused by reactivation of varicella zoster virus (herpes zoster) VZV lays dormant in dorsal root ganglia following resolution of chickenpox and if immunosuppressed the virus may reactivate
196
Risk factors for shingles
* Increasing age * Immunosuppressed: systemic disease, cancer, post-transplantation, chemotherapy, high-dose steroids * Transplant recipients * Autoimmune disease * HIV infection * Co-morbid conditions: CKD, COPD, DM
197
Presentation of shingles
* Prodromal paraesthesia and pain (throbbing, burning, stabbing) in affected dermatome * Unilateral erythematous maculopapular rash which doesn't cross the midline * Clusters of vesicles * Pustules, which burst and crust over * Systemic features: headache, fever, malaise, fatigue
198
How is shingles diagnosed?
Clinical diagnosis based on appearance of rash
199
How is shingles with an atypical presentation diagnosed?
Swab from suspected lesion for PCR testing
200
What investigations may you do for someone with shingles?
Immunosuppression testing: * HIV testing * Immunoglobulins * Cancer assessment
201
Management of shingles
* Oral anti-viral therapy (**aciclovir**) within 72 hours of rash onset * Simple analgesia (**paracetamol**, **NSAIDs**) or neuropathic agents (**amitriptyline**, **gabapentin**) * Severe shingles, complications (meningitis/encephalitis) or significant immunosuppression: admission for IV antiviral therapy and monitoring
202
How long do people with shingles remain infectious?
Until all lesions have crusted over (5-7 days)
203
Prevention of shingles
* Varicella zoster immunoglobulin for pregnant women (< 20 weeks) who have had significant exposure to chickenpox or shingles, or for severe neonatal infections * Shingles vaccination for those over 70
204
Complications of shingles
* Scarring * Post-herpetic neuralgia * Secondary bacterial infection * Ramsay Hunt syndrome * Herpes zoster ophthalmicus * Motor neuropathy * CNS involvement: encephalitis, meningitis, myelitis * Disseminated infection
205
Findings on an MRI of brain and spine of someone with MS
* Periventricular lesions * Callosal lesions * Juxtacortical lesions * Dawson's fingers
206
How do steroids and disease-modifying drugs help with MS?
Steroids: reduce length of time of relapse DMD: reduce number of relapses
207
What is muscular dystrophy?
Progressive degeneration of skeletal (and sometimes cardiac) muscle
208
What are the 1st and 2nd most common muscular dystrophies?
1. Duchenne's muscular dystrophy (DMD) 2. Becker's muscular dystrophy
209
Name types of muscular dystrophies
* Duchenne's muscular dystrophy * Becker's muscular dystrophy * Myotonic dystrophy * Facioscapulohumeral muscular dystrophy * Oculopharyngeal muscular dystrophy * Limb-girdle muscular dystrophy * Emery-Dreifuss muscular dystrophy
210
What is the pathophysiology of muscular dystrophy?
X-linked recessive Mutation in dystrophin gene Deficiency of dystrophy causes sarcolemma (cell membrane) to wilt and become unstable CK escapes and calcium enters, leading to cell death
211
Why is Duchenne's muscular dystrophy worse than Becker's muscular dystrophy?
In DMD there is an absence of dystrophin, whereas in Becker's there are low levels of dystrophin
212
In muscular dystrophy, what happens to the muscle short-term vs long-term?
Short-term: muscle generation resulting in muscle fibres of different sizes Long-term: muscle atrophy and infiltration by fat and fibrotic tissue
213
Risk factors for muscular dystrophy
* Designated male at birth * Family history
214
How does the age of presentation vary for Duchenne's and Becker's muscular dystrophy?
DMD: 3-5 Becker's: 8-12
215
Clinical manifestations of muscular dystrophy
* Start walking later * Waddling gait * Difficulty running * Difficulty rising to their feet * Pelvic muscle weakness * Gower's sign (downward dog, hands climb up their legs to stand) * Calf pseudohypertrophy (fat and fibrotic tissue)
216
When would you expect someone with muscular dystrophy to require a wheelchair?
DMD: wheelchair bound in teenage years Becker's: some require wheelchairs in late 20s-30s, others able to walk without assistance into late adulthood
217
How is muscular dystrophy diagnosed?
* Creatinine kinase grossly elevated * Muscle biopsy shows variation in muscle fibre size, necrosis, regeneration and replacement by fat; absence of dystrophin on immunochemical staining
218
Management of muscular dystrophy
* Medications: oral corticosteroids, creatine supplementation * Physical: wheelchair, brace, respiratory support, surgery for contractures/scoliosis * MDT: PT, OT
219
How to prevent muscular dystrophy
Genetic counselling * Prenatal diagnosis * Carrier detection (females with affected brother have 50% chance of carrying DMD gene)
220
Complications of muscular dystrophy
* Respiratory failure * MSK problems: joint contractures, chest deformities, spinal deformities (scoliosis) * Dilated cardiomyopathy and arrhythmias
221
Life expectancy of someone with muscular dystrophy
DMD: 25-35 Becker's: better
222
Causes of proximal myopathy
* Infection: HIV, EBV, hepatitis, bacterial pyomyositis, Lyme disease, toxoplasmosis, candida * Autoimmune: SLE, vasculitis, rheumatoid arthritis, systemic sclerosis, polymyositis/dermatomyositis * Metabolic: hypo/hyperkalaemia, hypermagnesemia * Iatrogenic: statins, **corticosteroids**, amiodarone, colchicine * Neoplastc: carcinomatous neuromyopathy * Congenital myopathies * Degenerative: muscular dystrophies * Endocrine: hypo/hyperthyroidism, hyperparathyroidism, Addison's disease, Cushing's syndrome * Other: alcohol, rhabdomyolysis, sarcoidosis, mitochondrial myopathy
223
Clues that proximal myopathy has a metabolic cause
* Difficulties with exercise * Cramps and myalgia * Episodes of weakness come and go
224
Clues that proximal myopathy is caused by mitochondrial myopathy
Other symptoms with heart, brain, GI tract, etc.
225
Risk factors for proximal myopathy
* Family history of myopathy * Designated male at birth (X-linked) * Autoimmune, metabolic or endocrine disorder * Exposure to certain medications or toxins
226
# Hair, chair and stairs Symptoms of proximal myopathy
* Difficulty performing ADLs (bathing, dressing, combing hair) * Trouble getting out of chair, climbing stairs or performing tasks that require reaching over your head * Falls * Muscle cramps or spasms * Muscle fatigue with activity * Shortness of breath with exertion
227
Signs of proximal myopathy
* Muscle wasting * Reduced power * Trouble standing from sitting and raising arms above head * In fetus: decreased movements * In neonates: floppy * In toddlers: motor delays (walking, hopping, running, climbing stairs, grasping a spoon/pencil)
228
Causes of proximal muscle weakness that isn't proximal myopathy
* Neurological: MND, GBS, myasthenia gravis, cerebral palsy, spinal muscular atrophy * Not true weakness: polymyalgia rheumatica, fibromyalgia, osteoarthritis, rotator cuff problems, fatigue/malaise/depression
229
Investigations for proximal myopathy
* Bloods: U&E, Ca, P, Mg; CK; TFT; rheumatological screen (ESR, ANA, RF) * Nerve conduction studies and EMG * MRI muscle * Muscle biopsy
230
What is myasthenia gravis?
