5) neuro Flashcards
Cranial nerve ONE
- name?
- function? 1
- effect of palsy / lesion? 1
1 = OLFACTORY
function = SMELL
lesion = Loss of smell
Cranial nerve TWO
- name?
- function? 1
- effect of palsy / lesion? 1
2 = OPTIC
function = ACUITY / SIGHT
lesion = ↓Acuity and Sight
nb commonest causes of monocular or binocular blindness is not a cranial nerve lesion but a problem with the eye itself (cataracts, retinal problems)
- neurological disorders more commonly cuase loss of part of the visual field
Cranial nerve THREE
- name?
- functions? 4
- effect of palsy / lesion? 3
3 = OCCULOMOTOR
functions = Eye movements (MR, IO, SR, IR) = Pupillary constriction = Accommodation = Eye lid opening
lesion
= eye is DOWN and OUT
= Ptosis (droopy eye lid)
= dilated fixed pupil
nb pupil isn’t always dilated - can have causes that ‘spare’ the pupil!
Cranial nerve FOUR
- name?
- function? 1 (name of muscle moved!)
- effect of palsy / lesion? 2
4 = trochlea
functions
= supplies SUPERIOR OBLIQU (SO) muscle
= down and inward movement of eye
“SO4 = sulphate”
lesion
= Defective downward gaze → vertical diplopia
= Head tilt
Cranial nerve FIVE
- name?
- functions? 4 (1 sensory, 1 motor, 2 reflex)
- effect of palsy / lesion? 5
TRIGEMINAL
functions = facial sensation (3 zones) = muscles of mastication = corneal reflex (efferent) = jaw jerk reflex
lesion = Trigeminal neuralgia (shock paroxysms of U/L V1-V3) = Loss of corneal reflex (afferent) = Loss of facial sensation = Paralysis of muscles of mastication = Deviation of jaw jerk to weak side
nb Sensory branches
V1 = Opthalmic
V2 = Maxillary
V3 = Mandibulary
Cranial nerve SIX
- name?
- function? 1 (name of muscle moved!)
- effect of palsy / lesion? 1
ABDUCENS
function
= ABDUCTS the eye
= controls LATERAL RECTUS (LR) nuscle
“LR6 - ie not sulphate…”
lesion
= Defective abduction (left eye abn) → horizontal diplopia
Cranial nerve SEVEN
- name?
- function? 5 (2 sensory, 2 motor, 1 reflex)
- effect of palsy / lesion? 4
FACIAL nerve
functions = facial muscles = lacrimation + salivation = taste (anterior 2/3rds) = some hearing thing... = corneal reflex (afferent)
lesions
= flaccid paralysis of upper AND lower face (bell’s palsy)
= Loss of corneal reflex (efferent)
= loss/change of taste
= Hyperacusis - ↑sensitivity to certain frequencies
BELL’S PALSY:
- upper or lower motor neurone lesion?
- which cranial nerve affected?
- main symptoms? 3
- management? 1
- main way to differentiate from stroke? 1
LMN
cranial nerve 7 = facial!
- ipsilateral facial nerve paralysis (WITH inability to raise eyebrow)
- ipsilateral ear numbness and pain (hyperacusis)
- decreased taste
high dose prednisalone
get forehead sparing if UMN lesion (eg stroke) - so would still be able to raise eye brows if stroke!
nb bell’s palsy is norm idiopathic (70%) but can be secondary to weird things like sarcoidosis, ramsay hunt, MS etc
Cranial nerve EIGHT
- name?
- function? 2 (2 sensory)
- effect of palsy / lesion? 3
8 = VESTIBULOCOCHLEAR nerve
“the number 8 is curly like a cochlear?”
functions
= hearing
= balance
lesion
= SEONSORI-NEURAL hearing loss
= Vertigo
= Nystagmus
nb things like pagets, excess noise and acoustic neuroma and can damage
Cranial nerve NINE
- name?
