Neurology shorts. Flashcards
Nerve conduction studies findings: (velocity, latency, amplitude)
- Demyelinating
- Axonal
- Demyelinating- decreased conduction velocity, increased distal latency, normal amplitude
- Axonal - decreased amplitude, normal velocity and latency
Carpal tunnel: focal slowing, conduction block
GBS: increased distal latency
Ddx: acute ascending motor paralysis.
Guillain Barre: good prognosis unless
- deficit >3/52 (rapid improvement if tx within 2/52)
- autonomic neuropathy.
Death from respiratory failure (ICU for ventilation if VC<1L) cardiac arrhythmias, PE.
Bulbar sx: diptheria, botulism, MG
Polio
Tick or snake bites + rhabdo
Polyarteritis nodosa
Acute intermittent porphyria
Arsenic poisoning
Consider critical illness myopathy and polyneuropathy.
DDx: autonomic neuropathy
D1A3
Additional features: postural hypotension, impotence, urinary retention, diarrhoea/constipation, horner’s syndrome.
- Diabetes
- EtOH
- Amyloidosis
- Acute intermittent porphyria
Neuro EXAM: Spastic paraparesis.
-General
-Neurological
-Other
-Extra
Ix:
-Imaging
-Bloods
-CSF
-Malignancy ?compression ?paraneoplastic
Mx:
-spinal cord compression: dex, RTX or surgery.
-MDT for neurorehab.
NEURO EXAM:
General: walking aids, scissoring gait.
Neurological: Bilateral lower limb spasticity (hip > knee) and weakness (flexor > extensor). UMN features of increased tone with clonus, hyperreflexia with extensor plantars and sensory loss at ? spinal level.
Other: cerebellar syndrome, cachexia, malignancy.
Extra: PR to check for anal tone (bowel and bladder).
Ix:
-MRI gold standard.
-FBC, VitB12, folate, ESR, CRP, syphilis serology
-CSF protein and oligoclonal bands
-myeloma screen
-tumour markers (lung, breast, prostate > kidney, thyroid).
Bulbar palsy - LMN.
Pseudobulbar palsy - bilateral UMN.
Unilateral lesion:
CN9/10 - palate moves away from affected side
CN12- tongue moves toward affected side
Including-
-causes
-features:
. reflexes (jaw, gag)
. tongue
. palatal movement
. speech
. emotions
. other (limb signs)
Bulbar palsy: LMN
Causes-
brainstem (infarct/mass/syringobulbia/subacute meningitis, carcinoma, lymphoma)
efferent nerves (MND, GBS)
NMJ (myasthenia gravis)
myopathies (rare bulbar myopathies).
Other- Neurosyphilis, Poliomyelitis.
Features-
LMN
-decreased reflexes (jaw jerk, gag)
- tongue wasting and fasciculations. “wasted, wrinkled, thrown into folds and increasingly motionless”.
- absent palatal movement
-nasal speech “indistinct (flaccid dysarthria), lacks modulation and has a nasal twang”
-Emotions – normal
-Other – signs of the underlying cause, e.g. limb fasciculations.
Pseudobulbar palsy: UMN
Causes-
bilateral internal capsule CVA,
MS,
MND,
brainstem tumours,
head injury.
Features-
-increased reflex (jaw jerk, gag)
-Tongue – spastic
“it cannot be protruded, lies on the floor of the mouth and is small and tight”.
- absent palatal movement.
-spastic speech “a monotonous, slurred, high-pitched, ‘Donald Duck’ dysarthria” that “sounds as if the patient is trying to squeeze out words from tight lips”.
-Emotions - labile. Pathological laughing or crying.
-Other – bilateral upper motor neuron (long tract) limb signs.
Note:
Cranial nerves-
9- Larynx
10- Pharynx
11- Soft palate
12- Tongue
UMN v LMN
-Wasting and fasciculation
-Tone
-Power (weakness)
-Reflexes
-Spastic v Flaccid paralysis
UMN (upper so MORE)
-No fasciculation or wasting
-Increased tone
-Pyramidal weakness (UL extensors, LL flexors)
-Increased reflexes
-Spastic paralysis
LMN
-Fasciculation and wasting
-Decreased tone
-Focal weakness
-Decreased reflexes
-Flaccid paralysis
Friedrichs ataxia.
-Young man
-Autosomal recessive: tri nuc rpt Chr 9
-Combination of spinocerebellar signs, corticospinal tract signs and dorsal column loss suggests friedrich’s ataxia.
Other features.
DDx (4)
Spinocerebellar tract degeneration:
-Past pointing, intention tremor, dysdiadochokinesis, rebound, heel-shin ataxia, dysarthria, nystagmus.
-Gait: ataxic/spastic gait
-Wheelchair/walking aid
Corticospinal tract degeneration = spastic paraparesis:
-Increased tone
-Pyramidal weakness
-Upgoing plantars and clonus
Peripheral neuropathy - absent ankle jerk and knee reflex with upgoing plantars.
-Dorsal column signs: loss of vibration/proprioception sense
-Rhombergs positive
Other- cardiomyopathy, cataracts and sensorineural deafness. Pes cavus, kyphoscoliosis and diabetes.
Clinical features:
Hands and arms: finger prick (diabetes), coordination (past pointing, intention tremor, dysdiadochokinesis, rebound).
Eye: nystagmus
Mouth: high arched palate, dysarthric speech
Ears: hearing aid, CN VIII (sensorineural hearing loss)
Chest: apex beat, heart sounds, pacemaker (hypertrophic cardiomyopathy, conduction defects)
Back: kyphoscoliosis
Fundoscopy: optic atrophy
DDx:
FA
Demyelination (would expect brisk reflexes)
Mixed upper and lower motor neuron signs
-cauda equina
-MND
-SCD cord
-dual pathology (cervical myelopathy and peripheral neuropathy)
Vit E deficiency
DDx mixed UMN and LMN signs (6)
Friedrichs ataxia
Subacute combined degeneration of cord
Conus medullaris pathology
Motor neuron disease
Syphilitic Taboparesis
Combined pathology: Cervical myelopathy and peripheral neuropathy
DDx spastic paraparesis
Compression
Transverse myelitis
Specific cord syndromes
Degenerative
Others
Compression
-tumour
-osteoarthritis
-trauma/fracture
-central disc prolapse
Transverse myelitis
-MS (cerebellar signs)
-Inflammatory
-Vascular
Specific cord syndrome
-anterior spinal artery
-brown sequard
-central (pain/temp and UMN weakness arms > legs), subacute),
Degenerative
-Hereditary spastic paraparesis
-Motor neurone disease (absent sensory signs, mixed UMN/LMN signs)
-Friedrich’s ataxia (cerebellar signs).
-Infective (HIV myelopathy)
Others: cerebral palsy, subacute combined degeneration of the cord
Spinal cord syndromes
-anterior spinal artery
-brown-sequard syndrome
-central cord syndrome
-subacute combined degeneration of the cord
Anterior spinal artery:
. Bilat lower limb paraparesis
. Bilat loss of pain/temp
. Cause: aortic surgery, artherosclerotic disease, cardiac arrest/emboli, Vasculitis,
Brown-sequard syndrome:
. Cause: hemisection spinal cord (traumatic v non-traumatic: disc, spondylosis, cyst, tumor, MS, radiation, vascular, infection: tb, transverse myelitis, herpes zoster, empyema, meningitis).
. Ipsilateral weakness and loss of proprioception / vibration
. Ipsilateral horners if T1
. Contralateral loss of pain/temp.
Central cord syndrome:
. Cause: hyperextension impingement,
. UL > LL motor, bladder dysfunction with sacral sparing, pain/temp > light touch “cape” distribution.
Subacute combined degeneration of cord:
. cause: Vit b12 deficiency (ddx: copper def)
. Spastic paraparesis, rombergs positive, loss of vib/proprioception, ataxia, reduced sensation (mixed umn and lmn signs).
. Complicated by high output anaemia and heart failure.
