Neurology - RF, pathology and symptoms Flashcards

1
Q

Alzheimer’s disease

RF
Pathology
Symptoms

A

RF - >65, family history, down syndrome, lifestyle factors (smoking, alcohol, obesity)

Pathology
MOST COMMON TYPE OF DEMENTIA
(Associated with ApoE-4 allele and down syndrome)

A chronic neurodegenerative disease due to
- Extracellular plaques of beta amyloid
- Intracellular neurofibrillary tangles composed of hyperphosphorylated tau protein.

Sx
- Memory loss
- Agnosia (can’t recognise people/things)
- Apraxia - can’t do basic motor skills
- Nominal dysphasia - difficulty naming things that are correctly perceived
- Disorientation

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2
Q

Parkinson’s disease

RF
Pathology
Symptoms
Unilateral or bilateral?

A

RF - >60 years male

Pathology
A chronic progressive neurological disorder due to degeneration of dopaminergic neurons in the substantia nigra

Symptoms
4 major
- Bradykinesia
- UNILATERAL Resting tremor (3-5Hz)
- Muscle rigidity (Cogwheel)
- Postural instability

Minor
- Micrographia
- Mask-like facies
- Depression - most common psychiatric feature
- Shuffling gait

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3
Q

Vascular dementia

RF
Pathology
Symptoms

A

RF- >60, history of stroke/TIA (doubles risk), obesity, hypertension, smoking

Pathology
STEPWISE progression (over several months - years)
Mostly due to cerebrovascular infarcts

Symptoms
- Cognitive - Difficulty concentrating/poor attention
- Memory disturbance
- Mood disorders
- Difficulty walking and keeping balance

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4
Q

Motor neurone disease

RF
Pathology
Symptoms
(Symmetric or assymetric?)

A

RF - Family history, >40 years, genetic predisposition

Pathology
An umbrella term of slowly progressive diseases affecting ONLY motor nerves.

1) Amyotrophic lateral sclerosis (most common) - both LMN and UMN sx
2) Primary lateral sclerosis - only UMN sx
3) Progressive muscular atrophy - only LMN sx

Sx
- Asymmetric limb weakness
- Wasting of small hand muscles
- Fasciculations
- ABSENCE of sensory signs/symptoms
- Respiratory/breathing issues (muscle weakness can progress to affect respiratory muscles)

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5
Q

Bulbar palsy

Pathology + most common cause
Symptoms

A

2nd most common MND (worst prognosis)

Most commonly caused by brainstem strokes and tumours

Affects CN 9-12
- Affects muscles of speech and swallowing

Sx - slurred speech, difficulty swallowing

(Treated via feeding tube. Speech and language therapy)

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6
Q

Multiple sclerosis

RF
Pathology
Symptoms

A

RF - 20-40 Females, family history, personal/family history of autoimmune disease

Pathology
Type 4 hypersensitivity - autoimmune demyelination in the CNS

1) Relapsing remitting –> flare ups of disease with periods of incomplete recovery
2) Primary progressive –> Gradually worsening symptoms and an accumulation of disability
3) Secondary progressive –> Develops from relapsing remitting

Sx (5 main ones)
(Spastic pareparesis) - UMN signs (Hoffmann’s)
- Numbness at different, unlinked sites
1) Charcot’s neurologic triad –> Nystagmus, intention tremor, dysarthria
2) Optic neuritis –> painful eye movements + blurred vision + red/green colour blindness
3) Internuclear ophthalmoplegia - inability to adduct the eye
4) Uhthoff’s phenomenon - worsening of MS symptoms due to heat (exercise/fever)
5) Lhermitte’s sign - Paraesthesia in limbs on neck flexion

(Urinary incontinence)

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7
Q

Huntington’s disease

RF
Pathology
Symptoms

A

RF - Family history, CAG repeat length on huntingtin gene

Pathology
Autosomal dominant –> Causing neurological dysfunction
(Regions affected have decreased GABA and acetylcholine. Increased dopamine)

Due to CAG repeat disorder – Genetic mutation of HTT gene on chromosome 4 (huntingtin protein)

Sx
- Chorea –> Involuntary, random, irregular body movements
- Psychiatry –> irritability, personality change
- Depression
- Saccadic eye movements

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8
Q

Meningitis

RF
Most common cause?
Pathology - 2 ways in which bacterial causes spread
Symptoms

A

RF - Crowding (e.g. student dorms/training camps), immunocompromised

Pathology
Mainly caused by viruses (but less severe) –> Coxsackie virus, herpes simplex virus

Bacterial causes (more severe)
- Streptococcus pneumoniae (MOST COMMON)
- Neisseria meningitidis (non blanching rash)
- Listeria monocytogenes (most common in elderly and immunocompromised)

Bacteria can spread via
- Haematogenous (more common)
- direct extension from a contiguous site (invade through the wall of an organ to other organs)

Sx
- Meningism –> Neck stiffness, photophobia, headache
- Fever, vomiting
- Rash (only in N.meningitidis)

(most common complication - sensorineural hearing loss)

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9
Q

Encephalitis
- Which lobe is usually affected?

