Neuro Flashcards
What is weakness
objective loss of muscle strength (formally tested)
Recording muscle strength
5 normal
4 moves against resistance but can be overcome
3 moves against gravity but not resistance
2 moves only when positioned to eliminate gravity
1 flicker of movement
0 complete paralysis
UMN signs
normal bulk
Increased tone (velocity dependent)
Brisk reflexes
localizing UMN weakness to the cortex
Aphasia (L hemisphere)
Spatial neglect (R hemisphere)
Face and arm>leg weakness - lateral motor cortex
Opposite - medial motor cortex
Localizing UMN weakness to the parietal cortex
Normal somatic and decreased cortical sensation
localizing UMN weakness to spinal cord
Presence of a sensory level, and sphincter disturbance
Above T1= arm and leg
Below T1= leg only
Localizing UMN weakness to the brainstem
Cranial nerve involvement
Above facial nucleus (pons) = F, A & L equal
Lower pons/medulla = A & L equal
Weakness in myasthenia
variable with abnormal fatiguability.
Onset pattern of myasthenia gravis
Ocular 65% (50% have AChR +ve Ab)
Bulbar 10%
Leg 10%
Generalized 10% (80% +ve Ab)
Differentials with proximal LMN weakness
subacute myopathy, chronic myopathy, myasthenia gravis
Differentials with distal LMN weakness
Motor neuron disease, motor multifocal neuropathy
Differentials LMN weakness with atrophy
chronic myopathy (proximal)
Motor neuron disease (distal)
Late motor multifocal neuropathy
Differentials LMN weakness without atropjy
Subacute myopathy
Myasthenia gravis
Differentials LMN weakness with ptosis
Myasthenia gravis
Mitochondrial chronic myopathy
Rarely in subacute myopathy
Differentials in LMN weakness with diplopia
Myasthenia gravis
Rarely in chronic myopathy
CK in subacute myopathy
Very high
CK in chronic myopathy
Normal-mildly elevated
CK in motor neuron disease
Normal-mildly elevated
CK in myasthenia
Usually normal
Mean age of onset in MND
65, but quite variable, and variable onset pattern. Median survial’ is 30 months from symptoms onset
Symptoms of MND
Progressive painless weakness
UMN: Weakness, spasticity, brisk reflex, planters up
LMN: Weakness, wasting, fasciculation
No sensory or sphincter dysfunction
Acute phase features of UMN localisation
generally hypotonia
Low/absent reflexes
Plantar responses extensor or mute
Chronic phase features of UMN localisation
clasp-knife (velocity dependent) increased tone
Brisk reflexes
Extensor plantar responses
Acute phase features of LMN localisation
no atrophy
Normal/reduced tone
Low/absent reflexes
Flexor plantar response
Chronic phase features of LMN localisation
atrophy
Reduced tone
Reduced/absent reflexes
Flexor plantar responses
Localizing UMN weakness to the subcortical white matter
equal weakness of F, A & L = hemiparesis )
- posterior limn of internal capsule
Hemisensory change - thalamus
Parkinsonism - basal ganglia
Homonymous hemianopia - optic radiations
Anterior horn motor neuron syndromes distribution of weakness
pure LMN pattern of weakness
Can affect any limb, possible distal wasting.
Additional features of Anterior horn motor neuron syndromes
fasciculations
Bulbar symptoms (dysphagia, dysarthria), tongue wasting
Respiratory failure may feature
Anterior horn motor neuron syndromes possible causes
spinal muscular atrophy
Early stages of amylotrophic lateral sclerosis
Acute flaccid myelitis (eg. Viral, polio and west Nile virus)
Multifocal motor neuropathy
onset can be indistinguishable from anterior horn syndromes.
Typically distal predominant weakness with late wasting, preference for upper limbs eg fingers, less bulbar.
Immune so treat w IV Ig or plasma exchange
Distribution of weakness in NMJ disease
typically proximal
Fluctuating and worse towards end of day
Fatiguable
Ptosis and complex ophthalmoplegia, and dysarthria and dysphagia common
Typically no atrophy unless disuse
Lambert Eaton myasthenic syndrome features
Repeated muscle contractions lead to increased muscle strength
Limb girdle weakness (lower limb first)
Hyporeflexia
Autonomic sympt (dry mouth, importance, difficult micturatikn)
Not commonly eye issues
Labert Eaton Myasthenic Syndrome EMG and Ab
Incremental response to repetitive electrical stimulation and Ab to Ca channels
treatment of Lambert Eaton Myasthenic Syndrome
Treatment of underlying cancer
Immunosuppression eg w pred
Trials of 3;4-diaminopyridine via blocking K channel efflux
Brown Sequard syndrome tracts affected and clin feat
Lateral corticospinal tract
Dorsal columns
Lateral spinothalamic tract
Ipsilateral spastic paresis below lesion
Ipsilateral loss of proprioception and vibration sensation
Contralateral loss of pain and temperature sensation
Subacute combined degeneration of the spinal cord
caused by vitamin B12 and E deficiency.
Tracts: Lateral corticospinal, dorsal columns and spinocerebellar tracst
Feat
Bilateral spastic paresis
Bilateral loss of proprioception and vibration sensation
Bilateral limb ataxia
Friedrich’s ataxia
Tracts: Lateral corticospinal, dorsal columns and spinocerebellar tracst
Feat
Bilateral spastic paresis
Bilateral loss of proprioception and vibration sensation
Bilateral limb ataxia
Cerebellar ataxia
Anterior spinal artery occlusion tracts and feat
lateral corticospinal tracts
Lateral spinothalamic tracts
Feat
Bilateral spastic paresis
Bilateral loss of pain and temp sensation
syringomyelia
tracts: Ventral horns
Lateral spinothalamic tract
Feat
Flaccid paralysis (typical intrinsic hand muscles)
Loss of pain and temp sensation
Neurosyphilis (tabes dorsalis)
affect dorsal columns
Lose proprioception and vibration sensarion
Botulism
a possible cause for NMJ disease
Infection from neuroparalytic toxin, rare in West although can be from home canned food
MND pure upper and pure lower forms
Pure UMN degeneration - primary lateral sclerosis
Pure LMN: Progressive muscular atrophy
Cervical myeloradiculopathy
Usually follows cervical/spinal trauma
Progression halts after some time
LMN finding usually solely cervical/upper thoracic region plus UMN signs in lower limbs
MRI myelopahty and multiple nerve root involvement.