DDx dissociated sensory loss
- spinothalamic (pain/temp)
- dorsal column (vibration/proprioception)
Spinothalamic only
- syringomyelia
- brown-sequard (contralateral)
- anterior spinal artery thrombosis
- lateral medullary syndrome
- small fibre peripheral neuropathy
Dorsal column only
- subacute combined degeneration of the card
- brown-sequard (ipsilateral)
Syringomyelia level
- inferiorly thoracic level
- superiorly
. horner’s syndrome
. internuclear opthalmoplegia
. bulbar palsy
. usual
- inferiorly thoracic level: chest
- superiorly
. horner’s syndrome (C8/T1)
. internuclear opthalmoplegia (C5)
. bulbar palsy (medulla)
. usual (C2-T9)
DDx cervical myelopathy.
Cervical myelopathy:
Bilateral symmetrical weakness
. spastic scissoring gait
UMN signs at C ?
. increased tone
. brisk reflexes
Symmetrical sensory deficit
. dorsal column / pain deficit in dermatomal distribution
IF profound proprioceptive loss = pseudoathetosis.
Other cervical cord disorders-
- extrinsic.
. mass/tumour
. epidural abscess
- intrinsic.
. infarction/vascular malformation
. transverse myelitis
. syringomyelia
. subacute combined degeneration
Amyotropic lateral sclerosis (MND): mixed UMN/LMN sings with no sensory deficit.
Transverse myelitis.
-Axonal demyelination.
-Symptom onset.
-Causes (3).
-Mx.
Axonal demyelination: Inflammation of the spinal cord across thickness of spinal cord.
Symptom onset: hours-weeks.
Causes: MS, viral (herpes, EBV, COVID)/ other infection, often no cause, disc infarct.
Mx: steroids, PLEX, neurorehabilitation.
DDx tremor
-rest
-intention
-action
Drugs that cause tremor.
Note. action tremor includes intention tremor.
Intention tremor = worse on getting closer to intended target.
Rest: parkinsonian
Intention: cerebellar
Action: thyrotoxicosis, anxiety, drugs, familial, idiopathic.
Drugs e.g. salbutamol, theophylline, nicotine, antihistamines
valproate/lithium, lamotrigine, TCA, MAO inhibitors
cyclosporin
amiodarone, thyroxine,
nifedipine
Neuro exam: Parkinsons
EXAM:
-general
-neurological
. UL
. gait
. speech
. writing
. extra
. non-motor symptoms
. eye / cerebellar for parkinsons plus
DDx parkinsons syndrome.
-idiopathic parkinsons
- medication PARKINS
- Parkinson’s plus (5)
. red flag features such as symmetrical onset, early falls (wheelchair), lack of response to levodopa, supranuclear gaze palsy (limited downgaze), cerebellar signs.
-vascular
-wilsons
-benign essential tremor
IX: clinical dx, levodopa trial / apomorphine challenge, cerebral imaging for exclusion, dopamine transporter (DaT scan).
Mx:
-MDT, specialist, physio, OT, specialist nurse, OT
-Meds: levodopa, dopamine agonist (rotigotine patch, pramipexole, ropinirole, apomorphine / caution for hyperreligiosity, gambling, hypersexuality, compulsive eating), anticholinergic (trihexyphenidyl for tremor), selegiline, rasagiline (MAOB inhibitors), talcapone, entacapone, opicapone, COMT-inhibitor
-surgery (DBS)
Complications:
-disability and cognition
-depression
Pathophysiology: degeneration of dopaminergic neurones in substantia nigra in basal ganglia, hallmark lewy bodies.
EXAM:
-General: poor facial expression, slow movement, reduced blinking (off-medication) or excess choreodystonic movements (on-medication).
-neurological:
. resting tremor
. rigidity (cog-wheeling)
. bradykinesia
. gait: failure to initiate, short steps, shuffling, festinating, freezing.
. speech: soft, monotonous
. micrographia
-extra: advanced therapy (infusion pumps = apomorphine subcut, duodopa via PEJ, DBS)
-mood, cognition, function
-eye and cerebellar exam.
DDX:
Parkinsons syndrome
Medication- symmetrical (neuroleptics)
Prochlorperazine
Antipsychotics (haloperidol, risperidone)
Reglan (metoclopramide, ondansetron)
Ketamine
Isoniazid
Non-dihydropyridine ccb (verapamil)
SSRIs
Parkinsons plus
- MSA (bilateral motor features, cerebellar signs, autonomic dysfunction, dysarthria).
- PSP (vertical gaze palsy, prominent axial rigidity, bilateral motor features, dysarthria).
- Corticobasal syndrome (asymmetric motor symptoms, cortical sensory deficits, cognitive changes, postural instability +/- dystonia, myoclonus, apraxia).
-Lewy body dementia.
- NPH (Parkinson’s gait, urinary incontinence, cognitive impairment).
Stroke or space occupying lesion of basal ganglia.
Post-encephalitis.
Wilsons disease
Benign essential tremor.
Motor neurone disease.
Subtypes
-ALS
-PMA
-PLS
-Progressive bulbar palsy
Prognosis: 3-5 years from dx, aspiration pneumonia/ventilatory failure cause of death.
INVESTIGATIONS:
-Primarily clinical diagnosis
-EMG
-NCS
-MRI
MX: PT, OT, Speech, swallowing/nutritional support (NG/PEG), respiratory support (NIV, tracheostomy, invasive ventilation), ACP, specific therapy (riluzole: glutamate inhibitor that acts by inhibiting voltage-gated sodium channels).
DDx MND.
Amyotrophic lateral sclerosis (ALS) (Gene: SOD1 Chr21).
* UMN (hyperreflexia) and LMN (wasting, fasciculations) features with limb weakness
* Sensation normal.
* bulbar / pseudobulbar palsy (LMN)
Progressive muscular atrophy (PMA) (anterior horn cells only- distal muscle groups).
* LMN only (flaccid weakness, fasciculation, wasting, hyporeflexia).
Primary lateral sclerosis (PLS)
* UMN only (begins lower limbs, spastic gait, hyperreflexia).
Progressive bulbar palsy
* Only lower cranial nerves affected (IX, X, XII).
* Speech: ‘Donald Duck’/nasal speech.
* Dysphagia (weak palatal muscles).
INVESTIGATIONS:
Primarily clinical diagnosis (UMN/LMN signs, progressive spread)
EMG: fasciculations, fibrillations and positive sharp waves.
NCS: abnormal motor nerve conduction, reduced muscle action potential, repetitive stimulation with decremental response, decreased velocity, increased latency
MRI: exclude cervical myelopathy, spondylosis or cord compression
DDx MND.
-denegerative: cervical cord compression, cervical spondylosis
-inflammatory/traumatic/inherited: syringomyelia, spinal muscular atrophy
-infectious: polio, syphilis
-malignant/paraneoplastic
Neuro exam: MS
- chronic inflammatory autoimmune disease of CNS
- >= 2 demyelinating CNS lesions, MRI separate time and space
-lhermitte’s: neck flexion with electric shock in trunk and limbs (also cervical spondylosis)
-uhthoff’s: increased sx severity with temperature or exercise.
-classification
. relapse-remitting
. secondary progressive
. primary progressive
DDx multiple sclerosis (4)
Ix:
-MRB + spine (areas of demyelination)
-LP CSF (IgG oligoclonal bands, protein in CSF)
-VEP (delayed response)
Mx:
-MDT, education, MS support group
-Acute relapse: IV methylpred
-disease modifying drugs
.interferon beta-1a/1b
.glatiramer acetate
.azathioprine
.natalizumab (highly active relapse-remitting dx)
-symptomatic treatment
.spasticity: PT, baclofen, tizanidine
.urinary dysfunction: oxybutynin, IDC
.constipation: laxatives, enema
.pain: amitriptyline, carbamazepine, gabapentin
.fatigue: amantadine
.depression: SSRIs, support groups
General:
-walking stick, wheelchair
-catheter
-ataxic gait
-dysarthria
-mood (depressed/elevated).
Eyes:
-INO
-optic neuritis (loss of colour vision
-RAPD
-Fundoscope: swollen, pale or normal), central scotoma, nystagmus
Neurological: spastic paraparesis, cerebellar signs
DDX:
Other demyelinating disorders:
1. NMO = neuromyelitis optica.
. AQP4-ab (optic neuritis + acute myelitis).
2. ADEM= Acute disseminated encephalomyelitis.
. post-infectious (CMV/EBV/HSV/influenza/HIV/mycoplasma pneumonia/Hep A).
. Ix: MRI T2 FLAIR.
