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??