Autoimmune disorder against acetylcholine receptors of the postsynaptic membrane at the neuromuscular junction
231
What ages most commonly get myasthenia gravis?
Women during 20s-30s Men during 50s-60s
232
What causes myasthenia gravis?
Autoimmune disease Caused most commonly by acetylcholine receptor antibodies, which block the AChR and prevent stimulation by ACh, and activate the complement system leading to cell damage Other antibodies (MuSK and LRP4 antibodies) destroy proteins involved in creation and organisation of AChR
233
Why do symptoms depend on muscle activity in myasthenia gravis?
With increased muscle activity, more receptors are used and become blocker, so there is less effective stimulation of muscle With rest, receptors are cleared and symptoms improve
234
Risk factors for myasthenia gravis
* Female * Family history * Other autoimmune diseases * Thymoma
235
Clinical manifestations of myasthenia gravis
Weakness that worsens with activity and improves with rest * Proximal muscles: difficulty climbing stairs, standing from sitting and raising hands above head * Ocular muscles: diplopia and ptosis * Neck and facial muscles: myasthenia snarl, fatigue in jaw when chewing, head drop, dysarthria and dysphagia * Respiratory: shortness of breath
236
How can you exacerbate signs in myasthenia gravis?
* Prolonged blinking - ptosis * Prolonged upward gazing - diplopia * Repeated abduction of one arm 20 times - unilateral weakness when comparing both sides
237
What scar should you check for in someone with suspected myasthenia gravis?
Thymectomy scar
238
What are the investigations for myasthenia gravis?
* Antibodies: AChR, MuSK and LPR4 antibodies * CT chest (thymoma) * Electromyography with repetitive nerve stimulation * Tensilon test (edrophonium improves muscle weakness) * Ice pack test (onto closed eyelid for minute improves symptoms)
239
Management of myasthenia gravis
* Acetylcholinesterase inhibitors: pyridostigmine and neostigmine * Immunosuppressants: prednisolone, azathioprine and methotrexate * Rituximab (monoclonal antibody) * Thymectomy
240
Management of myasthenic crisis
* Non-invasive ventilation * IV immunoglobulins or plasmapheresis
241
What should be monitored in someone with myasthenia gravis?
* Forced vital capacity * Bulbar function
242
Complications of myasthenia gravis
* Myasthenic crisis * Respiratory failure * Aspiration pneumonia * Thymus dysfunction
243
What is myasthenic syndrome?
Disorder of the neuromuscular junction
244
Causes of myasthenic syndrome
* Myasthenia gravis * Lambert-Eaton myasthenic syndrome * Congenital myasthenia
245
Risk factors for myasthenic syndrome
* Family history * Other autoimmune diseases * Thymoma (myasthenia gravis) * Cancer, particularly small cell lung cancer (Lambert-Eaton)
246
Symptoms of myasthenic syndrome
Weakness * Proximal musce weakness: *'hair, chair and stairs'* * Ocular muscle weakness: diplopia and ptosis * Facial muscle weakness: dysarthria and dysphagia
247
Signs of Lambert-Eaton syndrome
* Reduced power * Reduced tendon reflexes (cardinal sign), with improvement following a period of strong muscle contraction
248
Differential diagnoses of myasthenic syndrome
* Botulism * Myopathy * Chronic inflammatory demyelinating neuropathy * GBS
249
Investigations for myasthenic syndrome
* Antibodies: AChR, MuSK, LRP4 antibodies (myasthenia gravis) and voltage-gated Ca channel antibodies (Lambert-Eaton) * Nerve conduction studies and electromyography with repetitive nerve stimulation * CXR (small cell lung cancer in Lambert-Eaton) and CT chest (thymoma in myasthenia gravis)
250
Management of myasthenic syndrome
* Pyridostigmine * Immunosuppressants: prednisolone and azathioprine * Ventilation and IV immunoglobulins/plasmapheresis
251
Complications of myasthenic syndrome
* Respiratory failure * Aspiration pneumonia
252
What is radiculopathy?
Injury or damage to the spinal nerve or its root where it leaves the spine
253
Causes of radiculopathy
V - vasculitis I - epidural abscess, osteomyelitis (TB > Pott's disease) T - injury, compression fractures A - acute/chronic inflammatory demyelinating polyradiculopathy N - intradural tumour, metastasis, myeloma, lymphoma **D** - spinal canal stenosis, spondylosis, **osteoarthritis** (**osteophytes**) E - DM O - slipped disc, spondylolisthesis, scoliosis
254
Risk factors for radiculopathy
* Inactive * Overweight * Poor posture * Lifting heavy objects * Operating vibrating equipment * Trauma
255
Clinical manifestations of radiculopathy
* Sharp, shooting, radiating pain * Numbness or tingling * Weakness * Reduced reflexes
256
L1 radiculopathy
S - inguinal region P - hip flexion (uncommon)
257
L2-4
P - back into anterior thigh S - anterior thigh and medial lower leg (patella) P - hip flexion and adduction, knee extension and ankle dorsiflexion R - knee
258
L5
P - back into lateral aspect of leg to foot (big toe) S - lateral aspect of lower leg and dorsum of foot P - ankle dorsiflexion, big toe extension and ankle inversion/eversion
259
S1
P - back into posterior aspect of leg into foot S - posterior leg and lateral foot (5th toe) P - ankle plantarflexion, hip extension and knee flexion R - ankle
260
S2
P - ankle plantarflexion, pelvic floor
261
S5
S - perianal region
262
C5
P - neck, shoulder and scapula S - lateral aspect of upper arm P - shoulder abduction and elbow flexion R - biceps and brachioradialis
263
C4
S - clavicle P - shoulder elevation
264
C6
P - neck, shoulder, scapula, lateral arm, forearm and hand S - lateral forearm, thumb and finger (thumb) P - elbow flexion and supination/pronation R - biceps and brachioradialis
265
C7
P - neck, shoulder, hand and middle finger S - palm, middle and index fingers (middle finger) P - elbow, finger and wrist extension R - triceps
266
C8
P - neck, shoulder, medial forearm, hand and 4th/5th fingers S - medial forearm, hand and 4th/5th ginger P - elbow extension and finger flexion
267
T1
P - finger adduction
268
Thoracic
P - to chest and abdomen
269
T4
S - nipple
270
T10
S - umbilicus
271
T12
Inguinal region
272
Special test for L2-4 radiculopathy
Reverse straight leg raise (extend hip while in prone)
273
Special test for L5/S1 radiculopathy
Straight leg raise (raise leg with knee extended)
274
Special tests for cervical radiculopathy
* Lhermitte phenomenon (neck flexion = shock-like paraesthesia down spine) * Shoulder abduction relief test (arm of affected side raised and hand placed on head = relief) * Spurling manoeuvre (downward pressure on head when extended and rotated to affected side = worse)
275
Investigations for radiculopathy
* MRI/CT * X-ray * EMG and NCS * Myelography * Blood tests: ESR/CRP, FBC, glucose, vitamins
276
Conservative management of radiculopathy
* Rest * Activity modification * Physio * Weight loss
277
Medical management of radiculopathy
* NSAIDs (ibuprofen, aspirin, naproxen) * Opioids * Amitriptyline, pregabalin, gabapentin * Muscle relaxants: benzodiazepine (diazepam), baclofen * Corticosteroids PO or injections into spine
278
Surgeries for radiculopathy
* Spinal decompression surgery: discectomy, laminectomy * Spinal fusion * Foraminotomy * Disc replacement surgery
279
What is chorea?