. Mx: Acyclovir if evidence of meningitis. high dose IV steroids, Other-IVIG, PLEX or cyclophosphamide
3. CADASIL = cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
. migraine, small vessel ischemia early = early strokes and dementia.
. Dx: genetic, skin biopsy (granular osmiophilic material around smooth muscle in small arteries).
4. Anti-MOG disease
5. Friedrichs ataxia (cerebellar and UMN signs).
CN 3 palsy (3) oculomotor nerve
Causes:
-surgical (4)
-medical (3)
CN3 anatomy:
. midbrain nucleus (subnuclei innervating individual EOM, eyelids, pupils).
. single central subnucleus = levator palpebrae superioris (bilateral ptosis if midbrain lesion)
. exits nucleus for ventral course near red nucleus and corticospinal tracts
. subarachnoid space: lateral wall cavernous sinus
. superior orbital fissure: divides into superior and inferior branches
FEATURES:
1. Complete ptosis
2. Fixed dilated pupil (unreactive to light or accommodation)
3. Down-out gaze (divergent strabismus)
Extras:
-stigmata of diabetes
-MG
-graves (proptosis, neck lump/scar)
-MS: spastic paraparesis, cerebellar signs, walking aid/wheelchair
-mitochondrial disease: hearing aid, proximal myopathy, ataxia, PPM (cardiomyopathy)
-Miller-Fisher syndrome: peripheral neuropathy, ataxia, areflexia
Causes:
-Surgical: affecting pupil
. posterior communicating artery aneurysm
. space occupying lesion (midbrain, sphenoid wing/cavernous sinus) Note. cavernous sinus = CN3,4,5,6+horners syndrome
. haemorrhage
-Medical: not affecting pupil
. mononeuritis multiplex (diabetes)
. demyelination
. infarction
Mononeuritis multiplex: (WARDS PLC)
-mononeuropathy
-progressive motor and sensory deficits in the distribution of specific peripheral nerves.
-Pain (neuropathic - area of sensory loss and deep pain - affected extremity).
-mx: splint, physio, surgery
Wegners (GPA) granulomatosis
Amyloidosis
RA
DM
SLE, sarcoidosis
Polyarteritis nodosa
Leprosy
Churg strauss (EGPA), carcinoma
CN6 palsy (3) abducens nerve
Causes:
- bilateral (4)
- unilateral
. central (4)
. peripheral (4)
- Unable to abduct
- Inward deviation (severe)
- Diplopia (toward affected side) - horizontal and parallel images, outer image from affected eye and disappears on covering, also the more blurred image usually.
Causes:
DDx:
- bilateral (4): raised ICP, wernicke encephalopathy, trauma, mononeuritis multiplex.
- unilateral
. central (4): vascular, tumour, MS, infection (lyme disease, syphilis)
. peripheral (4): raised ICP, trauma, diabetes, idiopathic
INO
Aetiology (2)
One-and-a-half-syndrome.
Aetiology: MS, stroke, tumour of dorsal pons
R) eye adduction fail
L) eye nystagmus (working hard to pull failing eye).
Aetiology: MS, stroke (basilar artery or paramedian branches).
One-and-a-half-syndrome (INO + CN 6 palsy): MLF interneurons in abducens pontine nuclei means contralateral eye can’t adduct when there is failure of abduction.
Horners syndrome
Partial ptosis
Miosis
Anhidrosis
Anatomical region and area of anhidrosis.
-central (3): lateral medullary syndrome (ipsilateral nystagmus, pain/temp, cerebellar + contralateral pain/temp limbs)
-preganglionic (2): clubbing, hand wasting + abduction weakness.
-post-ganglionic (2): neck surgery scar / mass / lymphadenopathy.
Anhidrosis:
-central (face, arm, upper trunk):
. hypothalamic stroke/tumour
. brainstem (vertebral a. dissection=lateral medullary syndrome)
. Spinal cord (trauma = brown-sequard/syringomyelia) brown-sequard (above T1)
-preganglionic (face only):
. tumour (apical lung, thyroid, mediastinal).
. Iatrogenic/ trauma (neck/brachial plexus/first rib #)
-post-ganglionic (sweating not affected):
. trauma (carotid artery dissection/aneurysm = damage to superior cervical ganglion)
. tumour (NP ca, lymphoma)
. cavernous sinus lesion.
NEUROLOGY EXAM: Upper limb
UL neuro exam:
GEN INSP
-diagnostic facies
-scars
-skin (neurofibromata, cafe au lait)
-abnormal movements
SHAKE HANDS (myotonia)
MOTOR
LOOK- arms, shoulder girdle
-wasting, fasciculation
-tremor, pseudoathetosis
-drift
FEEL
-muscle bulk, tenderness
-thickened nerves (eblow: ulnar, median: wrist, radial: wrist)
TONE- wrist, elbow
POWER-
-shoulder (abd: C5-6 add: C6-8)
-elbow (flex: C5-6 ext: C7-8)
-wrist (flex: C6-7 ext: C7-8)
-fingers (flex/ext: C7-8 abd/add: C8/T1)
-ulnar lesion (loss of finger abd/add)
-median n lesion (thumb abd)
REFLEXES-
-bicep (C5-6), biceps
-tricep (C7-8), triceps
-supinator (C5-6), brachioradialis
-hoffmans (C8) UMN
COORDINATION-
-finger to nose (intention tremor, past pointing)
-dysdiadochokinesis
-rebound
SENSORY
-pain (pinprick)
-proprioception
-vibration
-light touch
OTHER
-thickened nerves
-axillae
-neck
-lower limbs
-cranial nerves
-urine analysis??
NEUROLOGY EXAM: Gait.
- Hemiplegic: cerebrovascular disease
- Diplegic gait: cerebral palsy
- Parkinsonian: extrapyramidal disorders
- Ataxic gait: cerebellar
- Stamping (sensory ataxic) gait: proprioceptive disorders
- Neuropathic (high stepping) gait: foot drop pathologies.
- Myopathic (waddling) gait: myopathic disorders
- Antalgic: pain
- Normal
- Hemiplegic: cerebrovascular disease
. UMN lesion = contralateral decorticate posturing (pyramidal weakness: UL extension weakness, LL flexion weakness). Arm flexed, leg extended.
. Distal > proximal weakness (wrist/hand weakness + foot drop > shoulder/hip).
. Contralesional lower limb circumduction during swing-phase
. Contralesional upper limb flexed with reduced arm swing - Diplegic gait: cerebral palsy
. as per hemiplegic
. bilateral
. hip ADDuction spasm = lower limbs crossing midline at end of swing-phase (scissoring) - Parkinsonian: extrapyramidal disorders
. flexed posture
. small steps/festinating gait with reduced arm swing
. difficulty initiating/stopping gait
. several steps to turn on the spot - Ataxic gait: cerebellar
. broad-based, staggering gait
. ipsilesional veering - Stamping (sensory ataxic) gait: proprioceptive disorders
. swing-phase limb, high knee raise and foot slap on ground (inability to judge distance of foot to ground).
. gait pattern exaggerated when visual stimulus removed (eyes closed or room darkened).
. Romberg’s test positive - Neuropathic (high stepping) gait: foot drop pathologies.
. swing-phase limb, high knee raise (enables clearance of toes/foot from ground) and differentiating from “stamping gait” normal planting of foot on ground (proprioception in tact). - Myopathic (waddling) gait: myopathic disorders
. trunk leans contralesional side at swing-phase initiation (compensation of inability of hip ABDuctors to raise pelvis on ipsilesional side to enable foot lift off)
. trendelenburg positive - Antalgic: pain
. minimal time on weight bearing during stance-phase on ipsilesional limb - Normal
NEUROLOGY EXAM: Speech
- Orientation to time/place/person
- Dominant hand
- Free speech: describe cookie jar picture
DYSPHASIA
- RECEPTION severely impaired
- FLUENT with paraphasic errors
DYSPHASIA.
4. Comprehension
5. Repetition
6. Naming function
EXTRA-
7. Reading
8. Writing
- SLOW and NON-FLUENT
- GLOBAL
DYSARTHRIA.
9. Cerebellar
10. Muscular
11. Cough
12. Fatigueability
IF DYSARTHRIA -> lower cranial nerves
IF DYSPHONIA (husky voice) -> cough
- Orientation to time/place/person
- Dominant hand
- Free speech: describe cookie jar picture
IF DYSPHASIA -> consider other parietal features : limb strength and visual fields
- if RECEPTION severely impaired, consider written commands
- if FLUENT with paraphasic errors (phonemic, similar sounding words or semantic, similar category of word errors) nominal and receptive dysphasia.