RF
Pathology
Symptoms

A

RF - Extremes of age, immunocompromised, animal bites (mosquito, bats, cats)

Pathology
Caused by human simplex virus 1 in 95% of cases
Affects temporal lobe (+ inferior frontal lobe)

Symptoms
Fever
Headache
Focal features - Aphasia
Seizures
Vomiting

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10
Q

Guillain Barre Syndrome

RF
Pathology
Symptoms

A

RF - Preceding viral/bacterial infection

Pathology
Immune mediated demyelination of the peripheral nervous system often triggered by infection (Gastroenteritis, UTI)
- Campylobacter.jejuni

The antigens of the pathogens match the antigens of myelin sheath and axons (molecular mimicry) - Type 2 hypersensitivity

Symptoms
- Progressive ascending symmetrical weakness
- Respiratory distress (SOB)
- Double vision, facial drooping

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11
Q

Cerebellar disorders

Causes
Symptoms

A

A group of neurological conditions that affect the cerebellum which coordinates and regulates movement.

Causes
- Friedrech’s ataxia (inherited)
- Neoplastic: Cerebellar haemangioma (and paraneoplastic)
- Stroke
- Alcohol
- MS
- Drugs: Phenytoin, lead poisoning

Symptoms
DANISH
Dysdiadochokinesia, dysmetria (past-pointing)

Ataxia (loss of coordination - poor balance and walking

Nystagmus

Intention tremor

Slurred staccato speech (+ scanning dysarthria)

Hypotonia

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12
Q

Bell’s Palsy

RF
Pathology
Symptoms

A

RF - Pregnancy (3rd trimesters + 1st week post partum), 20-40 years

Pathology
Unilateral, idiopathic, facial nerve paralysis

Symptoms
Lower motor neuron facial nerve palsy –> Forehead is Affected (lesion between nucleus and face)

Upper motor neuron facial nerve palsy –> Forehead is spared (due to forehead receiving bilateral UMN input between motor cortex and nucleus)

(Droopy eyelids, drooling, smile unevenly)
(possible dry eyes - keratoconjunctivitis sicca (due to loss of adequate blink function) and altered taste)

Post auricular pain (behind the ears)

Loss of taste on anterior 2/3 of the tongue

(Check for shingles in the ear)

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13
Q

Epilepsy

RF
Pathology
Symptoms

A

RF- Head trauma, family history, stroke, (dementia, cerebral palsy)

Pathology
Common neurological condition characterised by recurrent seizures

Symptoms

Absence - stare into space, abruptly return to normal
- Myoclonic - limb jerking
- Atonic - body relaxes, fall forwards

Focal seizures (Consciousness varies)
Temporal - Aura, dysphasia
Frontal - Jacksonian march
Parietal - Paraesthesia
Occipital - Visual changes

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14
Q

List the type of seizures

What are the order of events?

A

Generalised seizures (consciousness is lost)
- Tonic-clonic (grand mal)
- Tonic - body stiffens, fall backwards
- Absence - stare into space, abruptly return to normal
- Myoclonic - limb jerking
- Atonic - body relaxes, fall forwards

Focal seizures (Consciousness varies)
Temporal - Aura, dysphasia
Frontal - Jacksonian march
Parietal - Paraesthesia
Occipital - Visual changes

Predromal - Mood changes, lightheadedness, visual changes (flashing lights)

During - Tongue biting, incontinence, irregular breathing

Postdromal - confusion, irritable, todd’s paresis - paralysis of arms and legs

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15
Q

Ischemic stroke/TIA

RF
Pathology
Symptoms (generally)

A

RF - Older age, family history, hypertension, smoking, diabetes, atrial fibrillation

Pathology
Ischemic stroke - A sudden interruption in the vascular supply of the brain due to a blockage of a blood vessel. (Resulting in infarction)

TIA - Transient focal neurological deficit that lasts from a few minutes to 24 hours, without infarction.