Involuntary, irregular or unpredictable muscle movements, affecting the arms, legs and facial muscles
280
Causes of chorea
V - stroke I - rheumatic fever/post-strep infection, AIDS A - SLE, MS, antiphospholipid syndrome M - hypoglycaemia, kernicterus (bilirubin) I - levodopa, antipsychotics N - brain tumour near basal ganglia C - benign hereditary chorea D - Huntington's, Parkinson's, Wilson's E - thyrotoxicosis O - cocaine, amphetamines, carbon monoxide, pregnancy
281
What is Sydenham chorea?
Chorea caused by rheumatic fever or post-streptococcal infection
282
What is tardive dyskinesia?
Chorea caused by antipsychotics
283
What is chorea gravidarum?
Chorea caused by pregnancy
284
Risk factors for chorea
* Family history of Huntington's * Rheumatic fever, infection or head injury in childhood * Autoimmune condition * Over 40
285
Symptoms of chorea
* Involuntary arm/leg/face movements (fidget, twist, jerk) * Milkmaid's grip * Move tonue in and out of mouth * Abnormal gait
286
Medication for tardive dyskinesia
Tetrabenazine
287
Treatment for chorea caused by levodopa
Change to amantadine
288
Treatment of rheumatic fever
Corticosteroids and antibiotics
289
Management of chorea caused by dopamine
* Adjust or reduce dose * Spread dose out * Use extended-release formulations
290
Management of brain tumour
Surgery to remove tumour
291
Complications of chorea
* Difficulty swallowing foods and drinks * Behavioural or emotional challenges * Physical injury * Social stigmatisation
292
Risk factors for Huntington's
* Family history * Middle age
293
Cause of Huntington's
Autosomal dominant trinucleotide (CAG > glutamine) repeats over 35 times, resulting in mutation in huntingtin (HTT) gene on chromosome 4 Mutated protein aggregates in putamen and caudate of basal ganglia causing neuronal cell death and loss of brain tissue, which results in decreased GABA and ACh, and increased dopamine
294
What is the penetrance of Huntington's?
Reduced penetrance with 36-39 repeats 100% penetrance with over 40 repeats
295
Why do successive generations affected by Huntington's have earlier onset and worse disease?
Anticipation: expansion of trinucleotide repeat during meiosis
296
Why is anticipation of Huntington's disease worse when inherited from father?
Expansion of trinucleotide repeat during meiosis worse in spermatogenesis
297
Clinical manifestations of Huntington's
Insidious, progressive worsening of symptoms from around 40 * Psychiatric - dementia, depression, personality changes * Movement - chorea, athetosis, abnormal eye movements, dysarthria, dysphagia
298
Investigations for Huntington's
* Genetic test * CT/MRI brain
299
Medications for Huntington's
* Antipsychotics: risperidone, sulpiride, olanzapine * Tetrabenazine * Amantadine * Benzodiazepine (aggression) * Sertraline (depression)
300
MDT approach to Huntington's
* OT * PT * Psychologist * SALT
301
Counselling for those with Huntington's
* Genetic counselling regarding relatives, pregnancy and children * Advanced directives to document their wishes as the disease progresses * End of life care planning
302
Prognosis of Huntington's
Life expectancy 10-20 years after symptom onset Death usually from aspiration pneumonia or suicide
303
When someone presents with radiculopathy, what should you think about red flags for?
* Cauda equina syndrome * Infection * Fracture * Malignancy * Metastatic disease
304
With someone presenting with radiculopathy, what are red flags for cauda equina syndrome?
* Faecal incontinence * Urinary retention (painless, with secondary overflow incontinece) * Saddle anaesthesia
305
With someone presenting with radiculopathy, what are red flags for infection?
* Immunosuppression * IV drug abuse * Unexplained fever * Chronic steroid use
306
With someone presenting with radiculopathy, what are red flags for fracture?
* Chronic steroid use * Significant trauma * Osteoporosis * Metabolic bone disease
307
Symptoms of spinal claudication
Bilateral radiating leg pain or paraesthesia that comes on with walking and is such that they have to stop and rest for a number of minutes before walking again, symptoms relieved by leaning forward
308
What is myelopathy?
Spinal cord compression causing nerves not to work properly
309
Causes of myelopathy
V - haematoma I - epidural abscess, osteomyelitis, discitis T - spinal injury A - rheumatoid arthritis M - B12 deficiency I - radiation N - primary bone tumours, metastases, structural weakness secondary to cancer C - congenital stenosis D - spondylosis > osteophytes, spinal stenosis, Parkinson's, amyotrophic lateral sclerosis O - prolapsed disc, spinal cyst
310
What is subacute combined degeneration of the spinal cord?
Demyelination of lateral and posterior columns of the spinal cord, commonly due to vitamin B12 deficiency
311
Most common cancers to metastasise to the spine
* Kidney * Prostate * Breast * Lung * Thyroid * Multiple myeloma
312
Risk factors for myelopathy
* Cancer * Heavy lifting * Overweight * Smoking * Inactive * Scoliosis
313
Symptoms of myelopathy
* Neck, arm, leg or back pain * Tingling, numbness or weakness * Loss of fine finger movements * Difficulty walking * Loss of urinary or bowel control * Issues with balance and coordination
314
Investigations for myelopathy
* X-ray * MRI * Myelogram * Electromyogram or evoked potentials * Blood tests: FBC, ESR, B12, U&E, syphilis, LFT, PSA * CXR (lung cancer)
315
Signs of myelopathy
UMN signs * Spastic gait * Hypertonia * Hyperreflexia * Babinski sign * Clonus * Cross-adductors * Hoffman's sign * Deltopectoral reflex * Sensory loss
316
Management of myelopathy
Spinal decompression surgery (discectomy, laminectomy, laminoplasty, decompression and spinal fusion)
317
Conservative management of myelopathy
* NSAIDs * Corticosteroids PO or injection * Brace * Physical therapy * Hot and cold treatments * Acupuncture and chiropractic care
318
Treatment of spinal malignancy causing cord compression
IV dexamethasone and radiotherapy
319
Treatment of epidural abscess causing cord compression
Antibiotics
320
Complications of myelopathy
Paralysis
321
What causes spinal muscular atrophy?
Mutation in SMA1 gene
322
Clinical manifestation of spinal muscular atrophy
Proximal, symmetrical muscle weakness Legs more than arms Can affect bulbar function and breathing
323
Presentation of type 1 spinal muscular atrophy
First 6 months * Weak and floppy arms and legs * Problems moving, eating, breathing and swallowing * Unable to raise head
324
Presentation of type 2 spinal muscular atrophy
7-18 months * Can sit, can't stand * Weak arms or legs * Shaking in fingers and hands * Joint problems (scoliosis) * Weak breathing muscles and trouble coughing
325
Presentation of type 3 spinal muscular atrophy
After 18 months * Able to stand and walk, but may find walking and getting up from sitting difficult and may lose ability to walk * Balance problems * Difficulty running or climbing steps * Shaking in their fingers
326
Presentation of type 4 spinal muscular atrophy
Early adulthood * Weakness in hands and feet * Difficulty walking * Shaking and twitching muscles
327
What is somatic symptom disorder?