Numbness (hypoaesthesia)
decreased sensitivity to, or diminution of sensory perception in any modality. Most often referred to as pain (hypoalgesia)
tingling (paraesthesia)
abnormal, not painful, sensation that can be pins and needles, crawling, or electric sensatkk s
Dysaesthesia
Abnormal, painful sensations, usually burning or electric
Guillain-Barré presentaiton
occurs at any age but more common in elderly and M>F
2/3 have preceding infection most commonly respiratory. Campylobacter jej = worse prognosis
Progression of symptoms over days to 4 weeks - legs symmetrically weaker than arms, mild sensory symptoms, CN involvement
Ix for GBS
Elevated CSF protein with fewer than 10 cells
EMG/NCV: Conduction block, incr distal latency
Radiculopathies
Sensation loss in 1 nerve root territory
Pain nerve root irritation, can be provoked on exam
Often one reflex lost
Frequently structural, eg disc compression
Plexopathies
> 1 nerve root territory affected
Pain spontaneous or absent, can rarely be triggered
Often >1 reflex lost
Cause can be structural (eg tumours), or post radiation, or inflammatory.
Mononeuropathies
Lesion of a single peripheral merge, most commonly due to compression or trauma
Weakness and later wasti g in muscles innervated by said nerve, and numbness in same territory
Reflexes only affected if innervated myotomes are involved in motor response of reflex arc
Abnormal posturing of affected limb due to selective weakness pattern
Median mononeuropathy
sensory loss over Palmar side of fingers 1 to 3, and radial side of 4. Thenar atrophy
Mostly due to carpal tunnel
Phalen sign: Tingling in median nerve territory by forced flexion of wrist
Tinnel sign: Tapping on nerve can provoke abnormal sensation
Mononeuritis multiplex fearures
sequential or simultaneous sensory change and weakness in different peripheral nerves
Dangerous causes of mononeuritis multiplex
systemic vasculitis (especially when painful)
Diabetes mellitus
Infection eg HIV and leprosy
Length dependent peripheral neuropathy features
Sensory alteration in distal lower limn ascending to knee, at which point symptoms mat be experienced in fingers and hands (glove and stocking)
Predominantly distal weakness with early loss of ankle reclexese with ascension up limb as disease progresses. Proximal muscle strength only affected in extreme cases
Causes of length dependent peripheral neuropathy
Toxic-metabolic - artic diabetes mellitus, also B12/folste deficiency, thyroid dysfunction, liver/kidney derangements
Inherited = Charcot Marie Tooth: With high arched feet, absence of positive sensory symptoms (paraesthesia) but numbness distally and positive FHx
Non-parkinsonian hypomobile patient differentials
severe depression
Hypothyroidism
Toxic or metabolic encephalopathies
Ankylosing spondylitis
Cautious gate (eg past vestibulR neuritis)
Senile gate
Red flags for atypical parkinsonism
supranuclear gaze palsy
Cerebellar ataxia
Early falls
Early orthostatic hypotension
Early incontinence
Poor response to L Dopa
Gait apraxia
Myoclonus definition
Brief, shock like, involuntary movements caused by muscular contractions or inhibituons
dystonia definition
Sustained or intermittent muscle contractions causing abnormal, often repetitive movements, postures, or both
Chorea definition
Involuntary brief, random, irregular contractions flowing from one body part to another and giving, in less severe cases, an appearance if fidgetiness
Tic disorders definition
Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible, and are usually associated with awareness of an urge to perform the movement.
Degenerative causes of parkinsonism
Parkinsons disease
Lewy body dementia
Atypical: Multisystem atropjy, progressive supranuclear palsy, corticobasal syndrome
Secondary causes of parkinsonism
Drugs eg antipsychotics
Toxins or heavy metals
Vascular
Infections or other brain insults
Structural lesions (tumour)
symptoms of PD
Tremor
Rigidity
Akinesia
Postural instability
Hypomimia
Hypophonia
Micrographia
Dribbling
Bent spind
Non motor fearures of PD
Anosmia
Constipation
REM sleep behaviour disorder
Anxiety/depression
Other dysautonomic featurea of PD
ortostatic hypotension
Urinary dysfunction
Erectile dysfunciton
Cognitive impairment in pd
dementia often accompanied by visual hallucinations
Cognitive fluctuations with minutes to hours of drowsiness, slurred speech and cognitive slowing
Aetiology progressive supranuclear palsy
Rare degenerative disorder of about 5/100000
Mean onset age 63.
M>F
clinical features of PSP
Symmetrical parkinsonism with prominent axial rigidity
A “worried” or “surprised”facial appearance with prominent frowning
Problems with downgaze are an early feature
Frequent dementia and with frontal features, eg apathy and or disinhibitin
commonly urinary incontinence
Pathology of PSP
Accumulation of tau protein, rather than of alpha synuclein
Multisystem atrophy aetiology
rare degenerative, prevalence 4.4/100000.
Peak age of onset 55 to 60
M and F roughly equal
Clinical features of MSA
Parkinsonian syndrome with prominent early autonomic failure with orthostatic hypotension, urinary dysfunction and constipation
Cerebellar dysfunction can develop or be present from outset
Respiratory abnormalities during sleep assoc w poor prognosis (strifor and prominent sleep apnoea)
REM sleep behaviour disturbance
Pathology of MSA
Accumulation of alpha synuclein in the brain, but unlike in PD, there are no Lewy bodies
Corticobasal syndrome prefalence
rare neurodegenerative disorder w prevalence 4.4-7.3 per 100,000
Age of onset typically 50 to 70
F slightly more than M
Clinical features of corticobasal syndrome
Strikingly asymmetrical parkinsonism of the upper limbs
Early prominent loss of hand dexterity, and jerks or tremor common
Neuro exam finds:
Apraxia (incapable of imitating gestures)
Myoclonus (triggered by stimulating fingers or hand)
Dystonia (abnormal posturing of hand and fingerz)
Alien hand - moves independent of volitional control
Neglect to affected body part
Imbalance and loss of ambulatkon over time
Response to L dopa usually poor
Pathology of corticobasal syndrome
Accumulation of tau in the brain, some overlap with Alzheimer’s pathology
Functionable tremor characterized as
Entrainable: When doing repetitive task with contralateral limn then tremor adopts frequency of the repetitive voluntary movements of that limb
Distractible: Can decrease while performing different tasks or during the intervention
Suggestible: Commencing examination exacerbates the tremor
Medications causing, triggering or unmasking tremor
valproate
Topiramate
Beta agonists (inhalers)
Lithium
Essential tremor prevalence
affects 4% of people age 40 and increasing with age
Often mild, generally progressive but can be debilitating
Autosomal family nistory common
Clinical features of essential tremor
bilateral action tremor (kinetic and postural components)
MUST FIRST develop in upper limns but can evolve to include head, jaw or vocal cords
Can be exquisitely responsive to alcohol
Only in one axis - which can be seen if you ask the patient to draw a spiral
Treating essential tremor
often respond well to propranolol.