DYSPHASIA.
4. Comprehension:
- close your eyes
- touch your left hand to your right ear
- touch your nose, then your chin, then your forehead
- point to the ceiling after you point to the floor
5. Repetition:
- blue sky
- the orchestra played and the audience applauded
- baby hippopotamus
- no ifs, ands or buts
6. Naming function: thumb, watch, nose
EXTRA-
7. Reading paragraph
8. Writing favourite food
- if SLOW and NON-FLUENT (hesitant), expressive dysphasia -> hemiparesis
- if GLOBAL (unable to comprehend or speak + hemiparesis), large lesion
DYSARTHRIA.
9. Cerebellar function: baby hippopotamus
10. Muscular function-
- Me me me = lip (CN 7)
- la la la = tongue (CN 12)
- ka ka ka = palate (CN9/10)
- eeee = vocal cord (recurrent laryngeal nerve)
11. Cough (bovine = recurrent laryngeal nerve palsy)
12. fatigueability (count backwards from 20 = MG)
IF DYSARTHRIA -> lower cranial nerves
- jaw jerk
- mouth insp: ulceration or local lesions
- extrapyramidal disease and myopathies
IF DYSPHONIA (husky voice) -> cough
- laryngeal disorder
- recurrent laryngeal nerve palsy
- focal dystonia
NEUROLOGY EXAM: Higher function
GEN INSP.
- Dominant hand
- Orientation TPP
- SPEECH
- Parietal lobes
DOMINANT AALF or Gerstmanns syndome
NON-DOMINANT (1)
BOTH (inattention 2)
CORTICAL SENSORY LOSS (NON-LOCALISING) (5)
- Temporal lobe (MEMORY, 2)
- Frontal lobe (4)
- Other
GEN INSP:
-diagnostic facies
-cranial nerve or limb lesions
- Dominant hand
- Orientation TPP
- SPEECH
-naming
-repetition
-comprehension
-reading
-writing - Parietal lobes
DOMINANT AALF or Gerstmanns syndome
-Acalculia
-Agraphia
-Left right disorientation
-Finger agnosia
NON-DOMINANT
-dressing apraxia
BOTH
-sensory inattention: tactile inattention/extinction
-visual inattention: visual field testing
CORTICAL SENSORY LOSS (NON-LOCALISING)
- graphasthesia (draw number on hand)
- two-point discrimination (0.6cm finger, 3cm hand/feet)
- joint position sense
- stereognosis
- constructional apraxia: clock face
- Temporal lobe (MEMORY)
- Short term recall: 3 unrelated items (green, yacht, rose)
- Long term memory: year world war 2 ended - Frontal lobe
- frontal release signs (grasp reflex, glabella tap)
- proverb interpretation: rolling stone gathers no moss
- fundoscopy (foster kennedy syndrome: frontal lobe tumour causing ipsilateral papilloedema and contralateral optic atrophy)
- smell - Other
Further testing: speech / cranial nerve / peripheral neurological examination
Upper limb drift (3)
Cerebellar lesion- up (hypotonia)
UMN weakness - down (muscle weakness)
Posterior column loss- any direction (loss of joint position sense)
Limb girdle muscular dystrophy
- proximal muscle weakness + atrophy limb girdles
- cardiomyopathy and arrythmias
Fascioscapular humeral muscular dystrophy
Pathophysiology: autosomal dominant, genetic.
-SUMMARY
-DDX (4)
SUMMARY:
. myopathic facies- facial muscle wasting (no ptosis or frontotemporal balding)
. facial weakness
. arms- wasting upper limb girdle, scapula winging, tone (normal/reduced), power (weakness upper limb girdle/upper arm), coordination/sensation equivocal.
. legs- bilateral foot drop (weak dorsiflexion), lower limb girdle muscles in tact
. abdo muscle weakness (lower > upper) BEEOVERS SIGN
DDX:
1. myotonic dystrophy
2. limb girdle muscular dystrophy (prox muscle weakness and wasting)
3. polymyositis (prox muscle weakness with tenderness)
4. inclusion body myositis (wrist/finger, anterior thigh and anterior tibialis
NEURO EXAM: shoulder girdle
Shoulder
-shrug (traps, CN11, C3-4)
-pull shoulder blades together (rhomboids, C4-5)
-push against wall (serratus anterior, C5-7)
-abduct from side (supraspinatus, C5-6)
-abduct from 15 degrees from side (deltoid, C5-6 and circumflex n.)
-abduct with internal rotation + adduct (pec major C6-T1)
-external rotate (infraspinatus, C5-6)
-internal rotate (teres major, C5-7)
-cough with palpation of sides (latt dorsi, C7-8)
Myotonic dystrophy
Pathophysiology:
- DM1: autosomal dominant, CTG repeat in DMPK gene Chr19.
- DM2: milder, abnormal expansion ZNF9 Chr 3.
Clinical features:
-general
-face
-speech
-neck
-hands
-key points (myotonia)
-other complications
-summary
-ddx
-Ix
Management:
. decrease myotonia (phenytoin, carbamazepine) but may increase weakness
. monitor and mx complications
. avoid general anaesthesia (sedatives/NMJ blocking drugs causing cardiorespiratory complications and delayed recovery, depolarising neuromuscular blocking agents (suxamethonium) can induce myotonia)
. genetic counselling
. anticipation (earlier age with each generation)
General: myopathic facies, pinprick from BSL testing, cognition.
Face: bilateral ptosis, wasting facial muscles with hollow temples and cheeks, frontal balding, smooth forehead and cataracts.
Speech: nasal, dysarthric ?dysphagia
Neck: SCM atrophy with weak neck flexion
Chest: gynecomastia, ?cardiac device, cardiomyopathy (AF, MVP)
Resp: aspiration ?NIV
Abdo: constipation, gallstones
Hands/feet: wasting and fasciculation, generalised symmetrical distal-predominant weakness (DM1, becomes proximal later but DM2, neck/finger flexor and hip girdle early).
-gait: high steppage, foot drop, trendelenberg positive
-tone/reflexes/coordination/sensation preserved till late
Key points:
-grip myotonia
-percussion myotonia
Other complications:
-cataracts
-diabetes (and thyroid dysfunction)
-dilated cardiomyopathy
-AF and cardiac arrythmias
-MVP
-dysphagia
-aspiration / NIV
-constipation
-gallstones
-testicular atrophy and infertility
SUMMARY: Steven is a gentleman who presents with difficulty using his arms. The most salient findings were that of generalized symmetrical distal-predominant muscle wasting and weakness, with evidence of myotonia and characteristic facial features in keeping with myotonic dystrophy.
DDx:
1. Hereditary inclusion body myositis
2. Limb girdle muscular dystrophy
Ix:
CK
EMG: dive-bomber effect, high frequency activity that varies (whining sound).