Symptoms
- Motor weakness
- Visual issues
- Balance problems
- Speech problems (dysphasia)

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16
Q

Symptoms of a

1) Anterior cerebral artery

2) Middle cerebral artery

3) Posterior cerebral artery

4) Lacunar

Infarct (Stroke)

A

1)
- Contralateral hemiparesis and sensory loss, lower extremity more affected.

2)
- Contralateral hemiparesis and sensory loss, upper extremity more affected
- Contralateral homonymous hemianopia
- Aphasia

3)
- Contralateral homonymous hemianopia with macular sparing
( Visual agnosia - cannot recognise things/people)

4)
- Either isolated hemiparesis/hemisensory loss/hemiparesis with limb ataxia

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17
Q

Essential tremor

RF
Pattern of inheritance?
Symptoms

A

RF - Age, family history

(most common cause of head tremor)
Autosomal dominant

Sx
- Postural tremor that is worse with arms outstretched (bilateral upper limb action tremor)
- Improved with alcohol and rest
(Problems with fine motor tasks e.g. writing, eating, dressing)

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18
Q

Muscular dystrophies

RF
Most common muscular dystrophy and pattern of inheritance?
Pathology
Sx

A

RF - Males, family history

Pathology
Muscular dystrophies are progressive , generalised diseases of muscle most often caused by a defective or absent glycoprotein in the muscle membrane (e.g dystrophin)

Duchenne muscular dystrophy - X linked recessive (mutation in DMD gene encoding for dystrophin)

Sx
- Progressive proximal muscle weakness (from 5 years)
- Calf hypertrophy (due to ongoing regeneration of muscle fibres)
- Gower’s sign
- Difficult to walk without assistance (ambulation difficulties)

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19
Q

Difference between Becker’s and Duchenne muscular dystrophy

A
  • Duchenne is more common, severe and rapidly progressive.
  • Duchenne presents in early childhood
  • Becker’s usually in later childhood or adolescence

(symptoms generally the same)

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20
Q

Brain abscess

RF
Pathology
Sx

A

A life threatening condition where there is a suppurative (pus like) collection of microbes (within a gliotic capsule) in the brain parenchyma.

RF - <30yr Men, Sinusitis, otitis media, infective endocarditis, meningitis, dental procedures/infection, penetrating/traumatic head injuries.

Causes as mentioned in RF (basically an infection or injury)+ sepsis extending from previous infections.

Sx
(Recent infection)
- Persistent, dull headache
- Fever
- Increased ICP leading to
+ 3rd/6th cranial nerve palsy
+ Papilloedema
+ nausea
+ Seizures
(Possible motor findings if it affects the motor cortex)

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21
Q

Herpes Zoster

RF
Pathology
Sx

A

A reactivation of varicella zoster virus acquired during a primary varicella infection.

RF - >50 years female, anything that promotes being immunocompromised (chronic steroid use, HIV, malignancies)

(Characterised by dermatomal pain and papular rash)
Sx
- Burning/stinging/itching pain that is confined to a dermatome
- Erythematous maculopapular rash (bumps)
(pain in affected eye and reduced vision - corneal ulceration)

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22
Q

Malaria
Most common protozoal cause?

RF
Pathology
Sx

A

Plasmodium falciparum - most common cause overall (most common in africa)

Plasmodium vivax - most common outside africa

RF - Endemic area, pregnancy, immunocompromised + old age (severe disease)

Sx
- Fever
- Headache
- Myalgia
- N+v

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23
Q

2 traits that are protective from malaria

A

Sickle cell trait (anaemia)