Refractory severe cases maybe neuromodulation through DBS
Young patient with tremor and prominent neuropsychiatric symptoms?
consider Wilson’s disease
Holmes tremor
Usually due to lesions in upper brainstem or thalamic structures.
Large amplitude unilateral and multimodal (resting and action) tremor, worsening remarkably with action.
Hemi body chroea
consider structural causes, eg severe hyperglycemic states, vascular leskoms and space occupying lesions
Generalized chorea with subacute onset
in children: Sydenham’s chorea post strep infections
In adults: SLE
Dystonia
abnormal cocontractkon of antagonistic muscles leading to abnormal postures or twisting and repetitive movements.
Tend to be activated by tasks at least initially
Can happen due to drug reactions
Components required for normal gait
Locomotor system (musculoskeletal system and motor ouput)
Sensory function, partic proprioception
Sensorimotor integration and cognition
Cardiopulmonary function
Attention
Inputs important for gait (afferents)
Proprioception via large peripheral nerve fibres traveling via dorsal columns in spinal cord
Vestibular system (linear and angular head movemrnts)
Visual system
Only two out of three are required, hence why removing vision (Rombergs) = loss of sensory function
The gait cycle
Consists of a stance phase = double and single leg support alternate
Followed by a swing phase = occurs on single leg support, for forward propulsion
Approach to investigating falls
was the fall associated with an abrupt loss of postural tone?
-with loss of consciousness (syncope, seizure, basilar occlusive disease)
-without loss of consciousness (drop attacks)
Does the patient have a history of unsteadiness?
-abnormality in structures involved in gait
-is there weakness, is this UMN or LMN
Patterns of gait difficulty in proximal LMN weakness (eg myopathies)
wide based, waddling gait, struggle to climb stairs
Patterns of gait difficulty in distal LMN weakness (eg length dependent peripheral neuropathies)
Foot drop with steppage gait pattern
Patterns of gait difficultie in UMN weakness (spasticity)
short steps, struggle to lift feet off ground with circumduction of the affected limb
Lesions in cerebellar hemispheres pattern
limb incoordination on the ipsilateral side of the lesion = hemispheric cerebellar syndrome
lesions in the medial cerebellum (vermis) pattern
Axial instability (body sway, head tremor)
Vestibular manifestations (dizziness, vertigo, nystagmus, double vision) if the flocculonodular lobe is affected
Nb. Limbs mat be unaffected, so absence of limn dysmetria/dysdiadokinesia does not rule out cerebellar disturbance
Signals that ataxia (dyscoordination) is dud to a cerebellar lesion
Oculomotor abnormalities (saccadic dysmetria, vertical nystagmus, pure torsional or pure horizontal nystagmus)
Dysarthria (speech irregular in eate and amplitude, slurred, slow and segmented
Body sway (truncal instability) or head tremor
Limb incoordination (dysmetria, dysdiadokinesia, intention tremor)
Ataxia due to sensory dysfunction in proprioception
Patients complain of balance difficult in dark environments, eg at night or when closing eyes in shower
Unsteady and ‘stomping’ gait eith heavy heel strikes can be seen
ataxia due to sensory dysfunction in vestibular system
Acute unilateral vestibular dysfunction can lead to pronounced vertigo, nystagmus and vomiting
Chronic bilateral vestibular failure can present with subtle disequilibrium and oscillopsia (objects appear to jump or vibrate with head movements )
Differentials for acute onset cerebellar ataxia
Stroke of posterior circulation
Younger patients can be post viral
Differentials for subacute onset (days to weeks) cerebellar ataxia
Areflexia - consider Miller Fisher syndrome
Signs of raised ICP - consider space occupying lesion
History of autoimmunity - there are a number of immune disorders causing cerebellar syndrome
History of cancer - paraneoplastic cerebellar degeneration Dan be first manifestation
Differentials for chronic cerebellar syndromes
Degenerative and inheritable conditions
After structural, toxic and nutritional causes have been excluded
Acquired causes disrupting PNS causing sensory ataxia
peripheral neuropathy
- length dependent (diabetes, paraproteinemia)
- non-length dependent (infectious, paraneoplastic)
Ganglionopathy (immune mediated (Sjogren’s), infectious (HIV))
Acquired causes inCNS causing sensory ataxia
Myelopathy (spinal cord lesions any cause eg inflammatory, infections, autoimmune, toxic)
-B12 or copper deficiency -> subacute combined degeneration cord syndrome with selective involvement of dorsal columns
Features of B12 myeloneuropathy
Sensory ataxia
Encephalopathy
Optic atrophy
Ix in patients with cerebellar ataxia
MRI
consider LP in acute and subacute with no structural lesions, especially if possibility of immune mediated
Consider genetic test if chronic and positive FHx, or features suggestive of multisystem atrophy,
Ix for patients with sensory ataxia
MRI spine if suspect myelopathy
B12, copper, HIV and syphilis test in all patients
Neurophysiology if neuropathy suspected
- if NCS shows peripheral demyelinating neuropathy consider LP (immune mediated paraprotein, anyloid causesD
- axinal neuropahty then search for systemic condition eg diabetes, metabolic, nutritional deficiency
If vestibular dysfunction suspected consider
- vestib head impulse test
Audiometry
Management of patients with ataxia
avoid precipitants like excess alcohol
Physio
OT
SALT
patient/carer educaiton/counselling
Red flags in patient with neck/lower back pain
Age >50
History of signif trauma or recent manipulation (chiropractor)
Intractable pain at night or fest
Constitutional symptoms
History of cancer or suspect cancer
History of immunosuppression
History of foreign travel
History of osteoporosis
Progressive neurological symptoms
Symptoms and signs of conus medullaris
Pain not common or severe, bilateral and symmetric in perineum and thighs
Sensory deficit saddle distribution bilaterally
Motor loss symmetric no marked, sometimes fasciculations
Ankle jerks lost
Early and marked bowel and bladder dysfunction
symptoms and signs cauda equina
Spontaneous pain common and severe, mat be unilateral and asymmetric, in perk rum, thighs, legs, back, bladder, sacfal nerve distribution
Sensory deficit in saddle distribution but may be unilateral
Asymmetric motor loss, atrophy may occur, usually no fasciculations
Ankle and oafellar jerks loss
Bowel and bladder dysfunction relatively late
Causes pointing to underlying mechanical cause of neck or back pain
Dull, midline pain
Lack of radiation in a dermatomal pattern, or no radiation
Headache history
Types of headache
Position
Quality (throbbing? Severity?)
