Neurology Flashcards
Clinical features of bulbar palsy
This is a LMN problem of CNs X, XI and XII
speech:
unilateral - raspy
bilateral - nasal
tongue
- fasciculations
- wasting
reflexes
gag - decreased
jaw jerk - normal (LMN and CN V not involved)
Clinical features of pseudobulbar palsy
Syndrome: bilateral UMN lesions of CN IX, X, XII Increased gag reflex Brisk jaw jerk Spastic tongue - cannot protrude Absent palatal movement Labile affect Dysarthria - spastic Can also have bilateral UMN long tract signs
Causes: bilateral CVA, MS, MND, head injury, tumour
Clinical features of 4th nerve palsy
Innervates superior oblique - moves eye down and in
Patient reports diplopia, worse in down gaze (ie walking down stairs, reading)
Head tilt to opposite side
Causes: idiopathic, head injury, small vessel disease, aneursym, tumour, MS
Clinical features of lateral medullary syndrome
Sympathetic tract - Horners
Nucleus ambiguus - ipsilateral IX/X weakness, hoarseness, dysphagia, decreased gag reflex
Spinal trigeminal nucleus and tract - ipsilateral loss of pain and temp in face
Spinothalamic tract - contralateral loss of pain and temp in body
Cerebellar peduncle - ipsilateral cerebella signs (ataxia, dysmetria, dysdiadokokinesis)
Vestibular nuclei - nystagmus, vomiting, vertigo
Abducens/6th Nerve Palsy
Abduction deficit
Slowed ipsilateral saccades
Nystagmus
NUCLEAR:
Metabolic (Wernickes), Traumatic, Congenital, Demyelinating, Ischaemic, Neoplastic.
FASCICULAR:
Demyelination, Infarction, Neoplasm, Traumatic.
SUBARACHNOID SPACE LESION:
Aneurysm, Carcinomatous meningitis, Damage post procedure, Inflammatory: vasculitis, sarcoidosis, systemic lupus erythematosis. Infectious: Syphilis, Tb, Cryptococcal, HIV-CMV.
PETROUS APEX LESION:
Neoplasm, infection or inflammatory, thrombosis of inferior petrosal sinus, basilar skull fracture.
CAVERNOUS SINUS LESION:
Cavernous sinus thrombosis or fistula, neoplasm, Ischaemia, inflammatory or infectious, Internal carotid artery aneurysm or dissection.
ORBITAL LESION:
Neoplastic, inflammatory, infectious, traumatic.
Occulomotor/3rd Nerve Palsy
SIGNS Down and out -infraducted and abducted Partial or complete ptosis Aniscoria Accomodation deficit
NUCLEUS:
Complete ipsilateral 3rd nerve & contralateral SR weakness.
Causes: infarction, haemorrhage, tumour.
FASCICLES:
Contralateral hemiparesis; contralateral tremor; Ipsilateral ataxia.
Causes: infarction, haemorrhage, tumour, demyelination.
SUBARACHNOID SPACE:
Typically isolated. Headaches, orbital pain.
Causes: aneurysm of ICA/Post Com A/Basilar/PCA. Microvascular ischaemia, tumour, Meningitis, herniation, trauma.
CAVERNOUS SINUS:
CrN IV, V1, V2, VI. Oculosymoathetic dysfunction (Horner’s).
Causes: tumour, inflammation, carotid aneurysm, ischaemic, thrombosis, AV fistula.
ORBITAL APEX:
Proptosis. Visual loss. CrN IV, VI, V1, V2. Occulosympathetic dysfunction. Pain.
Causes: Trauma, tumour, inflammation, infection (fungal).
Internuclear opthalmoplegia clinical signs
Impaired horizontal eye movements with weak adduction of affected eye and abduction nystagmus of contralateral eye
Neurological facies
MYOPATHIC
- unwrinkled, expressionless, long, lean, gaping lips, partially open mouth, ptosis.