Symptoms suggestive of a physical disorder, but in the absence of physiological illness, e.g. pins and needles, headaches, visual disturbances
328
Clues that it might be a somatic symptom disorder
* Prolonged history of multiple physical symptoms, often occurring in different systems, that cannot be explained by any detectable physical disorder * Belief that they have a serious illness * Repeated medical consultations and requesting investigations * Do not accept reassurnace from doctors
329
Causes of cranial neuropathy
V - aneurysm, ischaemia, stroke, vasculitis I - Lyme disease, neurosyphilis, HIV T A - GBS, SLE, MS M - Wernicke's encephalopathy I - surgery N - brain tumour E - DM
330
Clinical manifestation of CN 1 neuropathy
Reduced smell and taste
331
Where would the cancer and trauma be to cause CN 1 neuropathy?
Tumour: frontal lobe, ethmoid, meningioma of olfactory groove Trauma: basal skull fracture, frontal fracture
332
Causes of monocular sight impairment
* MS * Giant cell arteritis
333
Causes of bilateral hemianopia
* Internal carotid artery aneurysm * Pituitary adenoma * Craniopharyngioma
334
Causes of homonymous hemianopia
* Stroke * Brain abscess * Brain tumour
335
Causes of optic neuritis
* Demyelination * Sinusitis * Syphilis * Collagen vascular disorders
336
Clinical manifestation of CN 2 neuropathy
* Visual field defects * Pupillary abnormalities * Pain on eye movement
337
Causes of third nerve palsy
V - posterior communicating/carotid artery aneurysm, cavernous sinus thrombosis I - syphilis, orbital cellulitis, invasive fungal sinusitis, meningitis A - giant cell arteritis N - meningeal carcinomatosis
338
Clinical manifestation of third nerve palsy
* Fixed dilated pupil, which doesn't accommodate * 'Down and out' eye appearance * Diplopia * Ptosis * Internuclear ophthalmoplegia * Trouble moving eye * Pain * Loss of eye reflex
339
How to prevent diplopia with third nerve palsy
Short-term: eye patch Long-term: prisms and strabismus surgery
340
Clinical manifestation of fourth nerve palsy
* Diplopia * Eye(s) turn abnormally * Tilt head away from affected side
341
Causes of fifth nerve palsy
* Herpes zoster virus * Nasophrayngeal carcinoma * Acoustic neuroma
342
Clinical manifestations
* Numbness or dysaesthesia * Weakness of jaw clenching and side-to-side movement * Fasciculations of temporalis and masseter muscles
343
Causes of sixth nerve palsy
* Pontine cerebrovascular accident * Meningitis * Cavernous sinus thrombosis * Pituitary adenoma
344
Clinical manifestation of sixth nerve palsy
* Diplopia, worse on horizontal gaze in direction of lesion * Failed eye abduction, eye deviates medially * Strabismus * Blurred vision * Dizziness * Eye pain
345
Causes of eighth nerve palsy
* Loud noise * Paget's disease of bone * Meniere's disease * Herpes zoster virus * Neurofibroma, acoustic neuroma * Aminoglycosides, furosemide, aspirin * Lead
346
Clinical manifestation of eighth nerve palsy
* Unilateral sensorineural deafness * Tinnitus * Nystagmus
347
Clinical manifestation of ninth nerve palsy
* Hoarseness * Nasal speech
348
Clinical manifestation of tenth nerve palsy
* Hoarseness * Nasal speech * Quiet * Bovine cough * Palate moves asymmetrically when they say 'ahhh'
349
Clinical manifestation of eleventh nerve palsy
Weakness and wasting of sternocleidomastoid and trapezius muscles
350
Clinical manifestation of twelfth nerve palsy
Ipsilateral * Wasting of tongue * Fasciculation of tongue * Deviation on protrusion of tongue
351
Investigations for cranial neuropathy
* Bloods: FBC, CRP, glucose * EMG and NCS * CT/MRI brain * Biopses of skin and nerves * LP * Angiography
352
Management of cranial neuropathy
* Treat underlying cause (abx, surgery, anti-HTN, anti-DM) * Eat nutritious food * Don't smoke and limit alcohol
353
What is Bell's palsy?
Unilateral facial nerve palsy
354
Causes of Bell's palsy
* Infection: Lyme disease, polio, otitis media, herpes zoster virus, HIV * Skull fracture * Cerebellopontine angle or parotid tumour
355
Clinical manifestation of Bell's palsy
* Unilateral facial weakness: loss of nasolabial fold, drooping eyebrow/corner of mouth, asymmetrical smile, speech/chewing difficulty * Ear pain * Incomplete eye closure * Drooling * Tingling in cheek and mouth * Hyperacusis
356
Difference between stroke and Bell's palsy
Bell's palsy (LMN) has paralysis of forehead, stroke (UMN) does not
357
What grades facial weakness?
House-Brackmann system
358
How is Bell's palsy diagnosed?
Clinically, based on unilateral facial weakness of rapid onset without forehead sparing
359
Features that point to a different diagnosis than Bell's palsy
* Slow onset and progressive * Overt pain * Systemic illness * Vestibular abnormalities * Other hearing abnormalities * Forehead sparing (stroke) * Mass * Recurrent palsy * Bilateral involvement (sarcoidosis, GBS, Lyme disease, HIV)
360
Management of Bell's palsy
* Prednisolone 50mg for 10 days, within 72 hours of onset * Straw for liquids, soft diet * Eye care: eye drops, tape eye when sleeping, sunglasses outdoors
361
Prognosis of Bell's palsy
Good, although may take several months to recover
362
What is bulbar palsy?
Neuropathy of cranial nerves 9, 10 and 12
363
Causes of bulbar palsy
Brainstem: infarction, syringobulbia, MND, tumour, central pontine myelinolysis Skull base: nasopharyngeal carcinoma, neurofibroma, jugular venous thrombosis, trauma Neck and nasopharynx: nasopharyngeal carcinoma, carotid artery dissection, trauma, lymph node biopsy in posterior triangle
364
Clinical manifestation of bulbar palsy
* Hoarse, nasal, quiet speech * Bovine cough * Palate moves asymmetrically when they say 'ahhh' * Ipsilateral wasting of tongues, with fasciculations and deviation on protrusion of tongue
365
What is cerebellar syndrome?