G6PD deficiency

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24
Q

Tumours that commonly spread to the brain

A

Lung (most common)
Breast
Bowel
Skin
Kidney

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25
Primary Brain Tumour - Glioblastoma RF Pathology Symptoms
(poor prognosis of 1 year) RF - White, male, history of exposure to ionising radiation, history of neurofibromatosis type 1. Most common primary tumour in adults (non-metastatic). It is a cancer arising in astrocytes (which provide structure and support for the neurons) Sx - Headache (worse with coughing, valsalva maneuvre or lying down due to increased ICP) - Gait abnormality - N+V - Seizure + Basically most of the neuro symptoms
26
Meningioma Risk factors Pathology Symptoms
A tumour arising from the meningeal cells of the arachnoid layer. (falx cerebri) RF - Radiotherapy, genetic predisposition Sx - Headache - Neurological deficit - Seizure
27
Vestibular schwannoma (previously called acoustic neuroma) Where does it arise from? Symptoms Which cranial nerves does it affect?
A benign tumour arising from the vestibular division of the 8th cranial nerve RF - Neurofibromatosis type 2 (associated with bilateral vestibular schwannoma) (At the cerebellopontine angle) Sx (Affects - CN 5,7 and 8) - Absent corneal reflex - Unilateral hearing loss (that could progressively worsen) - Dizziness - Tinnitus - Facial nerve palsy (due to compressing of neighbouring facial nerve)
28
Cerebral palsy RF Pathology Symptoms
An umbrella term referring to a non-progressive disease of the brain originating during antenatal, neonatal or early postnatal period --> resulting in disorders of movement and postural development. RF - Prematurity, low birth weight, meningitis, maternal illness, birth asphyxia - hypoxic ischemic encephalopathy, head trauma Sx - Delayed motor development (usually sit by 6 months and crawl by 9 months) - Abnormal gait - Muscle weakness - Delay in speech development (short sentences by 2 years) (some will have learning difficulties, epilepsy, hearing impairment)
29
Types of cerebral palsy and which is the most common?
Spastic (70%) - Diplegic (bilateral), hemiplegic (unilateral), quadriplegic (all 4 limbs) - Increased tone. e.g. clasp knife Dyskinetic - Athetoid movements - slow involuntary movements -- writhing (flexed elbow and wrist) - Speaking and swallowing issues Ataxic - Damage to the cerebellum. Problems with balance and coordination.
30
What is hypoxic ischaemic encephalopathy? Causes? Symptoms?
When the baby's brain does not receive enough oxygen and/or blood flow around the time of birth. Also referred to as birth asphyxia Causes - Issues with blood flow to the placenta - Heart disease - Pre-eclampsia (high BP and signs of organ damage) - Uterine rupture - Cord entanglement or compression Sx - Hyperalert or reduced level of awareness - Floppy or stiff - Unusual movements or seizures - Weak cry - Pauses in breathing (apnoea)
31
Common places for berry aneurysms
- Junction of the anterior cerebral artery and anterior communicating artery - Junction of the posterior communicating artery and internal carotid artery - Bifurcation of middle cerebral artery
32
Subarachnoid haemorrhage What does a painful 3rd nerve palsy indicate? RF Pathology Symptoms
RF - Hypertension, smoking, ADPKD (Chr 16 and 4) Pathology Bleeding in the subarachnoid space due to rupturing of a cerebral aneurysm. (most commonly caused by head injury, then spontaneous cause) -- If painful 3rd nerve palsy - Posterior communicating artery aneurysm Causes: Aneurysm, Trauma, Arteriovenous malformation Symptoms - Occipital thunderclap headache - Meningism- Photophobia and Neck stiffness - Nausea and vomiting - Seizures
33
Subdural haemorrhage RF Pathology Symptoms
RF - Recent trauma, anticoagulant use, advanced age (>65), alcoholism Pathology - Usually occurs due to recent trauma - Rupture of the bridging veins (seen in shaken baby syndrome) Symptoms (GILF) (Possible fall 5 weeks before) - Gradual onset of symptoms (possible slight lucid interval) - increasing progressive confusion - Increased ICP (Cushing's neurological triad - bradycardia, hypertension, irregular respiration - Loss of continence - Fluctuating GCS - range from mild confusion to coma, irritability (Unilateral weakness, aphasia)
34
Epidural/extradural haemorrhage RF Pathology Symptoms
RF- Young adults, head trauma at the pterion - fracture of the temporal bone Pathology - Trauma to the side of the head - Rupturing of the middle meningeal artery Symptoms (LIND) - Lucid interval - temporary improvement in patient's condition after which there is rapid deterioration - Increased ICP - cushing's neurologic triad - hypertension, bradycardia, irregular respiration - Nausea and vomiting - Decreased GCS
35
Giant Cell Arteritis RF Pathology Symptoms