MYOTONIC
- frontal balding
- hatchet facies (atrophy of temporalis muscle)
- ptosis and drooping of mouth
- wasting of sternocleidomastoid
PARKINSONIAN
Charcot marie tooth
Present under age 30 with distal leg weakness
Autosomal dominant
Overall pattern:
Hereditary chronic sensory motor polyneuropathy
Observation:
Champagne legs - atrophy of muscles below knees
Pes cavus - high arch feet
Claw toes
Bilateral foot drop
Tone: Normal
Strength: Distal muscle weakness with foot drop
Reflexes: Absent or diminished
Sensation:
No sensory complaints but have decreased vibration and proprioception
Pain and temperature sensation usually intact
Sensory ataxia - rhombergs positive due to impaired proprioception
Gait:
High stepping
Sensory ataxia
Upper limb: weakness later then lower limb, decreased reflexes
Other features:
Essential tremor
Sensorineural hearing loss in 5%
Scolosis
Thickened, palpable nerves
Other information you would ask for:
Family history
Nerve conduction studies: most common type is demyelinating
CIDP (chronic inflammatory demyelinating polyneuropathy)
Chronic - gradual over months (symptoms greater then 8 weeks for diagnosis)
Pattern: chronic symmetrical motor and sensory polyneuropathy (demyelinating)
Observation:
Weakness more prominant then wasting
Can affect upper and lower limb
Occasional cranial nerve involvement (affecting 7 and 3/4/6)
Occasional fasiculations
Tone: normal or decreased
Strength: distal and proximal muscle weakness, can involve lower and upper limbs, usually symmetrical
Reflexes: absent
Sensory: reduced proprioception and vibration, pain and temperature usually intact
Compared to weakness (proximal and distal) sensory loss is glove and stocking distribution
Gait:
Can be either high stepping due to distal weakness, or slapping due to loss of proprioception
Sensory ataxia due to loss of proprioception
Rhombergs positive
Tests:
Lumbar puncture with albuminocytological dissociation
Test serum electrophoresis - 25% have MGUS
Tests for CMT and hereditary neuropathy with predisoposition to pressure palsies as these are ddx
Friedreich’s ataxia
Autosomal recessive - males and females affected
Younger patients - death occurs by age 35
Pattern:
Sensory motor neuropathy with sensory and cerebellar ataxia
Observation:
Titubation (nodding movements of head or trunk)
Pes cavus
Scoliosis
Gait:
Widebased, constant shifting of position to maintain balance
Ataxic - both sensory and cerebellar
Strength: distal weakness, at first in legs then progressing to arms
Reflexes:
Extensor plantar responses with absence of deep tendon reflexes
Coordination:
Abnormal past pointing, intention tremor, slow RAM
Sensory:
Loss of vibration and proprioception
Others: Dysarthria Horizontal nystagmus Hypertrophic cardiomyopathy Diabetes
Ask for family history and gene testing (triplet repeat disease)
Fascioscapulohumeral muscular dystrophy
Autosomal dominant, signs presenting first in adolescence
Pattern: muscle disease affecting skeletal muscles of face, shoulders and upper arms without sensory involvement
Observation:
Facial muscle weakness (particularly midface - obicularis oris) with difficulty speaking), eyelid drooping, decreased facial expression
Wasting of shoulder girdle with sparing of deltoid
Abdominal muscle weakness with lumbar lordosis
Scapula winging (scapula moves upward of shoulder abduction)
Triangular shoulders
Tone: normal/decreased
Strength: proximal muscle weakness due to weak scapula fixation, bilateral foot drop
Sensation: no change
Other:
High frequency hearing loss
Retinal telangiectasias
Atrial arrhythmias
Sleep disordered breathing
Myasthenia Gravis
Pattern: Proximal muscle weakness with fatiguability
Observation:
Ptosis
Facial weakness (snarling smile)