Timing
Radiation
Associated symptoms (nausea, photo/phonophobia, aura, cranial autonomic features)
Better (meds? Lying flat.)
Worse (cough? Activity? Orthostatic?)
Triggsrz
Extra headache history qs if old
for GCA
Amaurosis fugax
Diplopia
Fever
Chills
Night sweats
Scalp tenderness
Jaw claudication
Tongue claudication
PMR
Chronic cough
Red flags for headache
Head or neck injury
New onset/new type
New level of pain/abrupt onset
Headache during pregnancy
Age >50
Focal neurological signs or symptoms
Systemic illness
Secondary risk factors (cancer, immunocompromise, recent travel)
Differentials for thunderclap headache
Subarachnoid hemorrhage
Intracerebral haemorrhage
Cerebral venous sinus thrombosis
Pituitary apoplexy
Carotid/vertebral artery dissection
CSF leaks
Spontaneous retrocoival haemaromas
Colloid cyst of third ventricle
Hypertensive encephalopathy
Primary headaches
Migraine
Tension
Cluster
Secondary headachws
Idiopathic intracranial hypertension
Giant cell arteritis
Medication overuse headache
Diagnosis criteria for migraine
At least 5 attacks
Attacks last 4-72 hours
Two of:
-severe intensity
-unilateral
-throbbing
-activity worsens headache
One of:
-nausea and or vomiting
-photo/phonophobia
Not attributed to anything else
Migraine aura
May be focal neurology, usually preceding:
-visual
-dysphagia
-somatosensory
-motor
-other
Develops over minutes and complete duration 15-20 mins
Migraine treatment
Identify and eliminate triggers
Abortice treatment: Simple analgesics, 5-HT1 agonists (triptans, ergotamine), anto emetics, opiates
Preventative treatmenr: (basically if bad and recurrent)
Tension type headache
mild to moderate intensity
No migrainous features
No cranial autonomic features
No secondary causes
Not exacerbated by daily activities
Treat
Aborticz: NSAIDs, acetaminophen
Prophylaxis: TCAs. Topiramate, valproic acid, gabapentin
cCluster headache diagnosis
At least 5 attacks
Severe or unilateral orbital, supraorbital and/pr temporal pain lasting 15-180 mins if untreated
Usually sign things like lacrimation, nasal confesfion, owdema, sweating, ptosis etc
Really frequent, dg one every other day to 8 per day
No other cause
Trigeminal neuralgia
Painful, unilateral shock pain im V2, V3 and a bit V1
Rapid onset/offset and lasting seconds
No sensory deficits or constant neuropathic pain
Triggered by wind, talking, chewing, touching
MRI w gadolinium required to exclude tumour, demyelination or stroke as cause
Trigeminal neuralgia treatment
Carbemazepine is drug of choice
Surgery eg microvascular decompression or destruction trigeminal ganglion
Idiopathic intracranial hypertension symptoms
symptoms of raised ICP (headache, oulsatile tinnitus, horizontal diplopia)
Symptoms of papilloedema (loss of perpipj vision, blurring, transient visual onscurations)
Medication overuse headache diagnosis
headache occurring on at least 15 days per month in a patient with a pre-existing headache disorder
Regular overuse for >3 months of one or more drugs that can be taken for acute and or symptomatic treatment of headache
Not better accounted for by another diagnosis
Risk factors for idiopathic intracranial hypertension
Female sex
Obesity
Reproductive age
Menstrual irregularity
Medications (cimetidine, corticosteroids, retinoic acid, minocycline, nitrofurantoin, trimethoprim and sulfamethoxazole)
Chronic renal insufficiency
SLE
Clinical examination for idiopathic intracranial hypertension
Visual function testing w fundoscopy, visual field testing, visual acuity and colour testing
Ocular motility testing
Neurological examination
Differential diagnoses for IIH
Dural venous sinus thrombosis
Intracerebral jazz lesion
Hydrocephalus
Meningeal infiltrative and inflammatory disease
Treatment for IIH
Medical: Lifestyle modification and weight loss, medications (acetazolamide, topiramate)
Surgical
Optic nerve sheath fenestration
CSF shunt for refractory cases
Examining cranial nerves in a comatose patient
ii: Ophthalmoscopic exam
Blink to threat
Ii and iii: Pupillary responses
Iii, iv, ci. Viii: Spontaneous extraocular movements, nystagmus, dysconjugate gaze, oculocephalic maneuve, caloric testing
V, vii: Corneal reflex, facial asymmetry, grimace response
Ix, x: Gag reflex
Examination of sensory and motor in a comatose patient
Spontaneous movements
Withdrawal from painful stimulus
Reflexes exam in comatose patient
Deep tendon reflexes
Plantar reflexes
Posturing reflexes
Special reflex in case of suspected spinal cord lesions
GCS components
Eye opening: Spontaneous (4), to speech (3), to pain (2), no response (1)
Verbal response:
Oriented (5), sentences (4), words (3), sounds (2), no response (1)
Motor response: Obeys commands (6), localizes to pain (5), flexion withdrawal to pain (4), abnormal flexion ro pain (3), extension to pain (2), no response (1)
Risk factors for delirium
Age >70
Dementia or mild cognitive impairment
Vision impairment
Hearing impairment
Functional limitation
Alcohol abuse
Malnourishment
Dehydration
Iatrogenic precipitants for delirium
Use of restraints
Urinary catheter
Recent surgery
Sleep deprivation
Untreated pain
Drugs
Red flags for delirium
Focal signs
Alcohol/malnourishment
History of epilepsy
Features suggestive of seizures
Prolonged over days kto weeks
Fever of unknown origin
Young patient
Weekly alcohol limit in units
14 units
Units for binge for Men and women
8 units for man and 6 for women
Harmful drinking units per week
> 50 units/week for men, >35 units/weeks for women
Alcohol bloods
AST:ALT ratio increased
Incr GGT and ALP
Macrocytic anaemia
Reduced platelets
Carbohydrate deficient transferrin
Motivational interviewing in brief
Feedback - personal risk/interviewing
Responsibility - you need to make change
Advise to cut down