- function? 3 (1 sensory, 1 motor, 1 reflex)
- effect of palsy / lesion? 4
GLOSSOPHARYNGEAL = 9
functions
= taste (posterior 1/3rd)
= swallowing
= gag reflex (afferent)
(sensation from posterior tongue and and pharynx)
lesion = loss of gag reflex (afferent) = difficulty swallowing? = change in taste? = Hyper-sensitive carotid sinus reflex
Cranial nerve TEN
- name?
- broad functions? 5 (4 mixed, 1 reflex)
- effect of palsy / lesion? 2 (just in head + neck)
VAGUS = 10
functions = phonation (muscles of vocal cords) = swallowing = gag reflex (efferent) = cardiac inhibition = autonomic functions of gut
(sensation from larynx and pharynx)
lesion
= Uvula deviates to CONTRALATERAL side of lesion (e.g. left deviating uvula = R vagus lesion)
= Loss of gag reflex (efferent)
also other effects with heart and gut etc but don’t need to know in detail!
Cranial nerve ELEVEN
- name?
- function? 1
- effect of palsy / lesion? 1
ACCESSORY NERVE = 11
functions
= trapezius + deltoid muscles (shrug shoulders, turn herad to left and right)
lesion
= Weakness turning head to contralateral side of lesion e.g. cannot turn head left = R accessory nerve lesion
Cranial nerve TWELVE
- name?
- function? 1
- effect of palsy / lesion? 1
HYPOGLOSSAL = 12
function = muscles of tongue
lesion
= tongue deviates to IPSILATERAL side (eg Left deviating tongue = Left hypoglossal lesion)
Possible causes of ALL / ANY cranial nerve lesions? (4 common, 3 rarer)
nb exclude causes that tend to only affecdt one or two specific nerves (this is on a seperate flashcard)
= tumour (secondary or primary)
= stroke
= MS
= diabetes mellitus
- sarcoidosis
- vasculitis (eg PAN or SLE)
- syphillis
What are the causes of a unilateral ptosis? 4
what other features would each cause have?
3rd NERVE PALSY
- Down and out eye + fixed dilated pupil
HORNER’S SYNDROME
- Ptosis + anhidrosis + miosis
MYASTHENIA GRAVIS
- Bilateral facial weakness + proximal weakness with fatiguability + weak voice
CONGENITAL
Specific cause to rule out of a 3rd nerve palsy? 1
what is the difference between a ‘medical’ and ‘surgical’ third nerve palsy in presentation? 1
remember this is OCULOMOTOR nerve
cause to rule OUT
= Compression from a posterior communicating artery aneurysm (ie berry aneurysm)
“Medical”: pupil sparing (and painless)
“Surgical”: pupil fixed and dilated
nb Pathology
Parasympathetic fibres are situated on the periphery of the 3rd nerve trunk and so are the first to be affected by compression resulting in a fixed and dilated pupil.
The classic cause of a “surgical” 3rd nerve palsy is a posterior communicating artery aneurysm. The vaso vasorum which supplies the 3rd nerve starts from the centre and supplies out radially.
In “medical” 3rd nerve palsies the centre of the 3rd nerve is affected first leaving the parasympathetic fibres and therefore pupillary constriction intact until the end.
Horner’s syndrome:
- describe features? 3
Disruption of the sympathetic nervous supply to the face resulting in:
= PTOSIS: unilateral partial drooping of eyelid
= MIOSIS: unilaterally constricted pupil
= ANHIDROSIS: unilateral loss of sweating
nb have Normal light and accommodation reflexes/eye movements
Common causes of damage to cranial nerve ONE? 5
- BLOCKED nasal passages e.g. common cold/flu
- Head TRAUMA
(Shearing of the olfactory bulbs as they pass through the holes of the cribriform plate) - ALZHEIMER’S and PARKINSON’S disease
(One of the earliest signs of cognitive decline and Alzheimer’s can be a loss of smell. This is the reason why patients with dementia often prefer stronger/more pungent foods) - MALIGNANCY: especially if unilateral (Meningioma / glioma / basal skull / frontal tumour)
- MENINGITIS
What visual field defect does a pituatory tumour classically cause?