Genetic test
Causes of myotonia (3)
- Myotonic dystrophy
- Myotonia congenita (myotonic tongue and thenar eminence, recessive more severe)
- Paramyotonia congenita (episodic myotonia after cold exposure)
Causes of proximal muscle weakness (3)
- Myopathic
-inflammatory myopathies
. polymyositis (anti-synthetase, anti-Jo1, anti-MDA5)
. dermatomyositis
. inclusion body myositis (distal, asymmetric involvement hip flexors, quadriceps, tibialis anterior and weak ankle dorsiflexion and forearm flexors with grip weakness) = anti-cN1A
. immune mediated necrotising myopathy (Anti-HMGCR)
-acquired myopathies
. inflammatory
. paraneoplastic
. endocrine - thyroid, cushings
. drugs- steroids and alcohol
-hereditary muscular dystrophies- Becker’s / duchennes, myotonic dystrophy, limb girdle, fascioscapulohumeral - Neuromuscular junction disorder: myasthenia gravis
- Neurogenic:
-spinal muscular atrophy ie anterior horn cell damage (proximal muscle wasting and fasciculation, autosomal recessive Kugelberg-Welander disease)
-motor neurone dx
-polyradiculopathy
-non-length dependent peripheral neuropathy
Causes of myopathy (3)
-Hereditary
-Congenital
-Acquired PACE PODS
- Hereditary muscular dystrophy
- Congenital myopathies (rare)
- Acquired (PACE PODS)
P Polymyositis or dermatomyositis
A Alcohol
C Carcinoma
E Endocrine (hypothyroidism, hyperthyroidism, cushing’s syndrome, acromegaly, hypopituitarism)
P Periodic paralysis (hyperkalaemia, hypokalaemic or normokalaemic)
O Osteomalacia
D Drugs (clofibrate, chloroquine, steroids)
S Sarcoidosis
Causes of proximal myopathy + peripheral neuropathy (3)
- Alcohol
- CTD
- Paraneoplastic syndrome
Dermatomyositis
Limbs
Cutaneous features
OTHER
-hand exam
-bulbar dysfunciton
-respiratory exam
-steroid SE
-aetiology
SUMMARY
DDX
IX
Symmetrical proximal muscle weakness arms and legs
- gait aids
- wasting
- tenderness
Characteristic cutaneous features
- hands: gottrons papules (fingers, knee extensor surfaces), mechanic hands, nailfold capillaries
- eyes: heliotrope rash
- back: shawl sign (poikiloderma = hyperpigment + telangiectasia in sun exposed and unexposed areas)
OTHER-
A. Hand exam
- non-erosive polyarthrits and arthralgia (palpation and ROM, for active synovitis and deformity)
- power (grip, pincer, opposition)
- function (open lid, button)
B. Bulbar dysfunction - cachectic, speech, CN 9, 10, 12
C. Respiratory exam
- pulmonary fibrosis
- pulmonary HTN]
D. Steroid SE
- cushingoid appearance
- bruising
- skin atrophy
- cataract
- proximal myopathy
E. Aetiology- malignancy
SUMMARY- pt presented with weakness/hand lesions asked to examine his upper limbs. Most salient findings were that of symmetrical bilateral proximal muscle weakness in upper and lower limbs associated with characteristic cutaneous features of gottrens papules, mechanic hands, heliotrope rash and shawl sign suggestive of dermatomyositis with no active synovitis in the small joints of the hands. This was complicated by evidence of pulmonary fibrosis and pulmonary HTN, bulbar dysfunction and features of corticosteroid excess. I was unable to identify underlying malignancy.
DDX- proximal weakness ddx
1. myopathic
2. neuromuscular junction disorder
3. neurogenic
Ix-
CK
Myositis Ab: anti-Jo1, anti-Mi2, anti-TIF1y, anti-MDA5
Muscle biopsy
Assess complications
-ILD: HRCT, RFT
-Pulm HTN: TTE
-Age appropriate malignancy screen
-Steroid SE: BMD, HbA1c, Lipids
Myositis antibodies
-polymyositis
-dermatomyositis
-IBM
-immune mediated necrotising myopathy
Polymyositis
-anti-synthetase
-anti-Jo1
Dermatomyositis
-Anti-Jo1
-Anti Mi2
-anti-MDA5 (rapid lung disease)
IBM
-anti-cN1A
Immune mediated necrotising myopathy
-Anti-HMGCR
LIST OF MYOTOMES
UPPER LIMB
C1/2 neck flex/ext
C3 neck lateral flexion
C4 shoulder shrug
C5 shoulder abduction
C6 elbow flex, wrist ext
C7 elbow ext, wrist flex
C8 finger flexion
T1 finger abduction
LOWER LIMB
L2 hip flexion
L3 knee extension
L4 ankle dorsiflexion
L5 great toe-extension
S1 ankle plantar flexion
ankle eversion
hip extension
S2 knee flexion
S3-4 anal wink
Clinical reflexes
Achilles reflex = S1, S2 “buckle my shoe”
Patellar reflex = L3, L4 “kick the door”
Biceps and brachioradialis reflex = C5, C6 “pick up sticks”
Triceps = C7, C8 “lay them straight”
Cremasteric reflex = L1, L2 “Testicles move”
Anal wink reflex = S3, S4 “winks galore”
DERMATOMES
UL
LL
See clinical exam document
Speech patterns.
DYSARTHRIA (patterns)
- Cerebellar
- pseudo-bulbar (UMN CN 9-12)
- bulbar (LMN CN 9-12)
- MND
- facial nerve/muscle weakness (UMN or LMN)
- extrapyramidal
DYSPHASIA
- Fluent dysphasia
- Non-fluent dysphasia
Paraphasic errors of speech:
- Phonetic
- Semantic
- Neologism
Aphasia subtype:
- Expressive (broca’s)
- Receptive (wernicke’s)
- Conductive (arcuate fasciculus)
- Anomic (angular gyrus, encephalopathy, left hemisphere brain)
- Global (left MCA)
DYSARTHRIA (patterns)
- Cerebellar: staccato, irregular, scanning (jerky speech that is broken into separate syllables with syllable stress (explosive/loud))
- pseudo-bulbar (UMN CN 9-12): slow, hesitant, hollow sounding speech with harsh strained voice (squeezing words out from tight lips)
- bulbar (LMN CN 9-12): nasal (air escaping into nasopharynx) with imprecise articulation
- MND can have mixed pseudo-bulbar and bulbar palsy.
- facial nerve/muscle weakness (UMN or LMN): slurred
- extrapyramidal: monotonous.
DYSPHASIA
Fluent dysphasia (speech flows freely, prosody maintained, normal length and structure).
Non-fluent dysphasia (effortful, no prosody, short sentences lacking filler words and prepositions.
Paraphasic errors of speech:
- Phonetic paraphasia: consonant subbed (fell v tell), similar sound
- Semantic paraphasia: associated word subbed (cat v dog)
- Neologism: non-existent word subbed (cookie v ratatat)
Aphasia subtype:
Expressive (broca’s): non-fluent with impaired repetition and naming, comprehension preserved.
Receptive (wernicke’s): fluent, PARAPHASIC, impaired repetition, naming and comprehension
Conductive (arcuate fasciculus): impaired repetition and naming.
Anomic (angular gyrus, encephalopathy, left hemisphere brain): impaired naming.
Global (left MCA): impaired.
Brachial plexus lesion
Complete
Upper trunk (C5/C6) Erb-Duchenne
Lower trunk (C8/T1) Klumpkes
Complete
- LMN signs
- sensory loss
- Horner’s syndrome
Upper trunk (C5/C6) Erb-Duchenne
- waiter tip
- decreased movement:
. shoulder
. elbow flexion
- sensory loss C5/C6 (lateral arm, forearm, thumb)
Lower trunk (C8/T1) Klumpkes
- claw hand (all lumbricals)
- sensory loss C8/T1 ulnar
- Horners
Ulnar neuropathy
C8-T1
Wasting
Weakness
Sensory loss
Other ddx for wasting of small muscle of hand
Wasting: small muscles of hand
- hypothenar (thenar spared)
- ulnar claw (medial 2 lumbricals, hand less clawed in higher lesions)
- wasting medial forearm
- weak finger ABD / ADD and thumb ADD
- sensory loss: little + medial half ring finger
- scars (fracture, dislocation) and OA at elbow
DDX wasting small muscles of hand:
-anterior horn cell disease (poliomyelitis)
-radiculopathy (trauma, prolapse disc)
-plexopathy (brachial plexus, pancoast tumour, cervical rib)
-peripheral nerve lesion
-myopathy
-muscle disuse atrophy
Median neuropathy
C6-T1
PROXIMAL (pronator teres, humerus)
ANTERIOR INTEROSSEOUS (pure motor)
CARPAL TUNNEL
PROXIMAL (humerus, pronator teres syndrome)
- hand of benediction, unable to flex lateral 2 fingers on making fist
- weak flexion / pronation forearm
- sensory: palmar cutaneous br thenar.
ANTERIOR INTEROSSEOUS SYNDROME:
- pure motor
- weak opposition
- weak pronation with flexed elbow (pronator quadratus)
DISTAL (carpal tunnel)
- ape hand, thenar wasting
- weak LOAF (lumbricals, OPP, ABD, FLEX)
- hand of benediction on making fist
- paraesthesia lateral 3 1/2 digits
- phalens (wrist flex), or tinel (tapping tingling)
- scar from carpal tunnel release, diabetes, hypothyroid, acromegaly, RA/ OA.