Cerebellar disease affecting co-ordination, balance and speech
366
Causes of cerebellar syndrome
V - stroke, AV malformation I - brain abscess, HIV, creutzfeldt-jakob disease T A - gluten ataxia, MS M - chronic alcohol use, Wernicke's encephalopathy, vitamin deficiencies (B12, E, folate) I - antiepileptics, antipsychotics N - medulloblastoma, paraneoplastic cerebellar degeneration C - chiari malformation, cerebral palsy, spinocerebellar ataxias, Friedreich's ataxia, ataxia telangiectasia D - multiple system atrophy, superficial siderosis E - hypothyroidism O - lead/carbon monoxide poisoning
367
Symptoms of cerebellar syndrome
* Feels drunk * Difficulty walking * Dizzy * Clumsy * Diplopia * Dysphagia * Problems with dexterity * Vomiting
368
# DANISH Signs of cerebellar syndrome
* Dysdiadochokinesia or dysmetria * Ataxia * Nystagmus * Intention tremor * Speech (slurred or scanning) * Hypotonia
369
Investigations for cerebellar syndrome
* Routine bloods: FBC, U&E, LFT, TFT, ESR/CRP, cholesterol, glucose * Other bloods: vitamin B12/E/folate, gluten serology, copper/caeruloplasmin, autoimmune screen, anti-GAD, paraneoplastic antibodies * MRI/CT brain * EEG * EMG * LP
370
Complications of cerebellar syndrome
* Respiratory arrest * Hydrocephalus * Tonic seizures
371
What is meningitis?
Inflammation of the leptomeninges (arachnoid and pia mater)
372
Causes of meningism
V - subarachnoid haemorrhage I - bacterial, viral, fungal and parasitic T - head injury A - SLE I - intrathecal drugs N - paraneoplastic, malignancy O - raised intracranial pressure
373
Viral causes of meningitis
* Enteroviruses: coxsackievirus, echovirus * Herpes simplex * Human immunodeficiency * Varicella zoster * Epstein Barr * Mumps
374
Fungal causes of meningitis
* Cryptococcus neoformans * Candida albicans
375
Parasitic causes of meningitis
P. fallicidum
376
How does infection spread to the brain in meningitis?
* Direct - nose, anatomical defect (spina bifida, skull fracture), shunt, head operation, trauma, overlying skin * Bloodstream
377
What is a risk factor for recurrent bacterial infection, often by pneumococcus?
Skull fracture (access to subarachnoid space)
378
Causes of chronic meningitis
* TB * Cryptococcus * Syphilis * Sarcoidosis * Behcet's
379
Risk factors for meningitis
* Immunocompromised * Head operation * Trauma * Students (crowded environment) * Travel * Contact with someone with meningitis/TB * HIV * Spina bifida * Skull fracture
380
What increases your risk of infection with Leptospira?
Working in drains, canals or polluted water, or recreational swimming
381
Meningism triad
1. Headache 2. Neck stiffness 3. Fever
382
Symptoms of meningitis
* Headache * Neck stiffness * Fever * Photophobia * Vomiting * Irritable
383
Signs of meningitis
* Kernig's sign * Brudzinski's sign * Reduced ROM in neck flexion * Temperature * In acute bacterial: petechial/purpuric non-blanching rash, seizures
384
Signs of complications in meningitis
* Progressive drowsiness * Lateralising signs * Cranial nerve lesions
385
Investigations in meningitis
* CT head * Lumbar puncture (contraindications: abnormal clotting (coagulation screen), petechial rash, raised intracranial pressure): microscopy, gram stain, Ziehl-Neelson stain, Indian ink, chocolate agar, PCR * Nose and throat swabs: chocolate agar, PCR * Stool PCR * Blood culture and PCR * Syphilis serology
386
Gram +ve diplococci
S. pneumoniae
387
Gram -ve diplococci
N. meningitidis
388
Acid-fast bacilli
Tuberculosis
389
Indian ink
Fungus
390
Chocolate agar
H. influenzae N. meningitidis
391
Appearance of CSF (normal, bacterial, viral and fungal/TB)
N - clear B - cloudy V - clear F - clear (cloudy fibrin web)
392
Opening pressure of CSF (normal, bacterial, viral and fungal/TB)
N - 90-180 B - elevated V - normal F - normal-elevated
393
WBCs of CSF (normal, bacterial, viral and fungal/TB)
N - <8 B - >1000 V - <300 F - <500
394
Main cell type of CSF (normal, bacterial, viral and fungal/TB)
B - neutrophils V - lymphocytes F - lymphocytes
395
Protein of CSF (normal, bacterial, viral and fungal/TB)
N - 15-45 B - >200 V - <200 F - >200
396
Glucose of CSF (normal, bacterial, viral and fungal/TB)
N - 50-80 B - <40 V - normal F - low-normal
397
What extra investigations would you do for chronic meningitis?
* MRI brain * Repeated CSF investigations
398
Management of viral meningitis
Acyclovir if HSV/VZV Conservative management for others
399
Management of tuberculous meningitis
* Antituberculosis drugs for at least 9 months: rifampicin, isoniazid, pyrazinamide * Adjuvant corticosteroids for 3 weeks, e.g. prednisolone
400
Management of malignant meningitis
Intrathecal cytotoxic agents
401
Complications of meningitis
* Neuro - brain abscess, cerebral oedema, hydrocephalus, brain herniation, seizures, hearing loss, memory loss, cerebral palsy * Sepsis
402
Prognosis of viral vs tuberculous vs bacterial meningitis
Viral generally self-limiting Tuberculous 60% mortality Bacterial 5% mortality when treated
403
What is acute bacterial meningitis?
Serious bacterial infection of the meninges
404
What organisms commonly cause bacterial meningitis in neonates?
* Group B streptococcus (gram +ve cocci) * E. coli (gram -ve bacillus) * Listeria monocytogenes (gram +ve bacillus) * S. agalactiae
405
What organisms commonly cause bacterial meningitis in children?
* N. meningitidis (gram -ve diplococci) * S. pneumoniae (gram +ve diplococci) * H. influenzae B (gram -ve bacillus)
406
What organisms commonly cause bacterial meningitis in adults?
* S. pneumoniae (gram +ve diplococci) * N. meningitidis (gram -ve diplococci)
407
What organisms commonly cause bacterial meningitis in immunocompromised?
* Listeria monocytogenes (gram +ve bacillus) * M. tuberculosis (acid -fast bacillus)
408
Other bacterial causes of meningitis
* S. aureus * Treponema pallidum (syphilis) * Leptospira
409
Why do cranial nerve palsies and hydrocephalus occur in acute bacterial meningitis?
Pia-arachnoid mater congested with poly,orphs and a layer of pus forms, which may organise to form adhesions
410
Clinical manifestation of acute bacterial meningitis
Develops within minutes to hours * Headache * Neck stiffness * Fever * Photophobia * Vomiting * Intense malaise * Rigors * Irritable * Prefers to lie still * Positive Kernig's sign * Positive Brudzinski sign * Seizures
411
Signs of meningitis in babies
* Hypotonia * Poor feeding * Lethargy * Hypothermia * Bulging fontanelle
412
Clinical manifestation of meningococcal septicaemia
* Petechial/purpuric non-blanching rash * Septic shock * Peripheral vascular infarcts
413
Management of acute bacterial meningitis
* IV antibiotics for at least 5 days * High-dose IV corticosteroid (dexamethasone) with or before dirst dose of antibiotics for 4 days
414
Empirical antibiotic < 3 months, and alternative
Cefotaxime + amoxicillin Benzylpenicillin + chloramphenicol
415
Empirical antibiotics 3 months - 50 years, and alternative
Ceftriaxone Benzylpenicillin + chloramphenicol
416
Empirical antibiotics > 50 or immunocompromised, and alternative
Cefotaxime + ampicillin Cefotaxime + co-trimoxazole
417
Antibiotics for meningococcal infection
Cefotaxime initially Benzylpenicillin if confirmed sensitivity
418
Antibiotics for pneumococcal infection, and alternative
Cefotaxime Penicillin
419
Antibiotics for haemophilus infection, and alternative
Cefotaxime Chloramphenicol
420
Antibiotics for Listeria monocytogenes infection
Amoxicillin + gentamicin
421
When should you add vancomycin in antibiotic treatment of meningitis?