Menu - options to change behaviour and targets (drink free days, drink diary, cut 10% per week, identify risks, other activities, information about sefvices)
Empathic - listen and avoid confrontation
Self-efficacy- encourage and promote self belief
When does lateral tentorial herniation happen
from unilateral expanding mass
What causes subfalcine midline shift
early occurrence in unilateral space occupying lesion. Seldom any clinical effect
What causes central tentorial herniation
midline lesion or diffuse swelling of cerebral hemispheres causes vertical displacement of the midbrain and diencephalon
What causes tonsillar herniation
Subtentoiral expanding mass causes herniation of cerebellar tonsils through the foramen magnum
Risks for subdural haemorrhage
Elderly
Alcoholics
Alzheimer’s
At risk for falls
Susceptibility to haemorrhage eg chronic liver disease
Differentials for thunderclap haemorrhage
SAH
Meningitis
Intraparenchymal haemorrhage
Hypertensive encephalopathy
Arterial dissection
CSF leak
Indications and contraindications for LP
Indict: Investigate for infection, 12h after SAH for xanthochromia
Contra:coagulopathy, mass lesion
Roles specific to the left hemisphere
analytical and logical thinking
Language (except emotional prosody
Expression of emotion
Writing and speech if right handed
Roles specific to the right hemisohere
spatial abilities
Comprehension of complicated patterns and drawings
Perceiving emotion
Functions of the frontal lobe
higher order processing, decision making
Motor cortex (precentrral gyrus)
Broca’s area (in dominant hemisphere) expressive for speech
Supplementary motor area
Social graces/inhibition
Functions of the parietal lobe
post central gyrus = sensory cortex
Supramarginal and anterior gyri of dominant hemisphere = Wernicke’s area (comprehension)
Non dominant = integratin of visuospatial awareness
Visual pathways pass through deeply
Posterior parietal cortex for planning movements
Lesion of parietal lobe on left causes
impaired:
Two point discrimination, stereognosis, graphaesthesia, localisation of touch, position sense
Disorders of language
Gerstmann Syndrome: Finger agnosia, acalculia, right left disorientation, agraphia
Lesions of parietal lobe on right causes
impaired:
Two point discrimination, stereognosis, graphaesthesia, localisation of touch, position sense
Disturbances in integration of personal and extrapersonal space
Hemispatial neglect
Constructional apraxia
Dressing apraxia
Expressive speech
broca’s area
Fluency of speech
Wernicke’s area
What does the insular lobe do
part of the paralimbic group
Connects limbic cortex with neocortex
Receives visceral, taste and pain sensory information
Role of the thalamus
powerful hub of integration
Eg w ascending reticular activating systems, basal ganglia, limbic system, somatosensory, auditory, visual, vestibular input and thalamocortical and corticothalamic projections’ so thalamic issues lead to broad spectrum of neurologic issues
Clues for NMJ disorders
(eg Myaesthenia gravis and lambert Eaton myasthenic syndrome)
Generalized FATIGUABLE weakness
Unexplained ptosis, bulbar problems
CN I issues - think…
Cold, parkinsons
CN III, IV, V(1&2), VI think
Cavernous sinus
CN V, VII, VIII think…
cerebellopontine angle - acoustic neuroma?
CN VI think
this is a false localizing sign. Be careful
CN IX, X, XI, XII thnk…
Base of skull
Eye movements which nerve
LR6 SO4. Everything else 3
Lateral recurs abducts eye
Superior oblique depressed adducted eye
CN III palys
down and out, and ptosis
Horner’s syndrome
thjnk hidden pathology: Pancoast’s tumour, Central line into subclavian, damage to internal carotid (dissection after heading a football really hard)
= sympathetic chain lesion between brain and eye
-> ptosis, meiosis, anhydrosis
Is it vasovagal syncopy?
prolonged upright position
Sweating prior to loss of consciousness
Pre syncopal symptoms
Pallor
Nausea
Cardiac syncope
bewAre, less warning and quite abrupt
>10% risk of death per year
History of IHD?
Investigate w ECG as possibly long QT, HOCM
More likely epileptic seuizure
stertorous breathing
Postictal confusion
Relatively short duration
Urinary incontinence and tongue bite can occur in either
More likely non epileptic attack
long duration
Asynchronous movements
Pelvic thrusting
Side to side head/body movements
Closed eyes
Ictal crying
Memory recall
Patient descriptions of non epileptic atracks
Metaphors of space/place patients go through
Resistance to focusing on individual seizure episodes, and only provide detailed description with prompting
Focusing on impact on their lives
Patient description of epileptic seizures
depicting seizure as agent/force or event/situation
Volunteered detailed first person accounts of seizures
Status epilepticus
any seizure longer than five mins
May have convulsive break but then resume without regaining consciousness
Excitotoxic danger so urgently end with high dose benzos
Identify cause
After benzos give usual medication
Once thirty mins have elapsed get ITU invovlved for midazolam/propofol infusion
Prognosis of epilepsy
50-60% controllable with one anti epileptic drug
Intractable in 10-20%, temporal lobe surgery?
Acute central bladder issues
flaccid, acontractile bladder
Reflex control of sphincter-> urinary retention, bladder distention, overflow incontinence
Chronic central bladder issues
hyperreflexic spastic bladder. Urinary frequency and urge incontinence due to detrusor-sphincter dysnergia
Most common type of brain tumour
Mets are most common
Partic: Lung, breast, bowel, , melanoma and kidney
Most common primary brain tumour in adults
glioblastoma multiforme
Imaging glioblastoma
Solid tumours with central necrosis and rim which enhances with contrast.
Disruption of blood brain barrier so assoc w vasogenic oedema
Treatment of glioblastoma
surgical with post op chemo and or radio
Dex often for oedema
Prognosis is about a year
Second most common primary brain tumour in adults
meningioma
Features of meningioma
typically benign extrinsic tumours of CNS
Arise from arachnoid xap cells and typically located next to dura
Partic AR falx cerebei, superior sagittal sinus, convexity or skull base
Cause symptoms by compression rather than invasion
Investigations and treatments for meningioma
ix with CT AND Mei
Treatment may be observation, radiotherapy or surgical resection
Features of vestibular schwannoma
benign tumour arising from eighth cranial nerve
Often at cerebellopontine angle
Presents with hearing loss, facial nerve palsy and tinnitus
Assoc w neurofibromatosis type 2
Most common primary brain tumour in children
pilocytic astrocytoma
Feat of medulloblastoma
aggressive paediatric brain tumour
Arises within infratentorial compartment and spreads theohfh cns
Treated with surgical resection and chemo
Pituitary adenoma fearures
benign tumours of pituitary gland
Micro = less than 1cm, macro = more
If secretory will present with consequences of hormone excess
If non secretory may be solely bitempral hemianopia due to crossing nasal fibres
Investigation and treatment of pituitary adenomas
pituitary blood profile and Mei
Can be hormonal treatment or transphenoidal resection
Craniopharyngoma features
most common paediateic supratentoorial tumour
Solid/cystic tumour derived from remnants of Rathke’s pouch,
Mt present with hormonal disturbance, symptoms of hydroceph or bitemporal hemianopia
What is vestibular neuritis
neuropathy of vestibular system probably caused by reactivation of latent HSV1 in vestibular ganglion.
Always preceded by prior upper respiratory tract infection
History vestibular neuritis
Sudden, spontaneous, severe ongoing vertigo
Not triggered by movement but may be exacerbated by it
Frequent nausea and vomiting
Hearing loss and tinnitus in labyrinthitis
Symptoms to ask about which indicate more serious than vestibular neuritis or labyrinthitis
Otorrhoea with middle ear disease or head trauma
Otalgia = herpes zoster otichs
Neck pain/stiffness = meningitis or vertebral artery dissection
Cardiovascular risk factors increased likelihood of being stroke or TIA
Family nistory of migraine or Meniere’s make these more likely
Drugs eg aminoglycosides, amlodipine, amdtodepressants and antiepileptics can all cause vertigo
Examination vestibular neuritis
Gait = tend to fall towards affected side when standing or walking
Weber’s test: Tuning fork on head, quieter In affected ear
Head impulse test: Get patient to fix their gaze on your nose then turn their head rapidly side to side. If intact zVOR then should be able to keep gaze on nose, if neuritis then will have catch up reflexive saccade
Horizontal nystagmus
HINTS exam
HINTS examination
Head impulse test, nystagmus type and skew
Vestibular neuritis management
resolves over days to weeks without treatment
If ant concerns like sudden onset unilateral hearing loss need to admit for possible acute ischaemia or labyrinth or brainstem
Otherwise reassure, and they can lie still with eyes closed during acute attack, prochlorperazine or antihistamines can help w vertigo nausea and vomiting
What is meniere’s disease
disorder of inner ear caused by change in fluid volume in the labyrinth, generally with multifactorial, inexact cause. Most commonly middle aged and qhite rare.
Symptoms of meniere’s disease
Vertigo, tinnofis and fluctuating hearing loss with a sensation of aural pressure.
Fluctuating and episodic nature is important, attacks are minutes to hours (2-3), and inclusters of 6-11 per year. Initially at least unilateral.
Examination in Meniere’s diseae
there are no signs, but should examine cardiovascular, neuro and ENT systems to look for other causes of similar symptoms.
Driving and vertigo
any vertigo = must notify DVLA , and case then considered individually depending on whether ant warning, severity and whether controlled.
Treatment of Meniere’s disease
Acute atracks: vertigo and nausea with prochlorperazine or other anti emetic, mat need to be IM if vomiting, and may need fluid rehydration
Prophylasis: Lifestyle avoid salt, caffeine, chocolate, alcohol, tobacco and fatigue. Consider betahistine?
Can be really debilitating
What is BPPV
most common cause of vertigo due to inner ear dysfunction. Otoliths detach and then circulate loosely in the semicicrcular canals so generating a sensation of ongoing movement
Affects approx 3% of people typically presenting in women age about 50
History with BPPV
episodes of vertigo provoked by head movements eg rolling over in bed, lying down, sitting up, turning head in horizontal plane
Comes on suddenly (typically 5 secs after provocative movement) and lasts 20-30s and rapidly resolving if head kept still.
Nausea common
Symptoms typically worse in morning
No hearing loss, tinnitus, mastoid pain, headache or photophobia
May present as a fall
Red flags with vertigo
unilateral hearing loss or tinnitus
New inset headache
Focal neurological signs
Cerebellar signs eg gait ataxia
Dix hallpike manoeuvre
Ask patient to keep eyes open and look straight ahead. Sit on couch with head turned 45 degrees to one side
Then lay person down rapidly, supporting head and neck, until head is extended 20-30° over end of couch with chin up and test ear down
Observe eyes for 30 secs for nystagmus and note what it’s like
Provoking vertigo and upbeating nystagmus = zBPPV normallt 5-20 sec after manoeuvre
Management of BPPV
advise symptoms ususallt self limiting over several weeks but may recur
Offer Epley’s manoeuvre
Advise no driving when dizzy ir if driving might provoke an attack
Epley’s manoeuvre
Sit patient upright with head turned 45° to affected side
Place hands on either side of head and lie down with head 30° over edge of bed, wait 30 sec ti 1 min
Rotate head 90° to opposite side with patient’s face upwards
Then roll patient on to side whilst holding head in position then rotate head to face ground
Sit patient up sideways while maintaining head rotation (still facing geound)
Rotate heae to central position
Midline cerebellar lesions symptoms
severe gait and truncal ataxia
If extending, fourth cranial nerve leskons, severe ipsilateral arm tremor, marked nystagmus, vertigo and vomiti g
Cerebellar hemisphere lesions symptoms
ipsilateral limb ataxia (intention tremor, past pointing, mild hypotonia)
Limb rebound
Tend to be more subtle nystagmus
Acute onset ataxia didferentials and presentation
cerebellar haemorrhage or infarction
Occipital headache, vertigo, vomiting, altered consciousness
Subacute ataxia differentia’s
viral infection: Children age 2-10 wieth pyrexia, limb and gait ataxia and dysarthria appearing over hours to days and lasting up to 6 months
post infectious encephalomyelitis
Other options: Hydrocephalus, posterior fossa tumours, absecesses
Differentials for episodic ataxiaa
may appear bizarre and seem a bit functional
episodic alone can develop in teenage years, lasting from minutes to hours anf usually triggered by something (eg stress, exercise, caffeine or alcohol)
Drugs
MS
Transient vertebrobzsilar ischaemic attacks
Foramen magnum compression
Chronic progressive ataxias didferentials
commonly caused by chronic alcohol abuse associated with malnutrition, may improve with thiamine and poss other vitamins
Also drugs eg phenytoin
Solvent abuse
Heavy metals
Structural lesions
Paraneoplastic cerebellar degeneration sssociated with carcinomas of the lung or ovaries
CJD
Examining for cerebellar signs
DANIISH: Dysdiadokinesia, ataxia, nystagmus, intention tremor, slurred staccato speech, hypotonia
Causes if cerebellar dysfunction in infantsz
cerebral palsy
Intrauterine infection
Pontocerebellar hypoplasia
Joubert’s syndrome
Trisomies
Pyruvate dehydrogenase deficiency
Friedrich’s ataxia
most common type of hereditary ataxia, affecting about 1/50000
Symptoms usually develop before age 25, and then worsen over time
Symptoms: Ataxia causing falls, dysarthria, increasing weakness in legs, dysphagia, vision and hearing loss, diabetes, hypertrophic cardiomyopathy, peripheral neuropathy
Ataxia telangiectasia
rare hereditary ataxia
Symptoms begin in early childhood
Most need wheelchair by age 10
Increasing dysarthria and dysphagia
Telangiectasia in corner of eyes and on cheeks
Immunodeficient
Incr risk cancer partic ALL
Usual life expectancy 19-25
Spinocerebellar ataxias
Group of hereditary ataxias that don’t begin until adulthood
Variable symptoms, but including ataxia, dysarthria, dysphagia, muscle stiffness and cramps, peripheral neuropathy , memory loss,slow eye mivenets, urinary urge or incontinence
Weber’s test
512Hz tuning fork placed in middle of forehead
Normal: Sound is heard equallt in both ears
Sensorineural hearing loss: Sounds heard louder on side of intact ear
Conductive hearing loss: Sound is heard louder on side of the affected ear
To differentiate between these, perform Rinne’s test
Rinne’s test
Place vibrating 512Hz tuning fork on mastoid process = test bone conduction
Confirm patient can hear, then ask then when can Bo longer hear it
Then move tuning fork in front of external auditory meatus to test for air conduction
They should then be able to hear sound again as air conduction is normally better than bone
If conductive hearing loss, bone conduction mat be better than air.
Other tests for hearing loss
Pure tone audio entry (measures decibel and pitch hearing)
Typanometry is measure of stiffness of eardrum and evaluates middle ear functoon
Qhat is sinusitis
inflammation of membranous lining of one or more sinuses, generally accompanied by inflammation of the nasal mucosa, but can be acute, recurring or chronic
Acute sinusistis
bacterial or viral infection of sinuses lasting fewer than four weeks and then completely resolving
Rends to be viral, and then secondary bacterial infection with strep pneumo, h influenzae, and morazella
Common
Present with non resolving cold >1 week with pain in affected sinus (partic fullness feeling worse on bending forward), possibly phrexia, discharge from nose and loss of smell.
Mostly reassure and give paracetamol/ibuprofen, a week of intranasal decongestant, nasal irrigation and warm face packs
History for vertigo
first attack
Duration
Precipitating factors
Otologic symptoms
Medications
Age
Acoustic neuromas
2-30/ million
Slowly progressive
Up to 50% don’t grow ar all
Present with; unilateral sensorineural hearing loss, unilateral tinnitus, Imbalance, unilateral facial nerve weakness, unilateral trigeminal paraesthesia
More likely to be syncope than seizure?
prolonged upright position
Sweating prior to LOC
pre syncopal symptoms
pallor
Nausea
Can be postural, reflex or cardiac (which is more dangerous and rends to have less of a warning prodrome)
Seizures and the DVLA
Always have to have 6 months off driving after first seizure (even if likely cardiovascular)
And if epilepsy must have 12 monrhs seseizure free
Investigations for epilepsy
CT/MRI
EEG - is this generalized epioepsy? Is there a specific focus? Is it non convulsive status?
ECG
Common side effecrs of carbemazepine
rash
Hyponatraemia
Cardiac conduction issues
Increased ADH
Pancytopaenia
Hepatotoxicity
Dizziness, unsteadiness, cramps
Side effects of phenytoin
gingival hypertrophy
Rash
Peripheral neuropathy
Pancytopaenia
Hepatotoxicity
Side effects of lamotrigine
rash - needs slow titration
Tremor
Side effects of levetiracetam
mood disturbance - low mood, aggressive behaviour
EEG in epilepsy
Demonstrates abnormal cortical excitability
Electrodes placed on scalp reflect asummation fexexcitatory inhibitory postsynaptic neurons at most superficial layer of cortex, and need relatively large areas to be activated at the same time in order to pick up activity
Hence specific but not sensitivity
Features of temporal lobe seizures
slow head turn at onset (towards seizure focus?)
Automatisms (fidgeting, picking)
nose wiping
Ictal spitting, vomiting, coughing
Frontal lobe seizures
Jacksonian seizures: March of limb jerking spread
Speech arrest or dysphagia suggest dominant bemisphere involvement
First line treatments forparkinsons
If motor symptoms affecting patients QoL: Levodopa
If motor symptoms not affecting patients QoL: Dopamine agonist ( eg bromocroptine, ropinirole, cabergoline) levodopa, or MAO-B inhibitor (eg selegiline)
Relative benefits of levodopa vs dopamine agonists vs MAO B inhibitors
levodopa more improvement in motor symptoms and ADLs
But levodopa also more motor complicatkons
dopamine agonists: More adverse events (excessive sleepiness, hallucinations, impulse control disorders)
Impulse control disorders in Parkinsons
can occur with any dopaminergic therapy but more common with:
Dopamine agonist therapy
History of previous impulsive behaviours
History of alcohol consumption and/or smoking
Levodopa info
nearly always combined with decarboxylase inhibitor eg carbidopa to precent peripheral metabolism of dopamine outside brain
Common adverse effecrs: Dry mouth, anorexia, palpitations, postural hypotension, psychosis
Plus effects from difficult achieving steady dose: Wearing off, and then to on off phenomenon, with dyskinesia at peak dose
Do not stop acutely asrisk of acute dystonias
Dopamine receptor agonists info
Parkinson’s meds
eg bromocriptine, ropinirole, cabergoline, apomorphine
Bromocriptine nd cabergoline are ergot derived and have been assic w pulmonary, retroperitoneal and cardiac fibrosis. Do echo, ESR, creatinine and CXR before trearment
Risk of impulse control disorders and excessive somnolence
More likely than levodopa to cause hallucinations in older patients
MAO-B inhibitors
Parkinson’s nedicarjons
Eg selegiline
Inhibits breakdown of dopamine secreted by dopaminergic neurons
Amantadine
Parkinson’s medication
Mechanism not fully understood, probably increases dopamine release and inhibits its uptake at dopaminergic synapses
Side effecrs: Ataxia, alurred speech, confusion, dizziness, livedo retivularis
COMT- inhibitors
Parkinson’s medication
Eg entacapone, tolcapone
COMT is enzyme involved in breakdown of dopamine so can be used as adjunct to levodopa therapy, in established PD
Peripheral neuropathy causing predominantly motor loss
guillain Barre syndrome
Chronic inflammatory demyelinating polyneuropathy
Porphyria
Lead poisoning
Hereditary sensorimotor neuropathies eg Charcot Marie Tooth
Diphtheria
Peripheral neuropathy causing predominantly sensory loss
diabetes
Uraemia
Leprosy
Alcoholism
Vitamins B12 deficiency (subacute combined degeneration of cord-> loss of joint position and vibration first from dorsal column
Amyloidosis
MRI investigations for MS
demonstrations of lesions disseminated in time and space
Contrast MRI -> high signal T2 lesions
CSF-> oligonclonal bands (not in serum), increased intrathecal synthesis of IgG
Common peroneal nerve lesoom
foot drop
Weakness of dorsiflexion
Weakness of foot eversion
Weakness of extensor hallucis longus
Sensory loss over dorsum of foot and lower lateral part of leg
Wasting of anterior tibial and peroneal muscles
Chronic myopathy (inherited) oresenfe and absence of symptoms
proximal weakness
Proximal atropjy
Ptosis present
rarelY displopia
frequent head drop
Normal to mildly elevated CK
Subacute myopathy presence and absence of symptoms
proximal weakness
no atrophy
Rarely ptosis
No diplopia
Frequent head drop
very high CK
AAnterior horn syndromes features
WEAKNESS: Pure LMN pattern, can affect any limb. Mat see wasting
Additional fearures: Fasciculations, bulbar symptoms (dysphagia, dysarthria) +/- tongue wasting
May have respiratory faikure
Possible causes of anterior horn syndromes
Spinal muscular atrophy
Early stages amylotrophic lareral sclerosis
Acute flaccid myelitis - eg viral: Polio, west nile virus
Multifocal motor neuropathy features
onset can be indistinguishable from anterior horn syndromes
Distribution of weakness: typically distal predominant with late wasting. Preference for upper limbs and very distal (fingers). Not typically bulbar symptoms
What is multifocal motor neuropatht
Rare disorder of autoimmune attack on multiple motor nerve, ttypically starting with asasymmetrical weakness in the patient’s hands
Antibody to GM1
Few sensory symptoms
Chronically progressive without remission
Very similar to CIDP but onset asasymmetric and not remitting
Treatment of multifocal motor neuropathy
IVIg or plasma exchange, ongoing every 2-5 weeks
Guillain Barre syndrome chcharacterized
Rapid onset ascending weakness, numbness and oaralysis of limbs, respiratory muscles and face
lost reflexes
50% after microbial infectiom
Neuromuscular junction diseases features
distribution of weakness: typIcaloy proximal, fluctuates and often worse towards the end of the day
ffatiguable weakness
Frequent ptosis
extraocular muscle iinvolvement > complex ophthalmoplegia
Bulbar involvement > dysarthria and dysphagia
Not typically muscke atrophy
Differentials for neuromuscular junction weakness
myasthenia gravis = immune mediated by antibody against ACh receptor or rarely anti-MUSK
Lambert Eaton myasthenic syndrome = antibodies to Ca channels. Frequently paraneoplastic
Botulism = neuroparalytic toxin rare in West simetines home canned foods
Congenital/inherited forms are rare
Cervical myeloradiculopathy
ususually following cervical/spinal trauma
Course is static and progression halts after some time
LMN findings in cervical/upper thoracic region plus UMN sign s in lower limbs
MRI: Myelopathy plus multiple nerve root involvement
Diagnosis of GBS
lumbar puncture for elevated protein AND normal WCC
EMG
Treatment of GBS
unpredictable, so unless very mild, need to be hospitalised to monitor respiration
Plasma exchange and IV Ig can be helpful to shorten the course
Mat well need lots of rehab, OT, social work and psychiatry input
Possible long term recurrence of fatugue and exhaustion
CIDP what is
Progressive autoimmine polyneuropathy with distal and proximal weakness and sesensory deficit developing over at least 8 weeks
Underlying cause not known but thought to be deranged immune response causing perioheralperipheral in damage
Can be pure motor or sensory oresentations
DifDifference between guillain barre and CIDP
guillain barre: Onset less than 4 weeks
Often clinical history of infection prior to onset of symptoms
More commonly involves cranial nerves and respiratory failure than CIDP
Treatment for CIDP
corticosteroids 60mg OD pred six weeks
plasma exchange and IV Ig for relief from symotoms
Approx 15% patients fail to respond to treatment
Becker muscular dystrophy
x linked recessive dystrophinopathy. Less severe version of Duchenne
Develops after age of 10, and intellectual impairment much less common than in duchenne
What is charcot Marie tooth
most common hereditary peripheral neuropathy
results in predominantly motor loss
No cure and management focused on physical and occupational therapy
Clinical fearures of charcot Marie tooth
history of frequently sprained ankles
foot drop
High arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
Stork leg deformity