Radial neuropathy
C5-8
Features
AXILLA
SPIRAL GROOVE
PROXIMAL FOREARM
WRIST
Wrist drop
Weak wrist EXT
Sparing intrinsic muscles of hand (if wrist passively EXT)
Impaired grip strength
Sensory loss first dorsal interossi
Scars elbow (fracture, dislocation), note if using crutches.
AXILLA
- weak elb EXT, forearm SUP, wrist/finger EXT
- sensory loss post-forearm, 1st dorsal interossi
- decreased reflexes (triceps, brachioradialis)
SPIRAL GROOVE
- weak forearm SUP, wrist/finger EXT
- sensory loss 1st dorsal interossi
PROXIMAL FOREARM
- weak wrist / finger EXT
- no sensory loss
WRIST
-no weakness
-sensory loss 1st dorsal interossi
DDX wrist drop (5)
Posterior interosseous nerve lesion
- weak finger ext
Peripheral neuropathy
C7/8 nerve root or plexus (weak wrist extension, finger ext)
- cervical spondylosis
- brachial plexus injury
Motor neuron disease (no sensory)
Corticospinal tract (hemiparesis)
Common peroneal neuropathy
Gait:
- high steppage gait
- foot drop
- unable to heel walk
Power:
- weak eversion / dorsiflexion
Reflexes intact
Sensory:
- lateral calf / foot dorsum
Other:
-fibula scar / compression around fibular neck
-wasting anterolateral compartment calf
DDx foot drop
Common peroneal nerve palsy
Peripheral neuropathy (CMT)
Sciatic nerve palsy: inversion and plantar flexion weak
L5 nerve root lesion: inversion weak (disc prolapse)
Lumbosacral plexopathy
Motor neurone disease (no sensory deficit)
Distal myopathy
L5 nerve root lesion
Gait:
- high steppage
- foot drop
- unable to heel walk
Weak:
- hip ABD
- knee flexion
- plantar flexion / dorsiflexion
- ankle INV / EVER
Sensory: dorsum of foot, lateral calf up to lateral thigh.
Sciatic nerve lesion
Gait:
- high steppage gait
- foot drop
Weak:
- knee flexion
- plantar flexion / dorsiflexion
- ankle INV / EVER
Reflexes:
- preserved knee jerk
- loss of ankle jerk and plantar
Sensory:
- below knee, tibial (posterolateral leg, lateral foot, sole of foot) and common peroneal nerve (lateral leg, dorsum foot)
Charcot Marie Tooth
(Polyneuropathy)
Hereditary sensory and motor neuropathy
Autosomal dominant
NO UMN SIGNS
INSP
Gait
Weakness
Reflexes
Sensation
SUMMARY
INSP:
- gait aids, shoe/ankle supports
- pes cavus
- clawing toes
- distal muscle atrophy:
-anterolateral compartment with preserved thigh musculature “inverted champagne bottle”
-peroneal muscle atrophy
Gait:
- ataxic
- proprioception: broad based, stamping, rhombergs positive
- peripheral neuropathy: foot drop, high stepping, heel walk impaired
Weakness: symmetrical
Reflexes: absent
Sensation: symmetric stocking distribution (variable)
- vibration
- proprioception
- pain/temp usually normal
Other: thickened nerves, optic atrophy (argyll robertson pupil)
SUMMARY: symmetrical distal predominant weakness with lower motor neuron signs, likely long standing given presence of pes cavus and severe atrophy of distal muscles suggesting hereditary neuropathy such as CMT.
DDx: symmetric distal weakness with LMN signs (4)
Peripheral neuropathy
Myotonic dystrophy
Anterior horn cell disease (polio, SMA, PMA)
Inclusion body myositis (no sensory changes)
—
NMJ (no wasting, reflexes preserved)
Myopathy (usually proximal, reflexes and sensation preserved)
DDx: sensorimotor neuropathy
Metabolic (6)
Immune mediated (more motor predominant, not length dependent ie prox = distal weakness) (2)
Paraneoplastic (2)
CTD / vasculitis
Idiopathic
Metabolic
- diabetes
- uraemia (CKD)
- thyroid
- B12 deficiency
- drugs
- eTOH
Immune mediated (more motor predominant, not length dependent ie prox = distal weakness)
- gullain barre
- CIDP
Paraneoplastic
- tumor
- paraproteinemia
CTD / vasculitis
Idiopathic
DDx Motor neuropathy (8).
GBS / CIDP
CMT
MULTIFOCAL MOTOR NEUROPATHY
Malignancy
Porphyria
Lead poisoning
Can consider diabetes and dapsone.
Chronic inflammatory demyelinating polyneuropathy (CIDP)
LMN signs
INSP:
-scar
-wasting
-fasciculations
Gait:
- high stepping gait (foot drop, distal weakness)
- waddling gait (proximal weakness)
- ataxia, broad based and stamping (sensory)
- rhombergs
Tone:
- decreased or equal
Power:
- symmetric motor deficit (non-length dependent distribution affecting prox = distal muscles)
Reflexes:
- decreased
Sensory:
- large fibre sensory loss
- proprioception
- vibration
- worse distal
- pain/temp loss less common
SUMMARY: non-length dependent (distal predominant) sensorimotor neuropathy
DDx non-length dependent sensorimotor neuropathy
CIDP
Subacute combined degeneration of the cord (B12 level, holotranscobalamin, methylmalonic acid)
Anterior horn cell disease
NMJ
Myopathy
DDX sensory neuropathy
Complete UL / LL exam, autonomic dysfunction (postural BP)
Metabolic
- diabetes
- uraemia (CKD)
- hypothyroid
- eTOH
- drugs
Paraneoplastic
Vasculitic
Sjogrens syndrome
Amyloidosis
vit b12 def, vit b6 excess
Electromyography (EMG)
- spontaneous activity: fasciculation, fibrillation, myotonic
- voluntary activity: amplitude, motor unit recruitment
FOR
Primary nerve disease
Primary muscle disease
Primary nerve disease
- Spontaneous activity: increased fasciculation and fibrillation due to denervation
- Voluntary activity: increased amplitude (surviving axons innervate more motor fibres each), decreased recruitment of motor units.
Primary muscle disease
- Spontaneous activity: myotonic discharges
- Voluntary activity: decreased amplitude of motor unit action potentials, increased motor units activated (early recruitment)
Neuro EXAM: Eye
GEN INSP
CORNEA
SCLERA
PTOSIS
EXOPTHALMOS
EYELIDS
LID LAG
ORBITS
NEUROLOGICAL EXAM
- ACUITY
- FIELDS
- FUNDI
- PUPILS
- EYE MOVEMENTS:
. III, IV, VI nerves
. Nystagmus
. Saccades
Corneal reflex (V)
OTHER
GEN INSP
- diagnostic facies
CORNEA
- arcus senilis
- band keratopathy
- kayser-fleischer rings
SCLERA
- jaundice
- pallor
- injection
PTOSIS ?miosis / horners.
EXOPTHALMOS
EYELIDS
- xanthelasma
LID LAG
ORBITS
- palpate: tenderness, brow for anhidrosis (horner’s)
- auscultate bruit
NEUROLOGICAL EXAM
- ACUITY: eye chart
- FIELDS:
. red hatpin: desat = optic neuritis
. red hatpin confrontation
. central vision for blindspot - FUNDI: cornea, lens, humour, disc colour and state of cup, retina- vessels, exudates, haemorrhages, pigmentation
- PUPILS:
. shape, size, symmetry
. light reflex (direct and consensual)
. RAPD
. accommodation - EYE MOVEMENTS:
. III, IV, VI nerves- movement, diplopia
+ gaze palsies (e.g. supraneuclear lesion)
+ fatigueability (myasthenia)
. Nystagmus
+ cerebellar (unilateral or bilateral, slow phase to centre, fast phase in direction of gaze)
+ peripheral vestibular (HINTS, corrective saccade if abnormal vestibular ocular reflex due to loss of vestibular apparatus or nerve)
+ monocular (weakness opposite eye, neuropathy - III, IV, VI, INO)
+ vertical (central)
+ congenital
+ multidirectional (cerebellar, drug toxocity e.g. anticonvulsants phenytoin)
. Saccades (overshoot or undershoot = cerebellar)
Corneal reflex (V)
OTHER
- other cranial nerves
- long-tract signs
- urine analysis (diabetes)
CN5
pain temp
light touch
jaw jerk
light touch lost- pontine
pain temp lost - medullary, upper cervical
jaw jerk (pseudobulbar palsy, increased)
Tabes dorsalis
DORSALIS
Demyelinating condition affecting dorsal column of spinal cord.