RF - >50 female (peak in 70s), smoking Pathology - Associated with polymyalgia rheumatica - Granulomatous inflammation of the medium and large sized arteries, leading to narrowing and occlusion of the lumen. Symptoms - Unilateral headache - Scalp tenderness (combing hair) - Jaw claudication (e.g. chewing) - Blurred/double vision (amaurosis fugax) - Aching and morning stiffness (neck, shoulders, hips)
36
Migraine RF Pathology - steps of migraine Symptoms
RF/triggers - females, Chocolates, hangover, orgasm, cheese, oral contraceptives, Lie in, alcohol, tumult, exercise stress Pathology Prodromal- mood changes days before Aura - visual disturbances Episode Resolution Symptoms (4-72 hours) - Unilateral throbbing headache - Nausea and/or vomiting - Photophobia and phononphobia
37
Tension headache RF Symptoms
RF - Stress, missing meals, lack of sleep, dehydration Sx - Tight band around the head --> Bilateral constricting pain - (low-moderate intensity with no aura/photophobia)
38
Cluster headache RF Symptoms
RF - Male smokers, strong smells, alcohol (Lasts 15 mins - 2 hours) Sx - Intense, intolerable, sharp stabbing pain around one eye. - Red, swollen, watery eye - Ptosis and miosis - Rhinorrhea
39
Delirium RF Definition Causes What are the 3 subtypes?
RF - Elderly, dementia, polypharmacy, dehydration, visual/hearing disability (lack of sensory input negatively impacts the brain) An acute, fluctuating change in mental status, with inattention, disorganised thinking and altered levels of consciousness. Causes CHIMPS PHONED Constipation Hypoxia Infection (e.g UTI) Metabolic disturbance Pain Sleeplessness Prescription Hypothermia/pyrexia Organ dysfunction Nutrition Environmental changes Drugs 3 subtypes 1) Hyperactive delirium - Patient has heightened arousal, restlessness, agitation, hallucinations and inappropriate behaviour 2) Hypoactive delirium - Lethargic, reduced motor activity, incoherent speech, apathy, excessive sleeping 3) Mixed delirium - Combination of the 2
40
Lewy body dementia RF Pathology Symptoms
RF - Old age, Lewy bodies made of alpha synuclein and ubiquitin accumulate in the substantia nigra Symptoms - Visual hallucinations - Memory loss - FLUCTUATING Cognitive issues --> Attention, arousal (unlike other dementias - Parkinsonism - bradykinesia, cogwheel rigidity, resting tremor
41
Frontotemporal dementia Average age of onset? Symptoms
RF - 45-65 years Sx - Usually changes in personality and habits precede development of memory impairment - Progressive aphasia (can speak but doesn't make sense) (associated with MND)
42
Posterior inferior cerebellar artery strokes Anterior inferior cerebellar artery strokes Symptoms
PICA - Ipsilateral dysphagia, facial pain/numbness, temperature loss, Horner's syndrome - Contralateral limb/torso pain/sensory loss and temperature loss - Ataxia, nystagmus AICA - Ipsilateral facial paralysis and deafness - Contralateral limb/torso pain and temperature loss
43
Idiopathic intracranial hypertension RF Symptoms
RF - Females, obese, pregnancy, medication (COCP, steroids, tetracycline antibiotics) Sx - Headache - Blurred vision - Papilloedema (usually present)
44
Trigeminal neuralgia RF Pathology Symptoms Triggers
A unilateral facial pain syndrome in at least 1 of the distributions of the trigeminal nerve. RF - Increased age, multiple sclerosis, female Most common in patients with MS (20x) Triggers: shaving, brushing teeth, talking, touch, (cold) Sx - Unilateral pain that is electric shock like, stabbing, pain - in one of the distributions of the trigeminal nerve. - Triggered generally by facial.oral mechanical stimulation
45
Causes of horner's syndrome
Stroke, multiple Sclerosis pancoast's Tumour, Trauma Cluster headache, Cavernous sinus thrombosis
46
Radial nerve palsy Innervation Pathology Symptoms
C5-T1 Sensory - Most of the skin of the posterior forearm, lateral aspect of the dorsum of the hand and dorsal lateral 3 and a half digits excluding nail bed - median nerve Motor - Triceps brachii and extensor muscles of the forearm (posterior) Pathology - Commonly due to midshaft humerus fracture Sx - Wrist drop - Sensation impaired over innervated area Tx - Wrist splint and simple analgesia
47
Ulnar nerve palsy (cubital tunnel syndrome) Innervation Pathology Symptoms
C8-T1 Sensory - Medial one and a half fingers and associated palm and dorsum. Motor - Flexor carpi ulnaris, medial half of FDP and intrinsic muscles of the hand. Pathology - Caused by trauma at the medial epicondyle Symptoms - Claw hand - Sensory impairment in innervated areas
48
Common peroneal/fibular nerve palsy Pathology Symptoms
Most commonly caused by fracture of the fibular neck Sx - Footdrop - Loss of sensation over dorsum of the foot and lateral side of the leg
49
Cranial nerves 3,4,6 palsy Presentation
CN3 - Down and out (unopposed superior oblique) - Dilated pupils (sphincter pupillae) - Ptosis (levator palpebrae superioris) CN4 - Up and out (unopposed inferior oblique) - Vertical diplopia CN6 - Forced adduction (unopposed medial rectus)