Nasal speech (palatal weakness) or dysarthric speech (tongue weakness)
Cranial nerves:
Variable diplopia
Ptosis
Fatiguability - ask to hold upgaze, see progressive ptosis
difficulty swallowing and speech disturbance
Upper limbs:
proximal upper limb weakness with preserved reflexes
fatiguability - forward abduction of arm for 5 minutes, continuous abduction/adduction movements then re-test strength
Other tests:
FVC
Ice pack test, tensilon test
anti-ACH receptor antibodies, Musk receptor antibodies
Imaging of thymus
NCS - rapid reduction in evoked responses
Patterns of gait disturbance
Hemiplegic
- arm flexed, leg extended
- circumduction of foot drop, reduced arm swing
Spastic diplegic
- extended, walk on toes, adductor spasm leads to scissoring
Neuropathic
- high stepping to compensate for foot drop
Myopathic
- waddling, trunk swings from side to side
Parkinsonian
- flexed posture, small steps
Choreiform
- abnormal movements throughout gait
Ataxic
- widebased gait, staggering, fall towards side of lesion
Sensory
- forceful stomping gait, worse in dark
Antalgic
Brown-Sequard (when you get stabbed in the back - but only on one side)
Hemisection of the spinal cord
MOTOR changes are IPSILATERAL
ipsilateral UMN signs below the lesion
ipsilateral LMN at the level of the lesion
Sensory:
- Pain and temp loss on CONTRALATERAL side usually a few segments below the level of the lesion (spinothalamic tract crosses at the cord)
- Vibration and proprioception loss on the IPSILATERAL
- light touch often normal
Causes: see Ddx
Clinical features of motor neurone disease (ALS)
Features are due to degeneration of upper and lower motor neurones
- there are no sensory changes
75% begin as limb onset
25% begin as bulbar onset
initial symptoms:
weakness
atrophy
bulbar signs - dysarthria, dysphagia
longer term:
UMN signs: spasticity, hyper-reflexia, positive Babinski’s, prominent gag reflex
- pseudo bulbar affect - emotional lability
LMN:
muscle atrophy, fasciculations
the oculomotor nerve is affected late in the disease
Spinocerebellar ataxia
Gait:
Broad based, staggering
Difficult tandem walk
Poor truncal control in sitting
Dysdiadochokinesia
Dysmetria (overshooting/undershooting)
Action tremor
Eye movements: Spontaneous nystagmus (downbeat) Square wave jerks on fixation Broken pursuit Hyper/hypometric saccades
Speech:
Slurring, reduced fluency
Cerebellar dysarthria - incoordinated speech, interuptions, dysdiadochokinesia of the tongue
SCAs have above (cerebellar features) + other findings that can be: spasticity paresis hyperreflexia decreased or absent reflexes sensory loss
Subacute combined degeneration of the cord
Affects dorsal and lateral columns - vibration/proprioception and corticospinal tracts
Symmetrical posterior column loss (vibration/proprioception) - associated sensory ataxia Peripheral sensory neuropathy Symmetrical upper motor neuron signs in the lower limbs Absent ankle jerks Knee jerks can be absent or increased Extensor plantar response Dementia Occasional optic atrophy
Absent ankle jerks are part of sensory peripheral neuropathy
Upgoing plantars and brisk knee jerks are part of symmetrical UMN signs in lower limbs
Hereditary spastic paraplegia
Spastic gait - scissoring
Only affects lower limbs
Hyperflexia, upgoing plantars
UMN pattern of weakness - pyramidal weakness
Additional features: Cataracts Ataxia Epilepsy Cognitive impairment Peripheral neuropathy Deafness
Mitochondrial disorder
Ptosis, chronic progressive external opthalmoplegia (bilateral ptosis and progressive weakness of extra-ocular muscles), optic atrophy
Sensorineural hearing loss Diabetes Stroke like episodes Seizures Exertional myalgia Myopathic pattern of weakness - more in a proximal distribution Cardiomyopathy Conduction problems