Dorsal column degeneration
Orthopaedic pain (Charcot joints)
Reflexes decreased (deep tendon)
Shooting pain
Argyle-Robertson pupil (impaired light reflex, intact accommodation)
Locomotor ataxia
Impaired proprioception, high stepping gait
Syphilis
CN 8
- Rinne and Weber (256Hz)
- ddx conductive deafness
- ddx sensorineural deafness
- Rinne (mastoid process):
- Conductive deafness = Bone > Air
- Sensorineural deafness = Air > Bone (normal) confirmed on Weber - Weber (forehead):
- Conductive deafness = louder in abnormal ear
- Sensorineural deafness = louder in normal ear
Causes of conductive deafness:
-wax
-otitis media
-otosclerosis
-paget’s disease of bone
Causes of sensorineural deafness:
-presbycusis
-trauma
-toxin (aspirin, alcohol, streptomycin)
-infection (congenital rubella , syphilis)
- tumour
- brain stem lesion
- vascular disease internal auditory artery
Nystagmus
-cerebellar
-peripheral vestibular
-monocular
-vertical
-congenital
-multidirectional
Cerebellar nystagmus: unilateral or bilateral.
-drift, pursuit / saccades
-associated with dysarthria and limb ataxia
Peripheral vestibular nystagmus: unidirectional, horizontal, worse in direction of fast phase.
-vestibulo-ocular reflex (eyes fix on bridge of nose when head moved)
Monocular: weakness opposite eye, CN palsy or INO
Vertical nystagmus: central disorder
Congenital nystagmus: dramatic
Multidirectional nystagmus: generalised cerebellar dysfunction or drug toxicity (anticonvulsants).
One and a half syndrome
Location: abducens nucleus + MLF
e.g. left sided lesion
Lateral medullary syndrome
-posterior inferior cerebellar artery (PICA)
-ipsilateral
-contralateral
-CN 9 10 11 (PIKA can’t CHEW) = dysphagia
Lateral pontine syndrome = CN 5 7 8 (fACIAl muscle weakness) = AICA = anterior inferior cerebellar artery
Ipsilateral (face)
-horners
-diplopia
-nystagmus
-pain/temp
-ataxia (limbs)
Contralateral (limbs)
-pain/temp
CN 1 - olfactory (anosmia) (5)
Infection: URTI / COVID-19, meningitis
Tumour: Meningioma of olfactory groove, ethmoid tumours
Trauma: Head trauma (cribiform plate fracture)
Hydrocephalus
Congenital - hypogonadotrophic hypogonadism
CN2 - optic nerve
- light reflex: CN2 (no cortical involvement)
- accommodation reflex: cortical origin with parasympathetic fibres via CN3.
DDx absent light reflex, intact accommodation
DDx absent convergence, intact light
DDx absent light, intact accommodation
- midbrain (argyll-robertson pupil) = syphilis
- ciliary ganglion (adie’s pupil, on eye bigger than the other)= viral or bacterial infection
- parinauds syndrome
- bilateral anterior visual pathway lesion (bilateral afferent pupil deficits)
DDx absent convergence, intact light
- cortical lesion
- midbrain lesion (rare)
Pupil abnormalities
Constriction (6)
Dilation (6)
Constriction-
- horner’s syndrome
- argyll robertson pupil
- pontine lesion (bilateral, reactive to light)
- narcotics (opiates)
- pilocarpine drops
- old age
Dilation-
- adies pupil
- third nerve lesion
- mydriatrics, atropine poisoning or cocaine
- iridectomy, lens implant, iritis
- post-trauma, deep coma, cerebral death
- congenital
Adies syndrome
Lesion efferent parasympathetic pathway (CN 3 -> ciliary ganglion)
Dilated pupil
Decreased light response (direct and consensual)
Slow or incomplete accommodation, slow dilation after
Decreased tendon reflexes
Impaired sweating
Young women
Argyll robertson pupil
Lesion of iridodilator fibres in midbrain
1. syphilis
2. diabetes
3. alcoholic midbrain degeneration
4. other midbrain lesions
Signs
-small, irregular, unequal pupil
-no light response
-accommodation response intact
-decreased reflexes, if tabes associated
DDx pappiloedema
Space occupying lesion
Hydrocephalus
-obstructive
-communicating (increased formation or decreased absorption)
Idiopathic intracranial hypertension
-idiopathic
-OCP
-addisons
-drugs (nitrofurantoin, tetracycline, vitamin A, steroids)
-lateral sinus thrombosis
-head trauma
HTN
Central retinal vein thrombosis
Cerebral venous sinus thrombosis
High CSF protein (GBS)
DDx optic atrophy
Chronic papilloedema or optic neuritis
Glaucoma
Ischemia
Familial - retinitis pigmentosa, Leber’s disease (mitochondrial), Freidrich’s ataxia
DDx optic neuropathy
MS
Toxic - ethambutol, chloroquine, nicotine, alcohol
Metabolic - vit B12
Ischemia - DM, temporal arteritis, atheroma
Familial- LHON (Lebers)
Infective - infectious mono (glandular fever, EBV)
DDx cataract
Age
Endocrine - diabetes, steroids
Hereditary or congenital- dystrophia myotonica
Ocular disease - glaucoma
Irradiation
Trauma
DDx ptosis
Normal pupils-
-age, fatigue, congenital
-myotonic dystrophy
-myasthenia gravis
-FSH, ocular myopathy (mitochondrial)
-thyrotoxic myopathy
-botulism, snake bite
Constricted pupils-
-horner’s syndrome
-tabes dorsalis
Dilated pupils-
-third nerve lesion
Supranuclear gaze palsy
Progressive supranuclear palsy
- loss of vertical down gaze / up gaze and later horizontal gaze
- saccades impaired
- associated : pseudobulbar palsy, long-tract sings, EPS, dementia and neck rigidity
Distinguished from CN palsy (3,4,6) ?
Loss vertical and / or downward gaze
Distinguished from CN palsy 3 , 4 , 6
- both eyes affected
- pupils unequal
- no diplopia
- reflex eye movements in tact (flexing / extending neck)
Parinaud (dorsal midbrain) syndrome
-Clinical features (4)
-Causes (central, peripheral)
Clinical features-
1. supranuclear gaze palsy
2. convergence-retraction nystagmus
3. lid retraction
4. light-near dissociation
Causes-
-central: pinealoma, MS, vascular lesions
-peripheral: trauma, diabetes, vascular, idiopathic, raised ICP
CN 5 palsy
Central
Peripheral
Trigeminal ganglion
Cavernous sinus
Other
Central: vascular, tumour, MS, syringobulbia
. Pons = light touch
. Medulla = pain
Peripheral (posterior fossa): aneurysm, tumour, chronic meningitis
Trigeminal ganglion (petrous temporal bone): meningioma, fracture of middle fossa
. sensory loss all 3 divisions
. post-ganglionic lesion if sensory loss 1 division
Cavernous sinus (associated CN 3, 4, 6 palsy): aneurysm, thrombosis, tumour
Other: sjogrens, SLE, toxins, idiopathic
CN 7
UMN
LMN
Bilateral LMN
UMN- vascular, tumour
LMN- pontine (associated 5, 6): vascular, tumour, syringobulbia, MS
-posterior fossa (acoustic neuroma, meningioma)
-petrous temporal bone (bell’s palsy, ramsay hunt syndrome, otitis media, fracture)
-parotid (tumour, sarcoid)
Bilateral LMN facial weakness- GBS, bilateral parotid disease (sarcoidosis), mononeuritis multiplex, myopathy and NMJ
CN 9 / 10
Central
Peripheral
Central: vascular (lateral medullary infarct - vertebral artery or PICA), tumour, syringobulbia, MND (vagus)
Peripheral (posterior fossa): aneurysm, tumour, chronic meningitis, GBS (vagus)
Neuro exam: peripheral neuropathy
DDx peripheral neuropathy
IX:
Drug and alcohol hx
FBC, MCV, UeC (urea and electrolytes), LFTs (GGT), VitB12, folate, glucose, TFT, autoimmune screen, immunoglobulins, hepatitis screen, HIV screen, lyme serology, syphilis serology
Urine: dip for glucose, protein, bence-jones protein
Imaging: CXR
LP and CSF: protein and CSF virology
NCS:
-demyelinating (diabetes, paraprotein, CMT, CIDP)
. decreased velocity, increased latency, normal amplitude
-Axonal (diabetes, toxins, metabolic, paraneoplastic)
. decreased Amplitude, normal velocity
FINDINGS:
General: walking aids, orthotics, prosthesis, insulin pen
Neurological:
-bilateral symmetrical length dependent peripheral sensory and/or motor neuropathy in glove and stocking distribution
-wasting, weakness, areflexia
Extra:
-finger-prick, cataracts, ulcers, charcot’s joints, callus (diabetes mellitus)
-clawing of toes, pes cavus (CMT)
-amiodarone facies
-anaemia (b12 deficiency)
-eTOH abuse
-arthritis / rash (vasculitis).