50
Cranial nerve 5 palsy Presentation
Jaw is deviated to the affected side Loss of corneal reflex Loss of sensation in specific distributions of the trigeminal nerve (trigeminal neuralgia)
51
Cranial nerve 7 palsy Presentation
UMN lesion - unilateral facial drooping with forehead spared LMN lesion - unilateral facial drooping affecting the whole face. Parotid inflammation Bell's palsy - drooping eyelid, drooping corner of mouth
52
Cranial nerve 8 palsy Presentation
Hearing loss Loss of balance Associated with paget's disease of the bone -- due to compression of CN8.
53
Cranial nerve 9 and 10 palsy Presentation
- Speech and swallowing difficulties - Impaired gag reflex
54
Cranial nerve 11 palsy Cranial nerve 12 palsy Presentation
11 - Inability to shrug shoulders or turn head against resistance 12 - Tongue deviates towards side of lesion - Atrophy of the tongue
55
Which nerve palsy is associated with Increased ICP?
3rd CN (bleeding on the right side means it affects the right cranial nerve 3)
56
Diabetic neuropathy RF Symptoms
Complication of diabetes RF - Poorly controlled hyperglycemia, >70, hypertension Sx - Sensory loss in a glove and stocking distribution - (Possible LUTS symptoms) - Painless injuries - Reduced/absent ankle reflex
57
Radiculopathy Definition and causes
Refers to the compression of nerve roots as they exit the spinal cord and spinal column, leading to motor and sensory symptoms. Causes - Spinal stenosis - Bone spurs - Disc herniation Sx - Radiating , sharp shooting, limb pain - usually in a dermatomal pattern (Treatment - Sometimes goes away on its own First line - NSAIDS, lifestyle management e.g. weight loss, stretches and exercises Corticosteroids)
58
Spinal cord compression (myelopathy) Pathology (causes) Symptoms
Myelopathy - The result of compression of the spinal cord (radiculopathy - compression of nerve roots as they exit the spinal cord) Pathology Most common - metastatic spinal cord compression --> BLTKP Other causes - Disc osteophyte cord compression - Traumatic e.g. vertebral fracture - Disc prolapse - herniation (leads to spinal cord stenosis) Sx - Back pain - earliest and most common symptom - Lower limb weakness (unsteady gait) - Sensory loss and numbness - UMN signs e.g. hyperreflexia, babinski's sign (patient's might say they struggle with keyboard texting, buttoning up shirt, legs not doing as they want) (Red flags - dual incontinence, saddle anaesthesia)
59
Cauda equina RF Pathology Symptoms
Caused by compression of the lumbosacral nerve roots that extend below the spinal cord. RF - Lumbar disc herniation, spinal surgery/trauma, anticoagulants (Late diagnosis can lead to permanent nerve damage resulting in long term leg weakness and dual incontinence) Most common cause - Central disc prolapse (L4/5 or L5/S1) Sx - Low back pain - Bilateral sciatica - Saddle anaesthesia - Dual Incontinence
60
Brown sequard syndrome Symptoms
Examining below the level of the lesion - Ipsilateral hemiparesis (corticospinal tract) - can test with Hoffmann's sign - Ipsilateral loss of proprioception and vibration sense (DCML) - Contralateral loss of temperature and pain sensation (spinothalamic tract) (methyl prednisolone, occupational therapy, surgery)
61
Friedreich's ataxia Causes Symptoms
Mutation in FXN gene on chromosome 9 (leads to mitochondrial dysfunction) - Bilateral spastic paresis (hypertonia +paralysis) --> Lateral corticospinal tracts - Bilateral loss of proprioception and vibration sense --> DCML - Bilateral limb ataxia --> Spinocerebellar tract Intention tremor Tx - omaveloxolone
62
Syringomelia Pathology Symptoms
The development of a fluid filled cyst within the spinal cord. Congenital Causes - Chiari malformation type 1(causing the cerebellum to push into the spinal canal) Acquired causes - Spinal cord tumours - Spinal cord injuries (Anterior white commissure pathology) Sx - Progressive weakness and muscle atrophy in your arms and legs - but typically affects hands(ventral horns) - Hypotonia (ventral horns - LMN sx) - Loss of pain and temperature sensation (spinothalamic) - cape like, over arms and shoulders (MRI) (Usually observation only if symptoms not bothersome.) Just manage symptoms e.g. pain, physical therapy. Definitive - surgical
63
Anterior spinal artery/anterior spinal cord syndrome Pathology Symptoms
Caused by - crushing injury, surgery, atherothrombotic disease, vasculitis Disruption of blood flow through the spinal artery --> spinal cord tissue ischemia and infarction from the level of disruption --> infarction of the corticospinal and spinothalamic pathways. Sx - Bilateral spastic paresis (corticospinal tracts) - Bilateral loss of pain and temperature sensation (spinothalamic tracts) - Bladder/bowel incontinence (MRI)
64
Meniere's disease Pathology Symptoms