Repeated abx use or foreign travel
422
What abx therapy should you offer if penicillin and cephalosporin allergy?
Chloramphenicol
423
Prophylaxis of bacterial meningitis
* Notify public health * Antibiotic prophylaxis * Vaccines: meningococcal, Hib, pneumococcal
424
What is the abx prophylaxis for meningitis?
Ciprofloxacin, rifampicin
425
Who gets abx prophylaxis for meningitis?
Prolonged close contact during 7 days before onset and exposure to respiratory droplets
426
Who gets meningococcal vaccine?
* MenC and MenB in infancy and during outbreaks * Combined A and C meningococcal vaccine sometimes before travel to endemic regions (Africa, Asia) * MenC for close contacts if partially/un-immunised
427
What is a complication of meningitis caused by N. meningitidis?
Waterhouse-Friedrichsen syndrome (adrenal gland failure due to bleeding into adrenal gland)
428
Permanent effects of acute bacterial meningitis
* Scars * Amputation * Hearing loss * Seizures * Brain damage
429
What is narcolepsy?
Long-term brain condition that can prevent a person from choosing when to wake or sleep
430
What causes narcolepsy?
Lack of brain chemical hypocretin (orexin), which regulates wakefulness Thought to be autoimmune
431
Which proteins are associated with narcolepsy?
HLA-DR2 and HLA-DQBI0602
432
Possible triggers of narcolepsy
* Hormonal changes: puberty, menopause * Major psychological stress * Infection or vaccination
433
Clinical manifestation of narcolepsy
* Excessive daytime sleepiness * Sleep attacks * Cataplexy (loss of muscle tone) * Sleep paralysis (inability to move or speak) * Excessive dreaming (as falling asleep or during waking) and waking in the night
434
Investigations for narcolepsy
* Sleep latency testing (rapid transition from wakefulness to sleep) * HLA testing
435
Management of narcolepsy
* Stop driving and inform DVLA * Good sleep hygiene * Modafinil dexamphetamine and methylphenidate (excessive daytime sleepiness) * Tricyclic antidepressants (clomipramine) or SSRIs (cataplexy)
436
What is Meniere's disease?
Long-term inner ear disorder that causes recurrent attacks of vertigo and symptoms of hearing loss, tinnitus and a feeling of fullness in the ear
437
Cause of Meniere's disease
Endolymphatic hydrops: excessive buildup of endolymph in the labyrinth of the inner ear, causing a higher pressure than normal and disrupting the sensory signals
438
Risk factors for Meniere's disease
* Autoimmune diseases * Viral infections * Migraines * Trauma * Syphilis * Female * 40-50
439
Clinical manifestation of Meniere's disease
UNILATERAL * Hearing loss (fluctuates, associted with vertigo) * Vertigo (20 minutes to several hours, in clusters over several weeks) * Tinnitus
440
How is Meniere's disease diagnosed?
Clinically, by an ENT specialist * 2 spontaneous episodes of vertigo lasting 20 minutes to 12 hours * Fluctuating hearing, tinnitus and/or perception of aural fullness * Hearing loss confirmed by audiometry to be sensorineural, low-mid frequency and of the affected ear * Not better accounted for by an alternative vestibular diagnosis
441
What investigations would you do to exclude other causes of vertigo, hearing loss and tinnitus?
* Bloods: FBC, U&E, TFT, lipid profile, syphilis screen * Audiogram * Imaging * Special tests: bithermal caloric test, electrocochleography and vestibular-evoked myogenic potential
442
What is the management of acute attacks in Meniere's disease?
* Bed rest and reassurance * Prochlorperazine * Antihistamine (cyclizine, cinnarizine, promethazine) NB: use buccal, IM or IV if vomiting
443
How to prevent attacks in Meniere's disease
* Avoid triggers (e.g. salt, caffeine, fatigue, stress, allergies, chocolate, alcohol) * Betahistine prophylaxis * Chemical labyrinthectomy with ototoxic drugs (e.g. gentamicin) * Surgical decompression of endolymphatic compartment of inner ear
444
Does someone with Meniere's disease need to inform DVLA?
DVLA advise all people with 'liability to sudden and unprovoked or unprecipitated episodes of disabling dizziness' to stop driving and inform the DVLA
445
446
What would NCS and EMG show for myopathy?
NCS normal EMG abnormal
447
What would an EMG show in myasthenia gravis?
**Jitter**: time between contractions of 2 muscle fibres varies
448
449
450
What is an investigation you could do for MND?
Motor evoked potential
451
What is transient loss of consciousness?
Spontaneous loss of consciousness with complete recovery
452
# Fits, faints and funny dos Causes of blackouts and funny turns
V - syncope, arrhythmia, TIA T - head injury M - hypoglycaemia O - non-epileptic seizure, epileptic seizure, panic attack, severe vertigo, narcolepsy, migraine, hydrocephalic attack
453
How can you tell the difference between syncope and epileptic seizures?
Syncope * Likely to be older with comorbidities and cardiac risk factors * Triggered by exertion or standing * Goes pale/clammy, palpitations/chest pain and goes dark * Eyes close, go floppy and collapse * Recover within seconds Epileptic seizure * Triggers include alcohol, sleep deprivation, illness and lights * May have aura or no warning * Eyes open, side of tongue bite, staring, posturing * Lasts minutes, with slow recovery (hours to days), feeling onfused and drowsy
454
How to tell the difference between epileptic and non-epileptic seizures
Epileptic: * Triggers include alcohol, sleep deprivation, illness and lights * Aura or no warning * Eyes open, side of tongue bite, staring, posturing * Lasts minutes, with a slow recovery (hours to days), feeling confused and drowsy Non-epileptic: * Tend to have a psychiatric history and social stressors * Can be triggered by heightened emotion * Thrashing violent movements, pelvic thrusting, back arching, eyes closed, tip of tongue bite, crying, distractible and waxing/waning * Long (up to an hour), with an unusually rapid recovery
455
What is an epileptic seizure?
Sudden, transient episode of abnormal and excessive, hyper-synchronous discharge of cerebral neurones causing symptoms or signs
456
What is epilepsy?
Ongoing tendency to experience recurrent epileptic seizures
457
Cause of epileptic seizures
Too much excitation or too little inhibition of clusters of neurones in the brain to bcome temporarily impaired and send out excitatory paroxysmal signals Fast/long-lasting activation of excitatory NMDA receptors and dysfunctino inhibitory GABA receptors
458
How do the causes of epileptic seizures vary with age?