DDX:
Drugs and toxins -
-amiodarone, gold, isoniazid, metronidazole, vincristine, phenytoin, nitrofurantoin, cisplatinum
- Vit B6 excess, Vit B12 deficiency
- alcohol (+/- thiamine deficiency)
- heavy metals
Metabolic
- diabetes
- CKD
- hypothyroidism
- porphyria
Infiltration - amyloid
Immune-mediated - GBS
Tumour- lung ca
Idiopathic
CTD or vasculitis- SLE, PAN
Hereditary
DDX painful peripheral neuropathy
diabetes, alcohol
vit b12 or b1 def
carcinoma
porphyria
arsenic or thallium poisoning
hereditary
DDx thickened nerves
Acromegaly
Hereditary motor and sensory neuropathy (CMT)
CIDP
Infiltration - amyloidosis, leprosy, others (sarcoidosis, neurofibromatosis)
DDx fasciculations
Benign idiopathic
MND
Motor root compression
Malignant neuropathy
Spinal muscular atrophy / bulbospinal muscular atrophy
Any motor neuropathy (less commonly)
Causes of carpal tunnel syndrome
Idiopathic
Arthropathy (RA)
Endocrine (acromegaly, hypothyroidism)
Pregnancy
Trauma and overuse
DDx chorea (corpus striatum)
- hemiballismus (subthalmic nucleus contralateral)
- athetosis (putamen)
Huntingtons disease
Sydenham’s chorea (rheumatic fever)
Wilsons disease
Drugs (OCP, L-dopa, phenytoin)
Vasculitis or CTD (SLE)
Thyrotoxicosis
Polcythemia or hyperviscosity
Viral encephalitis
Medical Research Council (MRC) power grade
0 no muscle contraction
1 flicker or trace
2 movement with gravity eliminated
3 movement against gravity but not resistance
4 movement against gravity and some resistance
5 movement against gravity with full resistance
DDx: cerebellar syndrome (7)
Midline vermis - truncal ataxia, ataxic gait.
Cerebellar hemispheres - ipsilateral dysmetria, dysdiadochokinesis, intention tremor and nystagmus toward lesion (fast phase)
MS
Alcohol
Space occupying lesion (posterior-fossa)
Vascular lesion (brainstem)
Inherited (freidrichs, SCA)
Paraneoplastic syndrome
Drugs (phenytoin, lithium, carbamazepine)
MSA
Metabolic: hypothyroid, b12 def, Wilson’s , coeliac??
Infection: HIV, toxoplasmosis
Inflammation: GBS (Miller fisher)
Neuro exam: cerebellar
Face:
-nystagmus
-saccades (overshoot, correction)
-dysarthria (staccato)
Truncal ataxia.
UL:
-hypotonia
-dysmetria
-dysdiadochokinesis
Gait:
-rhombergs negative
-ataxic gait (heel-toe impaired)
-veering to side of lesion
LL:
-hypotonia
-dysmetria with heel-shin
Stroke:
-hemiparesis
-investigations
-management
Hemiparesis: pyramidal pattern of weakness with UMN features.
Investigations: CT/MRI, BP, BSL, lipid profile, ECG, telemetry, echo, carotid artery doppler
IF YOUNG- thrombophilia screen, echo with bubble study ?PFO.
Management:
A. ACUTE.
-thrombolysis
-DAPT with aspirin lifelong
-NIHSS stroke scale
B. Long-term.
-rehab
-psychology
-nutrition
-atrial fibrillation: anticoagulation
CN4 palsy (trochlear nerve)
Weakness of downward movement
Vertical diplopia
SIN = Superior oblique INtorts the eye
Myasthenia gravis
Bilateral ptosis
Fatigueability
-eye
-speech
-chicken wing (arm)
Thymectomy scar
Miller Fisher syndrome (3)
-AIDP = acute inflammatory demyelinating polyradiculopathy
-Lower cranial and facial nerves, not usually motor weakness.
-anti-GQ1b ab (serum)
- ataxia
- areflexia
- opthalmoplegia
Wilsons disease
Wilsons disease:
-wing beating tremor
-stiffness
-coordination deficit
-kayser fleisher rings
-mood changes
-abdo pain, dark urine / light stool + jaundice.
Hereditary spastic paraparesis.
- axonal degeneration in distal long tracts with no primary demyelination.
-inheritance
-uncomplicated
-complicated
-tx (non-pharm, pharm for spasticity and bladder)
Inheritance: : X-linked, AD and AR.
Uncomplicated: hypertonic urinary bladder disturbance, and mild loss lower extremity vibration.
Complicated: hypertonic urinary bladder, mild loss lower extremity vibration, and other system / neurologic finding (ataxia, seizures, intellectual disability/dementia, muscle atrophy, extrapyramidal disturbance, peripheral neuropathy).
Tx: symptom mx.
. improve fitness (cardiac-daily exercise regimen, muscle strength and gait) to reduce spasticity
. occupational therapy, assisted walking devices, ankle-foot orthotics
. drugs to tx spasticity (baclofen, tizanidine, dantrolene, botox injections) and urinary urgency (oxybutynin (anticholinergic/antimuscarinic), solifenacin (antimuscarinic), mirabegron (b3 adrenoreceptor agonist), or botox injections).
DDx Visual field defects
- Optic nerve lesion
- Optic chiasm lesion
- Optic tract
- Optic radiation lesion
-Temporal lobe lesion
-Parietal lobe lesion - Visual cortex or optic radiation
Causes homonoymous hemianopia
Causes optic nerve lesion
Causes of bitemporal hemianopia
- Optic nerve lesion: partial or complete visual loss on side of lesion
- Optic chiasm lesion: bitemporal hemianopia (both temporal fields)
- Optic tract: homonymous hemianopia
- Optic radiation lesion: homonymous field defect - lobe dependent.
-Temporal lobe lesion: superior homonymous quadrantanopia
-Parietal lobe lesion: inferior homonymous quadrantanopia - Visual cortex or optic radiation: homonymous hemianopia
Causes homonoymous hemianopia
-vascular (stroke)
-trauma
-tumour
-infection (encephalitis)
-demyelination (MS)
Causes optic nerve lesion
-vascular (acute optic artery ischemia)
-trauma
-demyelination (MS, optic neuritis)
-compression: raised intraocular or intracranial pressure
-metabolic: VitB12 deficiency, diabetes, etOH
-inherited conditions (Leber’s optic neuropathy)
Causes of bitemporal hemianopia
-optic chiasm lesion
-pituitary fossa tumour
-craniopharyngioma
-suprasellar meningioma
Ddx LMN pathology
Muscle
NMJ
Peripheral nerve
Plexus
Nerve root
Anterior horn cell
DDx MND
MMNCB (LMN only)
UMN + LMN
-mixed pathology (cervical myelopathy + peripheral neuropathy)
-cauda equina
-freidrichs ataxia
-subacute combined degeneration of the cord
Hereditary muscular dystrophy
- X linked recessive
- more common in males
- DMD, Beckers
- Clinical
- Mx
Duchenne muscular dystrophy
- no dystrophin
Becket muscular dystrophy
- misshapen dystrophin
- mild age 10-20
Clinical features: symmetric proximal lower limb weakness more than distal.
Gait.
-waddle (wheelchair)
- calf hypertrophy
- gowers sign (leaning forward)
Other: weak diaphragm (resp failure), scoliosis, dilated cardiomyopathy and arrhythmias.
High CK.
Mx: steroids slow progression.