A disorder of the inner ear. Most likely due to over production or impaired absorption of endolymph leading to increased pressure in the endolymphatic system. RF - 40-50 peak onset, recent viral infection (weak rf) Sx (usually unilateral) (Fluctuating aural sx of the those mentioned below) - Vertigo (may lead to n+v) - Lasts minutes to hours - Tinnitus - Hearing loss (not progressive in contrast to vestibular schwanomma) - Aural fullness (a sensation of clogging of the ear/muffled hearing) (positive romberg's and nystagmus)
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Chronic fatigue syndrome (myalgic encephalomyelitis) RF Pathology Symptoms
Characterised as onset of persistent disabling fatigue, post-exertional malaise, pain, etc for at least 3 months. RF - Female sex, epstein barr infection in adolescents, Covid-19 Sx - Persistent disabling fatigue not alleviated with sleep or rest - Post exertional malaise - poorly tolerating minor levels of physical/mental exertion - Muscle/joint pain - Cognitive dysfunction - inability to think/concentrate, short term memory impairment - Sore throat - Headache
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Wernicke's encephalopathy RF Pathology Symptoms
A neuropsychiatric disorder caused by thiamine deficiency (most commonly seen in alcoholics) RF - Alcohol-use disorder, HIV infection and aids, cancer and chemotherapy, malnutrition, GI surgery Sx - Possible recent history of GI surgery - Mental status change e.g. confusion, slowing, impaired concentration, apathy - Nystagmus - CN6 palsy, conjugate eye palsy - Gait ataxia
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Complication of wernicke's encephalopathy?
Wernicke-Korsakoff syndrome - amnesia and confabulation (creating false memories) -Mental status change e.g. confusion, slowing, impaired concentration, apathy - Nystagmus - CN6 palsy, conjugate eye palsy - Gait ataxia
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Mononeuropathy Causes Common examples Symptoms
Mononeuropathy refers to pathology affecting a single peripheral nerve resulting in weakness, sensation disturbance and/or pain. Causes include 1) Trauma 2) Compression - Pressure from being in a fixed position for a long time e.g. confined to bed/paralysis 3) Inflammation - e.g. vasculitis --> Blocking transmission of impulses through the nerve Most common - Median nerve in the wrist - CTS - Ulnar nerve in the elbow - Radial nerve in the upper arm - Peroneal nerve below the knee Sx - Loss of feeling in the affected area - Pain/burning/pins and needles - Weakness in the affected area
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Mononeuritis multiplex RF Pathology Sx
An assymetrical peripheral neuropathy involving isolated damage to at least 2 separate limb or cranial nerves. (e.g. carpal tunnel and foot drop affecting the median and common peroneal nerves) RF - >50 years, sarcoidosis, vasculitis, hepatitis and HIV infections (recreational drug use) Most frequently results from nerve ischemia secondary to vasculitis. (including infections and malignancy) Sx - Numbness, weakness, pain in affected areas - Rash, livedo reticularis (vasculitis) e.g. polyarteritis nodosa
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Myasthenia gravis RF Pathology Symptoms
A chronic autoimmune disorder of the post-synaptic membrane at the NMJ in skeletal muscle - resulting in insufficient functioning acetylcholine receptors. RF - Family history of autoimmune disorders, females, >60 males (thymoma) Pathology Acetylcholine receptor antibodies (85%) bind to acetylcholine receptors at the postsynaptic nmj receptors --> blocking them and preventing Ach from binding and stimulating muscle contraction. (There is more build up with exertion so worse symptoms) Sx Muscle fatiguability - muscles become progressively weaker during periods of activity and slowly improves after periods of rest. Affects small muscles of the head and neck - Diplopia, ptosis, difficulty swallowing, slurred speech/speech fatiguability
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Lambert-eaton syndrome RF Pathology Symptoms
An autoimmune disorder at the NMJ RF - Small cell lung cancer, co-existing/family history of autoimmune disorder, smoking, - Paraneoplastic disorder in association with SCLC - Due to antibodies against voltage gated calcium channels in the presynaptic membrane of the NMJ Sx - Proximal Muscle weakness at (usually starting with lower limbs) e.g. difficult to climb stairs, standing from seated position - improves with exertion - Autonomic symptoms: dry mouth, impotence, difficulty micturating.
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What is tuberous sclerosis briefly?
An autosomal dominant disorder that presents with seizures, particularly in childhood. Seizures mostly occur at night. There may be roughened patches of skin over the lumbar spine (shagreen patch)
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What is hydrocephalus and what are the symptoms? What are the 2 types?