Children/teenagers: genetic, perinatal and congenital disorders Younger adults: trauma, drugs and alcohol Older adults (> 60): vascular disease and mass lesions (neoplasms)
459
Causes of epileptic seizures
V - stroke, arteriovenous malformation, cavernous haemangiomas I - encephalitis, cerebral abscess, tuberculomas, meningitis, HIV, cerebral toxoplasmosis T - TBI, depressed skull fracture, penetrating injury, intracranial haemorrhage A - autoimmune antibody-mediated encephalitis M - hypoglycaemia, hypocalcaemia, hyponatraemia, hypoxia (cerebral palsy) I - brain surgery, antipsychotics, tricyclic antidepressants, lithium, lidocaine, ciclosporin, withdrawal of antiepileptics and benzodiazepines N - brain tumour C - neuronal migration defects D - Alzheimer's O - primary generalised epilepsies (childhood absence epilepsy, juvenile myoclonic epilepsy), alcohol, cocaine, hippocampal sclerosis, genetic disorders
460
What is the most common cause of epilepsy after the age of 60?
Stroke
461
How can neurocysticercosis present and what causes it?
Epileptic seizures Pork tapeworm in India and South America
462
What fraction of people with cerebral palsy have epileptic seizures?
1 in 3
463
What % of people who have had brain surgery will have seizures after?
17
464
What is a common presenting feature of brain tumour?
Seizures
465
How can alcohol cause seizures?
* Chronic alcohol ues * Heavy bouts of drinking * Periods of withdrawal * Alcohol-induced hypoglycaemia and head injury
466
Risk factors for epileptic seizures
* FH of epilepsy or other neurological illness * Premature birth * Genetic conditions associated with epilepsy
467
How are focal seizures categorised?
* Aura - hallucination, deja vu, fear * Motor * Loss of awareness and responsiveness
468
What is a Jacksonian seizure?
One limb jerking
469
Clinical manifestation of temporal lobe focal seizure
* Deja vu or jamais vu * Fear * Olfactory, gustatory or auditory hallucinations * Altered perception * Abdominal rising sensation * Nausea * Vertigo
470
Clinical manifestation of frontal lobe focal seizure
Conjugate gaze deviates away from epileptic focus and head turns
471
Clinical manifestation of occipital lobe focal seizure
Visual phenomena: zigzag lines, coloured scotomas
472
Clinical manifestation of parietal lobe focal seizure
Tingling, pain, numbness, prickling sensation
473
Clinical manifestation of focal seizure originating within motor cortex
* Jerking typically on one side of the mouth or one hand, sometimes spreading to involve the entire side (Jacksonian march) * Todd's paralysis (temporary paralysis of affected limbs)
474
Clinical manifestation of focal seizure with altered awareness or responsiveness
* Aura followed by period of complete or partial loss of awareness of surroundings, lasting for 1-2 minutes, which they don't remember * Speech arrest * Automatisms, e.g. lip smacking, dystonic limb posturing, walking in circle, undressing, swallowing, chewing, finger movements
475
What is the difference between focal and generalised seizures?
Focal on one hemisphere, generalised both hemispheres
476
Clinical presentation of absence seizures
* Begin in childhood (childhood/juvenile absence epilepsy) * Loss of awareness and vacant expression for < 10 seconds before returning abruptly to normal as though nothing happened * Slight fluttering of eyelids
477
Clinical manifestation of tonic-clonic generalised seizure
* Often no aura * Initial tonic stiffening followed by clonic synchronous jerking of the limbs, reducing in frequency over 2 minutes until the convulsions stop * Eyes remain open * May bite their tongue, be incontinent, breathe irregularly and utter an initial cry * Flaccid unresponsiveness followed by gradual return of awareness with confusion and drowsiness lasting 15 minutes to over an hour
478
Clinical manifestation of myoclonic seizures
* Momentary brief contractions of muscle(s) * Typically in teenagers (juvenile myoclonic epilepsy) * Often in morning after waking * Triggered by lack of sleep, alcohol, strobe lighting
479
Clinical manifestion of tonic seizures
Stiffening of body and fall, usually backwards
480
Clinical manifestation of atonic seizure
Sudden collapse with loss of muscle tone, falling forwards
481
Clinical manifestation of clonic seizure
Violent muscle contractions, jerking of limbs and trunk which accelerate and decelerate
482
Investigations for ?seizures
* ECG (transient tachyarrhythmias) * MRI brain in those with focal seizures and older patients where there's a greater chance of focal brain lesions * Bloods: blood gas, U&E, glucose, ESR/CRP, Ca * Infection screen: urine culture, blood culture, LP * EEG (classification or epilepsy): sleep recordings, 24 hour ambulatory EEG, inpatient EEG videotelemetry
483
What is hippocampal sclerosis?
Damage with scarring and atrophy of the hippocampus and surrounding cortex Seizures
484
EEG signs of absence seizure
3 Hz spike-and-wave
485
Signs of primary generalised epilepsies on EEG
Focal cortical spikes or generalised spike-and-wave activity
486
EEG activity during status epilepticus
Continuous activty
487
What is EEG for?
Classification of epilepsy, not distinguishing epilepsy from other attacks (incidental abnormalities in 0.5% of healthy adults (higher proportion in people with learning disability or previous CNS insult) and high false-egative rates)
488
Safety advice for people who have had a seizure
* Avoid swimming and dangerous sports, e.g. rock climbing * Leave bathroom unlocked * Take showers, not baths * Have carpets and radiator covers * Microwave meals * Caution with heights, traffic and heavy equipment * Stop driving for 6 months after 1 seizure or withdrawing from AEDs, until seizure-free for 12 months if recurrent seizures (5 years before 70 year driving license)
489
Drugs for epilepsy
When firm clinical diagnosis of epilepsy with risk of recurrent seizures, consider AEDs: * Introduce at low dose and slowly titrate upwards until seizure-free or maximum tolerated dose * If not seizure-free, introduce second AED and slowly withdraw first * If still not seizure-free, try combination therapy
490
If seizures are not controlled with multiple drugs, what do you do?
* Check concordance * Serum AED level * Review diagnosis
491
AEDs for generalised tonic clonic seizures
* Levetiracetam * Sodium valproate * Lamotrigine
492
AEDs for focal seizures
* Levetiracetam * Carbamazepine * Lamotrigine
493
AEDs for absence seizures
* Ethosuximide * Valproic acid * Lamotrigine
494
AEDs for myoconic seizures
* Levetiracetam * Sodium valproate NOT carbamazepine
495
Management of epilepsy where drugs don't work
* Resective epilepsy surgery (focal structural abnormality), e.g. temporal lobectomy (hippocampal sclerosis) * Vagal nerve stimulation * Ketogenic diet
496
When can you consider withdrawing AEDs?