fancy name and actually describe it
BITEMPORAL HEMIANOPIA
- loose OUTSIDE bit of vision on both sides (ie loose peripheral vision)
PITUATORY ADENOMA is the classic cause for a lesion at the OPTIC CHIASM
ALSO look for any other signs of pituatory disease (eg cushings, acromegaly, hypopituatirism, gynaecomastia)
Nb other causes of lesions at the optic chiasm include:
- Sarcoidosis/Tuberculosis
- Metastatic disease
- Internal carotid artery aneurysm
Causes of bell’s palsy? 5
which cranial nerve affected? 1
- VIRAL infection (likely Herpes Simplex Virus)
NB Treat with high dose steroids (60-80mg OD for 5 days) if diagnosed promptly
- BRAINSTEM LESION: demyelination / stroke / tumour
- CERELLOPONTINE ANGLE LESION: acoustic neuroma (vestibular schwannoma)
- MIDDLE EAR INFECTION
- PAROTID GLAND: tumour / post-surgical resection of tumour
affects cranial nerve 7 (facial)
UNILATERAL CEREBELLOPONTINE ANGLE (CPA) lesion:
- most common causes? 2
- three main cranial nerves affected? 3
- other neurological signs that may be present?
most common = acoustic neuromas (vestibular schwannomas)
2nd most common = meningiomas
nb need MRI imaging to image the lesion
CRANIAL NERVES AFFECTED
- TRIGEMINAL (5) (reduced facial sensation on affected side + loss of corneal reflex + impaired muscles of mastication)
- Lower FACIAL (7) (Weakness in upper and lower aspects of the face + loss of taste in anterior 2/3rds of the tongue)
- VESTIBULOCOCHLEAR (8) (Sensorineural hearing loss on affected side)
also get CEREBELLAR signs
nb If the Cerebellopontine angle lesion is very large it can extend and affect IX → XII nerves in order
Which way does the tongue deviate in a CN12 lesion?
which way does the uvula deviate in a CN10 lesion?
The tongue deviates TIOAWRDS the side of the CN12 lesion
Uvula is deviated AWAY from the side of the CN10 lesion
What are the final peripheral nerves that supply the arms? 5
1) musculocutaneous
2) axillary
3) radial
4) median
5) ulnar
MUSCULOCUTANEOUS nerve
- which nerve routes originate from? 3
- how normally injured? 1
- affect of injury? 2
MUSCULOCUTANEOUS
- C5-7
brachial plexus injury
effect of lesion
= inability to flex and supinate arm at elbow
AXILLARY nerve
- which nerve routes originate from? 2
- how normally injured? 1
- affect of injury? 3
AXILLARY
- C5-6
high humeral neck fracture / dislocation
effect of lesion
= flattened deltoid
= inability to abduct arm
= reduced sensation to reg badge
RADIAL nerve
- which nerve routes originate from? 4
- how normally damaged? 1
- affect of injury? 1
RADIAL
- C5-8
humeral midshaft fracture
effect of lesion
= wrist drop
MEDIAN nerve
- which nerve routes originate from? 3
- how normally injured? 1
- affect of injury? 3
MEDIAN
- C8, C8, T1
carpal tunnel (can also be complication of wrist fracture)
effect of lesion = CARPAL TUNNEL
= inability to flex wrist
= reduced sensation to lateral 3.5 fingers
= pain
also get HAND of benediction = fixed extension of lateral 3 digits
ULNAR nerve
- which nerve routes originate from? 2
- how normally injured? 1
- affect of injury? 2
ULNAR
- C8-T1
medial epicondyle fracture
effect of lesion
= Inability to abduct fingers
= Claw hand (inability to flex lateral 1 ½ fingers)
nb hand of bendiction and claw hand look similar - go by rest of hx and exam to differentiate between them
LONG THORACIC NERVE
- which muscle supply?
- how normally injured? 2
- affect of injury? 1
serratus anterior
- during high impact sports (eg blow to the ribs)
- complication of mastectomy
effect of injury
= winged scapula
ERBS PALSY
- which nerve roots damaged? 2
- how normally injured? 1
- affect of palsy? 3
C5-6
damage to upper trunk of brachial plexus due to SHOULDER DYSTOCIA during birth
WAITER’S TIP
- Arm adducted
- internally rotated
- elbow extended
KLUMPKE’S PALSY
- which nerve roots damaged? 2
- how normally injured? 2
- affect of palsy? 1
Damage of lower trunk of bracial plexus (c8, T1) due to:
- shoulder dystocia / extensive stretching of UL during delivery
- Sudden upward jerk of hand
get CLAW HAND
nb is a/w Horner’s syndrome – ptosis (droopy eye lid), miosis (pupil contriction), Anhidrosis (loss of sweat)
What does haemorrhage look like on a CT scan? (new and old)
Haemorrhage – new (Hyperdense - white), old (hypodense - dark grey)
SUBDURAL bleeds
- two main risk factors? 2
- norm mechanism of injury?
- which vascular structure damaged / bleed from? 1
- clinical presentation?
SUBdural
- elderly
- alcoholics
(or anyone with atrophic brains)
small fall or LOW-impact head injury in someone with RFs
BRIDGING VEINS torn
gradual fluctuating consciousness → unstable
EPIDURAL bleeds
- norm mechanism of injury?
- which vascular structure damaged / bleed from? 1
- clinical presentation?
EXTRAdural
HIGH impact temporal bone injury/fracture
MIDDLE MENINGEAL ARTERY)
loss of consciousness (always) → lucid interval → massive deterioriation
TENSION HEADACHE
- clinical presentation? (ie do socrates)
S = tight band squeezing the head (symetrically bilaterally - frontal)
O = gradual
C = band / squeezing
R = none
A = none (maybea bit of fatigue)
T = episodic (<15 days a month) or chronic (>15 days a month -norm dt meds)
E - stress (and dehydration)
S = mild intensity
Main DDx for a ‘HEADACHE’:
- primary headaches? 3
- secondary headaches? 7
- additional in pregnancy? 1
nb this is common and serious (ignore rare and less serious for now!)
PRIMARY
- tension
- migraine
- cluster
SECONDARY
- meningitis
- sinusitis
- SAH
- space-occupying lesion
- subdural / epidural (norm secondary to trauma)
- temporal / giant cell arteritis
- acute glaucoma
pregnancy = pre-eclampsia
nb also trigeminal neuralgia and idiopathic intracranial hypertension (also headache during flu or other acute illness)
What specific things do you want to know in hx/exam for HEADACHE to rule in/out each of these DDx:
- migraine 4
- cluster 4
- meningitis 4
- sinusitis 3
- SAH 4
- space-occupying lesion 6
- subdural / epidural 6
- temporal / giant cell arteritis 4
- acute glaucoma 4
- pre-eclampsia 4
- trigeminal neuralgia 3
- idiopathic intracranial hypertension 3
MIGRAINE
- aura
- nausea + vomiting
- photophobia
- better when lying still
CLUSTER
- pain behind one eye
- tearing / red eye
- nausea + vomiting
- better when pacing
MENINGITIS
- fever
- neck stiffness
- photophobia
- rash
SINUSITIS
- rhinitis
- fever
- headache over facial sinuses
SUB-ARACH HAEMORRHAGE
- sudden onset thunderclaop headache to back of head
- LOC
- photophobia
- neck stiffness
SPACE OCCUPYING LESION
- insidious onset (norm)
- pain wakes from sleep or wake with the pain and better when sitting/standing
- focal neuro deficits
- visual changes (papiloedema)
- vomiting
- seizures
SUB / EPIDURAL
- history of trauma (even if mild)
- LOC
- reduced GCS
- repeated vomiting
- seizures
- focal neuro deficits
GIANT CELL ARTERITIS
- polymyalgia rheumatica (proximal myalgia)
- jaw claudication (pain on chewing)
- scalp tenderness
- loss of vision (late)
ACUTE GLAUCOMA
- Seeing HALOS
- Ipsilateral headache
- redness
- sudden blurry / reduced vision
PRE-ECLAMPSIA
- pregnant (at least 20 wks)
- persistent headache
- changes in vision
- swelling in face (or hands/legs)
TRIGEMINAL NEURALGIA
- unilateral severe, sharp, shooting facial pain
- lasts few secs - 2 mins
- ache or burning between attacks
IDIOPATHIOC INTRACRANIAL HYPERTENSION
- headache worse on lying and morning
- nausea + vomiting
- visual changes incl diuplopia
What RED FLAG associated SYMPTOMS should you ask about if you suspect TENSION HEADACHE to rule out anything else?
nb this is excluding things like onset etc (incl head trauma) which you’d also ask about in socrates hx
what examination should you do?
- aura (migraine)
- visual changes (SOL, giant cell arteritis)
- photophobia (meningitis, SAH, migraine)
- LOC (SAH)
- neck stiffness (meningitis, SAH)
- fever (meningitis)
do FULL neuro exams (CN, UL, LL) and any focal neuro deficits = red flag (also any reduced GCS!)
nb also think of things like pregnancy (pre-eclampsia), head injury, pain waking from sleep, thunderclap, morning headache (SOL), hx of Ca (mets)
Management of tension headache:
- non-pharm? 3
- pharm? 2
- relaxation
- exercise
- massage
- paracetamol
- nsaids
be careful about analgesia overuse headaches though!
Socrates of migraine?
(1 for each, except 3 for exacerbating)
S = normnally UNIlateral (can be bi) O = couple of hours C = THROBBING R = none (can go face or neck) A = nausea + vomiting T = recurring, last 3hrs-3days
E
= movement
= light (photophobia)
= sound (phonophobia)
S = moderate-severe
nb diagnose migraine if someone has at least 2 attacks with aura or at least 5 attacks without aura
MIGRAINE
- describe common auras? 4
- how long these typically last?
1 in 3 people w migraines get auras!
- visual problems (flashing lights, zig zigs, blind spots)
- numbness or parasthesia in unilateral hand/arm
- dizziness or feeling off-balance
- difficulty speaking
(hnb can also get LOC but this is rare)
typically develop over course of 5 mins and last up to an hour
MIGRAINE
- main risk factors? 2
- most common triggers? 4
- food + drink triggers? 4
- women (F 2:1 M)
- obesity (excess oestrogen)
TRIGGERS = stress = lack of sleep = anxiety = dehydration - chocolate - cheese - alcohol - caffeine
nb in 50% of attacks, no triggers are identified!
MIGRAINE
- lifestyle advice? 1
- acute medical management? 3
- medical prophylaxis if serve/frequent? 2
encourage weight loss
ACUTE
- paracetamol
- nsaids
- triptan (PO or nasal)
nb don’t use triptans if also on SSRIs - risk of serotonin syndrome!
PROPHYLAXIS
- topiramate
OR
- propranolol
use prophylaxis if >2 a month
nb there are other meds that can be used as prophylaxis but these 2 are first line
nb topiramate CI in pregnancy
nb propranolol CI in asthma and PVD
ENCEPHALITIS
- commonest group of causes? 1 (and commonest specific cause 1)
- other possible groups of causes?
VIRAL = COMMONEST
HSV-1 most common in adults (95%)
(nb HSV-2 norm neonates)
nb everything below this is optional to know!
other viral infections: CMV, EBV, VZV, HIV, measles, mumps, japanese B encephalitis
NON-VIRAL
- bacterial meningitis, TB, malaria, legionella, listeria, scghistomiasis, typhus
ENCEPHALITIS
- three characteristic symptoms? 3
- condition that commonly coexists? 1
- additional features that may see in viral encephalitis? 4
1) FEVER
2) HEADACHE
3) ALTERED MENTAL STATUS
- ↓GCS or odd “encephalopathic” behaviour (confusion)
often co-exists with MENINGITIS (neck stiffness, photophobia etc)
- cold sores (HSV)
- conjunctivitis
- rash
- lymphadenopathy
nb also hx of travel or animal bite
ENCEPHALITIS - 1st line imaging? 1 - other main investigation? (nb also would do loads of bloods!) - empiric 1st line treatment for all? 1
CONTRAST enhanced CT
HSV = focal B/L temporal lobe involvement
(nb Meningitis = meningeal enhancement)
ALSO need LP (after CT)
- if viral: ↑↑Protein ↑↑Lymphocytes ↓Glucose
nb also Send for Viral PCR = 95% specific for HSV1
nb BLOODS to consider:
- Blood culture
- Viral PCR (HSV) – also throat swab
- Toxoplasma IgM titre
- Malaria film – thick and thin blood film
- Glucose – check not DKA
- LFTS – check not Hepatic encephalopathy
1ST LINE TREATMENT FOR ALL = IV ACYCLOVIR (start asap - ideally within 30 mins)
nb prognosis: 70% die untreated; 10-20% die if Mx is started promptly
FIBROMYALGIA:
- main symptoms? 5 (incl how long last before diagnose)
- criteria for diagnosis?
Chronic, widespread PAIN > 3 months –
- Multiple sites – pain all over
- Absence of inflammation
- Multiple ‘tender areas’
LETHARGY
SLEEP DISTURBANCE
HEADACHES
feeling DIZZY
‘brain fog’ - CONCENTRATION difficulties
diagnosis:
- multiple tender points
- > 3months
- no other cause found
nb fibromyalgia is often triggered by a stressful life event: eg giving birth, grief etc
Main DDx for fibromyalgia? 3
- multiple sclerosis
- RA
- chronic fatigue syndrome
nb common to have IBS, depression and/or anxiety alongside fibromyalgia
FIBROMYALGIA:
- non-pharm management? 3
- pharm management options? 3
- aerobic exercise
- CBT
- accupuncture / massage
- low-dose pre-gabalin
- duloxetine
- amitriptyline
nb don’t use nsaids as no inflammation
HYDROCEPHALUS:
- difference betweeen obstructive/non-communicating and non-obstructive/communicating?
- causes of obstructive? 3
- causes of non-obstructive? 3
OBSTRUCTIVE
= due to a structural pathology blocking the flow of CSF
- tumours
- acute haemorrhage (SAH or intraventricular)
- developmental abnormalities (eg aquaduct stenosis)
nb Dilatation of the ventricular system is seen superior to site of obstruction
NON-OBSTUCTIVE
= due to an imbalance of CSF production and/or absorption
- failure or reabsorption at the arachnoid granulations (e.g. meningitis or post-haemorrhagic)
- increased production of CSF (e.g. choroid plexus tumour (very rare))
HYDROCEPHALUS:
- main symptoms? 3
- 1st line imaging? 1
- other test to consider? 1
- Headache (typically worse in the morning, when lying down and during valsalva)
- Nausea and vomiting
- Papilloedema
^ie signs of raised ICP
nb additional in children:
- bulging fontanelle
- increase in head circumference
- failure of upward gaze (‘sunsetting’ eyes) due to compression of the superior colliculus of the midbrain
1ST LINE = CT SCAN
CONSIDER: lumbar puncture
- NOT in obstructive though (as can cause brain herniation)
Normal pressure hydrocephalus
- what is it?
- classic triad of symptoms? 3
unique form of non-obstructive hydrocephalus characterised by large ventricles but normal intracranial pressure
CLASSIC TRIAD
- dementia
- incontinence
- disturbed gait
MYASTHENIA GRAVIS
- pathology? (brief)
- conditions associated with? 3
- triggers for acute crisis / relapse? 5
autoimmune condition
- have auto-antibodies which bind to ACh receptors -> defieciency of nicotinic Ach receptors -> MUSCLE FATIGUE
90% have auto-auto-antibodies
ASSOCIATED WITH
- thymic hyperplasia (50-70%) and thymoma (15%)
- pernicious anaemia
- autoimmune thyroid disorders
(nb also other autoimmune conditions eg SLE and RA)
TRIGGERS
- pregnancy
- infection
- emotion
- exercise
- medications (beta blockers, gentamicin, opiates, tetracycline, quinine)
MYASTHENIA GRAVIS
- which muscles normally affected first? (then list which norm affected next)
- what muscle weakness improve and worsen with?
symptoms are BILATERAL and SYMMETRICAL
proximal muscle weakness in order:
1) EXTRA-OCULAR
2) bulbar (swallowing, chewing)
3) face
4) neck
5) limb girdle
6) trunk
Extraocular muscle weakness → Diplopia and Ptosis
Bulbar → Dysphagia, Dysarthria, Dysphonia (rare)
Increasing muscle fatigue with activity, slow improvement with rest
- ie WORST at the END OF DAY!
MYASTHENIA GRAVIS
- main 2 complications to worry about 2
1) Weak muscles of ventilation → acute resp failure
2) Weak pharyngeal muscles → compromise airways
Monitor PO2 + FVC, if poor NEED ventilate
Plasmapheresis (plasma exchange) or IV immunoglobulins
NB general management is Long acting Ach esterase inhibitors – Pyridaostigmine (1st line) and neostigmine (increase amount of Ach)
Cholinergic SE: saliva, sweat, vom, miosis, diarrhoea, colic
^opposite of anti-cholinergic SEs
Relapses: treat with Prednisolone and/or DMARDS
Plasma exchange and IV Immunoglobulin for crisis
MOTOR NEURONE DISEASE
- brief pathophysiology?
- most common type?
- UMN or LMN affected?
degenerative disease of MOTOR neurons → selective loss of neurons in motor cortex, cranial nerve nuclei, anterior horn → UMN/LMN dysfunction → progressive paralysis → death
ALS (amyotrophic lateral sclerosis or archetypal 50%)
In ALS get mix of UMN and LMN signs
- in rarer forms can get exclusively UMN or LMN signs
MOTOR NEURONE DISEASE
- most common presentation?
- other presentation?
LIMB ONSET (= commonest)
- Limb weakness (typically upper limbs)
- Muscle wasting of hands (drop objects)
- Difficulty manipulating objects (turning key, writing, dressing)
- Fasciculations of muscles prior to weakness
- Leg – foot drop (early), gait disorder, falls, fatigue whilst walking, difficulty rising from chair (prox myopathy)
“think eddie redmayne in film about steven hawking: dropping things and tripping over own foot!”
20% present w BULBAR onset
- Slurred speech (1st sign)
- Wasting and fasciculations of tongue
- Dysphagia (late, w/ significant speech difficulty)
- Difficulty eating, drooling, dysarthria, dysphonia, choking, aspiration
- Emotional lability
nb can rarely have respiratory onset as well!
EXAIMNATION FINDINGS
- UMN signs: weakness, hypertonia, hyper-reflexia
- LMN signs: weakness, hypotonia, hyporeflexia, fasiculations
DO A FULL CRANIAL NERVE, UL + LL NEURO EXAMS
MOTOR NEURONE DISEASE
- who to refer to if suspect? 1
- prognosis?
- focus of management?
refer to NEUROLOGY
2-4 years prognosis from time of diagnosis (dt resp issues)
management is focused on symptoms:
- hyoscine for drooling
- PEG for feeding
- quinine for cramps
- analgesia ladder
- opioid and benzos for SOB
How to differentiate between MS and Motor neurone disease?
in MS get a mixture of:
- sensory sx (parasthesia, numbness etc)
- motor sx
- autonomic sx (bladder urgency, freq, incontinence, sexual dysfunction etc)
in MND
- ONLY motor symptoms!! (no sensory loss or sphincter dysfuntion)