It is excess CSF within the ventricular system of the brain Obstructive (non-communicating) hydrocephalus - Blockage of flow of CSF due to tumours, acute haemorrhage and developmental abnormalities e.g. aqueduct stenosis (flor of CSF is blocked along one or more narrow passages connecting the ventricles) Non obstructive hydrocephalus (communicating) - Imbalance of CSF production and absorption. Increased CSF production (choroid plexus tumour) - RARE Failure of reabsorption at the arachnoid granulations (meningitis, post haemorrhagic) - more common - Normal pressure hydrocephalus Sx - Headache typically worse in the morning, when lying down and valsalva maneuvre - N+V - Papilloedema (Coma in severe cases) (In infants, increased ICP can increase their head circumference)
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DVLA requirements for 1) One off seizure 2) Multiple unprovoked seizures 3) Stroke/TIA 4) Multiple TIAs over a short period of time
1) 6 months no driving 2) Seizure free for 12 months 3) 1 month off driving - no need to inform DVLA 4) 3 months off driving and inform DVLA
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Normal pressure hydrocephalus Causes Sx
A form of non obstructive hydrocephalus characterised by large ventricles but normal intracranial pressure. RF - >65 years, vascular disease, diabetes Congenital - Arnold chiarimalformation (Secondary cause) Can be due to head trauma, subarachnoid haemorrhage, meningitis - resulting in reduced CSF absorption at the arachnoid villi Sx (develops over months) - Dementia (reversible) - Urinary Incontinence - Abnormal gait
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Autonomic dysreflexia Symptoms Treatment
- Spinal cord injury above T6 level Sx (most commonly triggered by faecal impaction/urinary retention) - Extreme hypertension - Flushing and sweating - (Uncomfortable abdomen) Tx - Removal of stimulus and treat hypertension
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Which antiepileptic drug can cause peripheral neuropathy - reduced sensation, numbness in a glove and stocking distribution. and bleeding gums (and lymphadenopathy)
Phenytoin
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Dementia, incontinence and abnormal gait =?
Normal pressure hydrocephalus
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Symptoms of subacute degeneration of the spinal cord (Taking folate supplements before B12 can precipitate this)
- Distal sensory loss and paraesthesia - Gait abnormalities/Romberg's positive (loss of proprioception and vibration sense with hyper reflexia) - dorsal columns and corticospinal affected (absent ankle jerk) So it is hyperreflexia BUT absent ankle jerk - due to peripheral neuropathy in vitamin B12 deficiency
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Wernicke's dysphasia sx Broca's dysphasia sx
Wernicke's - Fluent speech, impaired repetition, abnormal sounds Broca's - Non-fluent speech, laboured and halted speech, words make sense
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Dexamethasone, acetazolamide, mannitol (in increased ICP)
Dexamethasone - for oedema related to tumours Mannitol - Acute management of increased ICP Acetazolamide - inhibits carbonic anhydrase - increases excretion of bicarbonate (idiopathic intracranial hypertension)
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Causes of obstructive and non obstructive hydrocephalus
Obstructive - Head trauma resulting in haemorrhage - Tumour - Aqueductal stenosis - Complications of surgery Non obstructive - Impaired absorption of CSF (Problem with arachnoid granulations)
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Key features of neurofibromatosis type 1 and type 2
Type 1 - Cafe au lait spots - Axillary/groin freckles - pheochromocytoma Type 2 - Cafe au lait spots - Tinnitus, vertigo, hearing loss - Bilateral vestibular schwannoma - Meningioma
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3 main symptoms of normal pressure hydrocephalus
Dementia, abnormal gait, incontinence
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When should sumatriptan be avoided?
In patients with coronary artery disease
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4 main features of neuroleptic malignant syndrome
Dangerous condition seen in patients taking antipsychotics can also occur with parkinsonian drugs (e.g. levodopa) - usually when drug is suddenly stopped or reduced --- occurs due to the reaction to dopamine antagonists rigidity, hyperthermia, autonomic instability (hypotension, tachycardia) and altered mental status (confusion). (Causes muscle damage and neurotoxicity) - CK is extremely high - A known cause of AKI (secondary to rhabdomyolysis) Tx Stop antipsychotics and give IV fluids (Can also use dopamine agonists) - Transdermal dopamine agonist patch
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