Seizure-free for 2 years (50% have another seizure)
497
Initial management of seizure
* Place in safe position * Airway * High concentration oxygen * IV access * Prepare emergency medication * Blood glucose * If hypoglycaemic 150-200ml 10% glucose stat and commence 10% glucose infusion at 100ml/hour if seizures continue * If alcohol excess/malnutrition 1 pair Pabrinex IV before glucose replacement
498
Management of seizure at 5-15 minutes
Benzodiazepine, wait 5 minutes, 2nd dose * IV lorazepam 4mg bolus * Buccal midazolam 10mg * Rectal diazepam
499
Management of seizures after 2 doses of benzodiazepine
* Inform anaesthetic team and ITU * Dose 3: IV phenytoin (ECG monitoring!), valproate or levetiracetam * Following complete infusion, consider 2nd IV AED infusion of a different drug or IV phenobarbital * General anaesthesia with intubation and ventilation: propofol, repeat bolus, followed by continuou infusion
500
Care after seizure
* ABCDE at regular intervals * Consider critical care * Reinstate previous AED via NG tube * Look for underlying cause (CT/MRI head) * Review management of epilepsy
501
Complications of epilepsy
* Todd's paralysis (paralysis of limbs afected, subsides after 2 days) * Status epilepticus * Sudden unexplained death in epilepsy * Recurrent seizures (75%)
502
Side effects of AEDs
* Unsteadiness * Nystagmus * Drowsiness * Significant cognitive side effects * Skin rash * Blood dyscrasias
503
What is status epilepticus?
A seizure lasting more than 5 minutes, or 2 or more seizures without regaining consciousness in the interim
504
What % of status epilepticus have epilepsy?
50
505
Presentation of status epilepticus
Sustained generalised tonic clonic seizure which eventually becomes little movements (e.g. twitching around eye) or long absence/impaired awareness (altered mental state, subtle twitching, unusual behaviour)
506
What % of status epilepticus are non-epileptic seizures?
25%
507
Complications of status epilepticus
* Death * Permanent cerebral damage: epilepsy, focal neurological deficits, encephalopathy * Severe hypoxaemia > end organ damage * Rhabdomyolysis > AKI * Hyperthermia * Cardiac arrhythmias * Metabolic acidosis * Shock * Cerebral oedema
508
What is a non-epileptic seizure?
Episodes of change in movement, sensation or experience that resembles epileptic seizures, but without the cerebral discharges
509
Risk factors for non-epileptic seizures
* Psychiatric illness * Other somatoform disorders * Childhood sexual abuse * Female * Social/personal stressors
510
Clues that a seizure may be non-epileptic
* Long, up to an hour * Unusually rapid recovery * Situational, when heightened emotion * Eyes closed * Thrashing violent movements * Crying * Pelvic thrusting * Back arching * Tip of tongue bite * Waxing and waning symptoms * Distractible * No cyanosis or incontinence
511
Is oxygen affected in non-epileptic seizures?
No
512
Management of non-epileptic seizures
* Reassure * Psychiatric referral * Avoid AEDs and benzodiazepines * Support groups and online resources
513
What is a febrile convulsion?
Seizure that occurs in children with a high fever
514
Difference between simple and complex febrile convulsion
Simple - generalised tonic clonic seizure lasting less than 15 minutes and only occurring once during a single febrile illness Complex - partial or focal seizures lasting more than 15 minutes or occurring multiple times during the same febrile illness
515
Typical presentation of febrile convulsions
Child around 18 months presents with 2-5 minute tonic clonic seizure during high fever, usually caused by an underlying infection, e.g. tonsilitis
516
Management of febrile convulsion
* Identify and manage source of infection * Control fever with simple analgesia (paracetamol, ibuprofen) * Safety net: stay with child, put them in safe place (carpeted floor with pillow under head), call ambulance if lasting more than 5 minutes
517
What is Guillain-Barre syndrome?
Autoimmune, rapidly progressive demyelinating condition of the peripheral nervous system, often triggered by an infection
518
What are the different types of GBS?
* Acute inflammatory demyelinating polyneuropathy (90%) * Acute motor axonal neuropathy * Acute motor and sensory axonal neuropathy * Miller-Fisher syndrome
519
What causes GBS?
Infection Molecular mimicry: pathogenic antigen and myelin gangliosides in PNS share homologous epitopes, so the pathogen induces an antibody response (anti-ganglioside antibodies) against the myelin sheath of sensory and motor nerves, causing demyelination in patches along the length of the axon (segmental demyelination)
520
Risk factors for GBS
* Male * 15-35 or 50-75 * GI or respiratory tract infection * Lymphoma * Influenza vaccine
521
What are the most common organisms of infection that trigger GBS?
Bacterial: campylobacter jejuni, mycoplasma pneumoniae Viral: CMV, influenza, EBV, zika virus
522
Clinical manifestation of GBS
* Infection 1-2 weeks prior * Lower back and leg pain * Symmetrical paralysis and/or acroparaesthesia (tingling, numbness, etc.) affecting the distal limbs first (legs) and progressing proximally over several days to 6 weeks (upwards) * Loss of tendon reflexes in affected limbs * May progress to respiratory muscle weakness (SOB) * Cranial nerve involvement: speech problems, diplopia, facial droop * Autonomic dysfunction: tachycardia, HTN, postural hypotension, urinary retention
523
Clinical manifestation of Miller-Fisher syndrome
Ocular muscle palsies and ataxia
524
What criteria diagnoses GBS?
Brighton criteria
525
What criteria is in the Brighton criteria?
* Symmetric flaccid limb weakness * Lack or absent deep tendon reflexes in limbs with weakness * Monophasic course and time between appearance of signs from 12 hours to 28 days * CSF cellularity < 50 /uL (normal) * CSF protein concentration greater than normal (1-3 g/L) * Nerve conductino studies consistent with subtype of GBS * Lack of an alternative diagnosis for weakness
526
What would nerve conduction studies show in GBS?
Abnormal in 85% Slowing of conduction, prolonged distal motor latency and/or conduction block
527
What must you screen for before starting Ig therapy in GBS and why?
IgA deficiency Severe allergic reactions due to IgG antibodies if congenital IgA deficiency
528
What investigations are needed to diagnose GBS?
* Lumbar puncture * Nerve conduction studies
529
What antibodies are involved in GBS?
Anti-ganglioside antibodies
530
What imaging for GBS?
MRI brain and spinal cord
531
Treatment of GBS
* IV immunoglobulin for 5 days within the first 2 weeks * Repeat IV Ig if low and stable but not improving after 2 weeks OR * Plasma exchange
532
Major complication of GBS and management
Ventilatory failure Admit to ITU if signs of bulbar dysfunction or respiratory depression Mechanical ventilation
533
What prophylaxis needs to be considered in GBS?
Thromboprophylaxis with LMWH and compression stockings
534
What is it important to monitor in someone with GBS?
Vital capacity, if maximal expiratory pressure reduced by more than 30% go to ITU as emerging respiratory muscle weakness and may need mechanical ventilation
535
Complications of GBS
* Type 2 respiratory failure * Impaired mobility > venous thrombosis, PE * Intubation > respiratory tract infaction * Psychiatric: depression, PTSD, anxiety
536
Prognosis of GBS
7% die Prolonged recovery phase (months to years) 20% unable to walk unaided for 6 months 75% fully recover
537
538
What is anterior horn cell syndrome?
Disorders of the anterior grey matter horns of the spinal cord, presenting with flaccid weakness and areflexia
539
Causes of anterior horn cell syndrome
* Motor neurone disease * Spinal muscular atrophy * Poliomyelitis * Anterior spinal artery ischaemia * Hopkins syndrome (status asthmaticus)
540
541
How does carbamazepine work in epilepsy?
Inhibits sodium channels
542

Phase 3a Medicine (7 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions