Neurology Flashcards
Frontal lobe abnormalities?
- Difficulties with task sequencing
- Difficulties with executive skills
- Expressive aphasia (Broca’s) in the inferior frontal gyrus
- Anosmia
- Primitive reflexes
- Perseveration (repeatedly asking same question or doing same task)
- Changes in personality
- Inability to generate a list
- Disinhibition
Parietal lobe abnormalities?
- Apraxias: loss of the ability to execute learned purposeful movements
- Neglect
- Astereognosis (unable to recognise object by feeling) = tactile agnosia
- Homonymous inferior quadrantanopia
- Sensory inattention
- Acalculia: inability to perform mental arithmetic
- Gerstmann’s syndrome (lesion of dominant parietal):
- Alexia: in ability to read
- Acalculia
- Finger agnosia
- Right-left disorientation.
Temporal lobe abnormalities?
- Homonymous superior quadrantanopia
- Prosopagnosia (difficulty recognising faces)
- Wernike’s (recepTive) aphasia
- Memory impairment
- Auditory agnosia
Occipital lobe abnormalities?
- Cortical blindness (blindness due to damage to visual cortex, may present as Anton syndrome: there is blindness but patient is unaware or denies blindness)
- Homonymous hemianopia
- Visual agnosia (seeing but not percieving objects - it is different to neglect since in agnosia the objects are seen and followed but cannot be named)
Spinal tract dysfunction?
- Dorsal column dysfunction: (joint position and light touch)
- Spinothalamic dysfunction: (pinprick and temperature)
Mnemonic for homonomous quadrantanopia?
PITS
Parietal = inferior, Temporal = superior
Upper and lower bitemporal hemianopia?
Optic Chiasm
- Upper > Lower = inferior compression - pituitary tumour
- Lower > Upper = superior compression - craniopharyngioma
Causes of nystagmus?
- Visual disturbances
- Lesions of the labyrinth
- The central vestibular connections
- Brain stem or cerebellar lesions.
Causes of SAH?
- 85% are due to rupture of berry aneurysms
- Associated with APKD, Ehlers-Danlos syndrome and coarctation of the aorta
- AV malformations.
- Trauma
- Tumours
ECG changes in SAH?
- Deep symmetrical T wave Inversion
- Prolonged QT interval
Features of specific intracranil venous thromboses?
Sagittal
- May present with seizures and hemiplegia
- Parasagittal biparietal or bifrontal hemorrhagic infarctions are sometimes seen
Cavernous
- Other causes of cavernous sinus syndrome: local infection (e.g. Sinusitis), neoplasia, trauma
- Ophthalmoplegia due to IIIrd, IVth and VIth nerve damage
- Trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
- Central retinal vein thrombosis
- Swollen eyelids
Lateral
- VIth and VIIth cranial nerve palsies
Features of Wernicke’s
- Nystagmus
- Ophthalmoplegia
- Ataxia
- Confusion, altered GCS
- Peripheral sensory neuropathy
- Impairment of short-term memory
Triad of normal pressure hydrocephalus?
- Urinary incontinence
- Dementia and bradyphrenia (slowness of thought)
- Gait abnormality (may be similar to parkinson’s disease)
Cause = reduced CSF absorption by arachnoid villi
Features, risk factors, investigations, management of Idiopathic Intracranial HTN (BIH)?
Features
- Headache
- Blurred vision
- Papilledema (usually present)
- Enlarged blind spot
- Sixth nerve palsy may be present
Risk Factors
- Obesity
- Female sex
- Pregnancy
- Drugs: oral contraceptive pill, steroids, tetracycline, vitamin A
Ix
- CT Scan
- LP
- Cerebral MRI with MR Venography
Mx
- Weight loss
- Diuretics e.g. Acetazolamide
- Steroids
- Repeated lumbar puncture
- Surgery: optic nerve sheath decompression and fenestration may be needed to prevent optic nerve damage. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
Criteria for carotid endarterectomy?
- Recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled
- Should only be considered if carotid stenosis > 70% according ECST criteria or > 50% according to NASCET criteria
Stroke by anatomy?
Anterior Cerebral Artery
- Contralateral hemiparesis and sensory loss, lower extremity > upper
- Disconnection syndrome (akinetic mute patient)
Middle Cerebral Artery
- Contralateral hemiparesis and sensory loss, upper extremity > lower
- Contralateral hemianopia
- Aphasia (Wernicke’s)
- Gaze abnormalities
Posterior Cerebral Artery
- Contralateral hemianopia with macular sparing
- Disconnection syndrome
Atypical strokes?
Lacunar
- Present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
Lateral Medulla (Posterior inferior CA - Wallenberg’s syndrome)
- Ipsilateral: ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy
- Contralateral: limb sensory loss
Pontine
- VI nerve: horizontal gaze palsy
- VII nerve
- Contralateral hemiparesis
Basilar Artery
- Most of patients present with nausea, vertigo and vomiting
- Some present with motor deficits, dysarthria and speech involvement or headaches
- May present with visual disturbances. This includes abducens nerve palsy, conjugate gaze palsy,
internuclear ophthalmoplegia and ocular bobbing - Locked-In Syndrome: patient is awake but is unable to respond in anyway except by vertical
gaze and blinking (lesion is in ventral pons) - 70% of patients presenting with basilar artery territory stroke are hypertensive.
- Management includes vigorous control of hypertension and antiplatelet agents.
Bamford Stroke Classification?
Spinal Cord Syndromes
Syringomyelia - what is it? Features?
Developmental, slowly enlarging cavitary expansion (longitudinal) of the cervical cord that produce progressive myelopathy
Associated with chiari malformation
Features
- Maybe asymmetrical initially
- Slowly progressives, possibly over years
- Motor: wasting and weakness of arms
- Sensory: spinothalamic sensory loss (pain and temperature)
- Loss of reflexes, bilateral upgoing plantars
- Also seen: horner’s syndrome
Subclavian steal syndrome?
Retrograde flow in the vertebral artery due to proximal subclavian artery stenosis. Neurological symptoms are precipitated by vigorous exercise with the arm above the head, such as painting a wall.
Subacute combined degeneration of the spinal cord? Features? Causes?
Degeneration of the posterior and lateral columns of the spinal cord as a result of vitamin B12 deficiency (most common), vitamin E deficiency or Friedrich’s ataxia. It is usually associated with pernicious anemia.
- Patchy losses of myelin in the dorsal and lateral columns.
- Present with progressive weakness of legs, arms, trunk,
tingling and numbness. - Visual & Mental changes may also be present.
- Bilateral spastic paresis may develop and pressure, vibration
and touch sense are diminished.
Von Hippel-Lindau?
Autosomal Dominant - chromosome 3
Retinal and cerebellar haemangiomas
- Cerebellar hemangiomas (Can secrete crythyropiotiene that causes secondary polycythemia)
- Retinal hemangioma: vitreous hemorrhage
- Renal cysts (premalignant)
- Pheochromocytoma
- Extra-renal cysts: epididymal, pancreatic,hepatic
- Endolymphatic sac tumours
Freidreich’s Ataxia?
Autosomal Recessive
Trinucelotide repeat disorder - GAA repeat, X25 gene, chromosome 9
Onset = 10-15 yrs
Neuro Features
- Absent ankle jerks/extensor plantars
- Cerebellar ataxia
- Optic atrophy
- Spinocerebellar tract degeneration
Other Features
- Hypertrophic obstructive cardiomyopathy (90%, most common cause of death)
- Diabetes mellitus (10-20%)
- High-arched palate
Tuberous Sclerosis?
Autosomal Dominant
Cigarettes and coffee with a rough stupid person with a butterfly on his nose while he is dancing
Cutaneous Features
- Depigmented ‘ash-leaf’ spots which fluoresce under UV light
- Roughened patches of skin over lumbar spine (Shagreen patches)
- Adenoma sebaceum: butterfly distribution over nose
- Fibromata beneath nails (subungual fibromata)
- Café-au-lait spots may be seen
Neurological Features
- Developmental delay
- Epilepsy (infantile spasms or partial)
- Intellectual impairment
Other Features
- Retinal hamartomas: dense white areas on retina (phakomata)
- Rhabdomyomas of the heart
- Gliomatous changes can occur in the brain lesions
- Polycystic kidneys, renal angiomyolipomata
Neurofibromatosis general details?
Autosomal Dominant
NF1
- Chromosome 17 (17 characters in NF)
NF2
- Chromosome 22 (all the 2s)
Neurofibromatosis features?
NF1
- Café-au-lait spots (= 6, 15 mm in diameter)
- Axillary/groin freckles
- Peripheral neurofibromas
- Iris: Lisch nodules in > 90%
- Scoliosis
NF2
- Bilateral acoustic neuromas
Hereditary Sensorimotor Neuropathy (Charcot-Marie-Tooth Disease)?
Autosomal Dominant
- Defect in PMP-22 gene - codes for myelin
- Onset - puberty
- Motor symptoms predominate
- Distal muscle wasting, pes cavus (high arched), clawed toes
- Foot drop, leg weakness often first features
- Nerve Conduction Velocity is greatly reduced <30m/second
MND Patterns?
Amyotrophic Lateral Sclerosis
- Typically LMN signs in arms and UMN signs in legs
Primary Lateral Sclerosis
- UMN signs only
Progressive Muscular Atrophy
- LMN signs only
- Affects distal muscles before proximal
- Carries best prognosis
Bulbar Palsy
- Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function
of brainstem motor nuclei - Carries worst prognosis
Features of MND?
- Fasciculation
- Absence of sensory signs/symptoms
- LMN signs in arms and UMN signs in legs
- Wasting of the small hand muscles/tibialis anterior is common
- Doesn’t affect external ocular muscles
- No cerebellar signs
- Abdominal reflexes usually preserved
Lesions producting both UMN and LMN signs?
- Subacute combined degeneration of the cord
- Motor neuron disease
- Friedreich’s ataxia
- Syringomyelia
- Syringobulbia
- Taboparesis (syphilis)
- Conus medullaris lesion
Drugs for generalised vs partial seizures?
Generalised = sodium valproate –> lamotrigine, carbamezapine
Partial = carbamezapine –> lamotrigine, sodium valproate, gabapentin
Absence seizures?
Mostly seen in kids
3-10 years, girls 2x more than boys
Features
- Absences last a few seconds and are associated with a quick recovery
- Seizures may be provoked by hyperventilation or stress
- The child is usually unaware of the seizure
- They may occur many times a day
- EEG: bilateral, symmetrical 3Hz (spike and wave) pattern
Management
- Sodium valproate and ethosuximide are first-line treatment
- Good prognosis - 90-95% become seizure free in adolescence
Stopping AEDs?
AED cessation can be considered if seizure free for > 2 years – Stop AEDs over 2-3 months
AEDs in pregnancy?
- Sodium valproate: associated with neural tube defects
- Phenytoin: associated with cleft palate - need VIT K in last month of pregnancy
- Lamotrigine: studies to date suggest the rate of congenital malformations may be low. The dose of lamotrigine may need to be increased in pregnancy
- Carbamazepine: often considered the least teratogenic of the older antiepileptics
Breast feeding considered safe
Seizure features suggestive of NEAD?
- Asynchronous limb movements
- Undulating motor activity
- Purposeful movements
- Rhythmic pelvic movements
- Side-to-side head shaking
- Biting the tip of the tongue (as opposed to the side)
- Ictal crying
- Vocalisation during the ‘tonic–clonic’ phase
- Closed eyelids, resistance to eyelid opening
- Lack of cyanosis and rapid post-ictal reorientation
Frequency of tremor in PD?
3-5 Hz
Differences between drug induced parkinsonism and PD? Causes of DIP?
In DIP…
- Motor symptoms are generally rapid onset and bilateral
- Rigidity and rest tremor are uncommon
Causes
- Phenothiazines: e.g. Chlorpromazine
- Butyrophenones: haloperidol, droperidol
- Metoclopramide
PD Drugs?
Dopamine Receptor Agonists (>75 yrs)
- Bromocriptine, Pramipexole, Ropinirole, Cabergoline, Apomorphine
- Associated with fibrosis (pulm, retroperitoneal, cardiac) - Ropinirole least so
- Can cause impulse control disorders and excessive daytime somnolence
L-Dopa (<75 yrs)
- Combined with decarboxylase inhibitor (prevents peipheral metabolism)
- Decreased effectiveness over time
MAO-B Inhibitors
- Selegiline
- Inhibit breakdown of dopamine
Amantadine
- Increases dopamine release and inhibits uptake at synapses
COMT Inhibitors
- Entacapone
- COMT is an enzyme involved in dopamine breakdown - used as an adjunct in levodopa therapy
- Used in established PD
Multiple system atrophy?
Features
- Parkinsonism
- Autonomic disturbance (atonic bladder, postural hypotension)
- Cerebellar signs
Example = Shy-Drager syndrome
Progressive supranuclear palsy?
Parkinsonism, impairment of vertical gaze
- Gradual deterioration and death of selected areas of the brain
- Features
- Impairment of vertical gaze
- Parkinsonism
- Falls
- Slurring of speech
- Cognitive impairment
May complain of difficulty reading or descending stairs
CJD Features?
- Rapidly progressive, severe and invariably fatal (usually within few months)
- Dementia
- Cerebellar ataxia
- Defuse myoclonic jerks
- Other neurological abnormalities.
Investigation results in CJD?
- The EEG patern is characteristic (diffuse non specific slowing periodic sharp wave complexes- PSWCs of 1 – 2 Hz), but diagnosis relies on either espiecalized tests for prion protein in CSF or direct brain biopsy.
- Pulvinar Sign on cranial MRI > 90% pathological in vCJD (Not Sporadic)
- Prion protien in tonsils
- 14-3-3 protien in CSF
Hemiballism?
- Involuntary, sudden, jerking movements which occur contralateral to the side of the lesion
- Primarily affect the proximal limb musculature whilst the distal muscles may display more choreiform-like movements.
- Symptoms may decrease during sleeping.
- Etiology: stroke in elders, infection or inflammatory in young.
- Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment. Tetrabenazine may be considered when long-term therapy is required.
Causes of Chorea?
- Huntington’s disease, Wilson’s disease, ataxic telangiectasia
- SLE, anti-phospholipid syndrome
- Rheumatic fever: Sydenham’s chorea
- Drugs: oral contraceptive pill, L-dopa, antipsychotics
- Chorea gravidarum
- Thyrotoxicosis
- Polycythemia rubra vera
- Carbon monoxide poisoning
- Neuroacanthocytosis
- Cerebrovascular disease
Types of MS?
Relapsing-remitting
- Characterized by unpredictable relapses followed by periods of months to years of relative quiet (remission) with no new signs of disease activity.
- 85–90% of individuals with MS.
Secondary progressive
- Initial relapsing-remitting MS, then begin to have progressive neurologic decline between acute attacks without any definite periods of remission.
- Median time between disease onset and conversion from relapsing-remitting to secondary progressive MS is 19 years.
Primary progressive
- 10–15%
- Never have remission after their initial MS symptoms. It is characterized by progression of disability from onset, with no, or only occasional and minor, remissions and improvements.
- Age of onset for the primary progressive subtype is later than other subtypes.
Progressive relapsing
- Describes those individuals who, from onset, have a steady neurologic decline but also suffer clear superimposed attacks. This is the least common of all subtypes.
Features of MS?
Visual
- Optic neuritis: common presenting feature
- Optic atrophy
- Uhthoff’s phenomenon: worsening of vision following rise in body temperature (hot bath)
- Internuclear ophthalmoplegia
Sensory
- Numbness
- Trigeminal neuralgia
- Lhermitte’s syndrome: paraesthesiae in
limbs on neck flexion
Motor
- Spastic weakness
Cerebellar
- Ataxia, Tremor
Others
- Urinary incontinence
- Sexual dysfunction
- Intellectual deterioration
Good prognosis features in MS?
- Female sex
- Young age of onset
- Relapsing-remitting disease
- Sensory symptoms
- long interval between first two relapses
Typical patient carries better prognosis than atypical presentation
Investigations in MS?
Diagnosis = demonstration of lesions dissemintated in time and space
MRI
- High signal T2 lesions
- Periventricular plaques
CSF
- Oligoclonal bands (and not in serum)
- Increased intrathecal synthesis of IgG
Visual evoked potentials
- Delayed, but well preserved wave form
Criteria for beta interferon in MS? Other drugs?
- Relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
- Secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
Reduces number of relapses and MRI changes.
However doesn’t reduce overall disability
- *Glatiramer acetate** - immunomodulating drug
- *Natalizumab** - a recombinant monoclonal antibody that antagonises a4b1-integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium into parenchymal tissue
Argyll-Robertson pupil?
- small irregular pupils that do not react to light but react to accommodation
- Whore’s eye - associated with tertiary syphilis
- “Accommodate but do not react”
Other causes = MS, Sarcoidosis, DM
Location of lesion in Horner’s syndrome from location of anhydrosis?
(STC)
Gaze palsies?
Surgical third nerve palsy?
- Down and out
- Ptosis
- Pupil dilated (posterior communicating artery aneurysm) - can be PAINFUL
Weber’s syndrome?
ipsilateral third nerve palsy with contralateral hemiplegia - caused by midbrain strokes (cerebral peduncle)
Investigations in Myasthenia Gravis?
Tensilon test: IV edrophonium reduced muscle weakness temporarily
CT thorax to exclude thymoma
CK normal
Drugs that exacerbate myasthenia?
- Beta-blockers (theoretical worsening based on propensity to cause side effects of fatigue and weakness)
- Gentamicin, Aminoglycosides and Tetracycline
- Lithium
- Magnesium
- Penicillamine
- Phenytoin
- Quinidine, procainamide, verapamil, contrast agents.
- Chloroquine
- Prednisolone
Paraneoplastic syndromes affecting nervous system?
Lambert-Eaton myasthenic syndrome
- Associated with small cell lung cancer (also breast and ovarian)
- Antibody directed against pre-synaptic voltage gated calcium channel in PNS
- Can also occur independently as autoimmune disorder
- Increased muscle strength followed by weakness eventually.
Anti-Hu (imagine H sticks as 2 lungs or 2 brain hemispheres)
- Associated with small cell lung carcinoma and neuroblastomas
- Sensory neuropathy - may be painful
- Cerebellar syndrome
- Encephalomyelitis
Anti-Yo (imagine Y as lady’s private organ)
- Associated with ovarian and breast cancer
- Cerebellar syndrome
Anti-GAD antibody
- Associated with breast, colorectal and small cell lung carcinoma
- Stiff person’s syndrome or diffuse hypertonia
Anti-Ri
- Associated with breast and small cell lung carcinoma
- Ocular opsoclonus-myoclonus
Peripheral Neuropathy - axonal vs demyelination?
Peripheral neuropathy - sensory vs motor?
Nerve conduction studies
- Demyelination - Reduced conduction velocity, normal amplitude
- Axonal - Normal conduction velocity, reduced amplitude
Drugs causing peripheral neuropathy?
- Antibiotics: nitrofurantoin, metronidazole
- Amiodarone
- Isoniazid
- Vincristine and most of the anti-cancer chemotherapy
- Tricyclic antidepressants
Features of HSV Encephalitis? Pathophysiology? Investigation?
Features
- Fever, headache, psychiatric symptoms, seizures,
vomiting - Focal features e.g. Aphasia
- Peripheral lesions (e.g. Cold sores) have no relation
to presence of HSV encephalitis
Pathophysiology
- HSV-1 responsible for 95% of cases in adults
- Typically affects temporal and inferior frontal lobes
Investigation
- CSF: lymphocytosis, elevated protein
- PCR for HSV
- CT: medial temporal and inferior frontal changes (e.g. Petechial hemorrhages) - normal in one-third of patients
- MRI is better
- EEG pattern: lateralised periodic discharges (2 Hz)
Sub-acute sclerosing panencephalitis?
Result of chronic measles infection
Features
- Initially: decline in proficiency in school.
- Followed by diffuse myoclonic jerks in association with focal and generalised seizures and visual deterioration due to choiroidoretinitis.
- Followed by pyramidal signs, rigidity and progressive unresponsiveness.
- Finally the patient lies decorticated followed by death.
HIV neurocomplications? (Generalised neurological disease)
-
Encephalitis
- May be due to CMV or HIV itself
- HSV encephalitis but is relatively rare in the context of HIV
- CT: edematous brain
-
Cryptococcus
- Headache, fever, malaise, nausea/vomiting, seizures, focal
neurological deficit - CSF: high opening pressure, India ink test positive
- CT: meningeal enhancement, cerebral edema
- Meningitis is typical presentation but may occasionally cause a space occupying lesion
- Headache, fever, malaise, nausea/vomiting, seizures, focal
-
Progressive multifocal leukoencephalopathy (PML)
- Widespread demyelination
- Due to infection of oligodendrocytes by human papovirus (jc virus)
- Symptoms, subacute onset : behavioural changes, speech, motor, visual impairment
- CT: single or multiple lesions, no mass effect, don’t usually
enhance, Low attenuation diffusely - MRI is better - high-signal demyelinating white matter lesions are seen in advanced HIV.
-
AIDS Dementia Complex
- Symptoms: behavioural changes, motor impairment
- CT: cortical and subcortical atrophy
HIV neurocomplications? (Focal neurological lesions)
-
Toxoplasmosis
- Constitutional symptoms, headache, confusion, drowsiness
- CT: usually single or multiple ring enhancing lesions, mass
effect may be seen - Management: sulfadiazine and pyrimethamine
-
Primary CNS Lymphoma
- Associated with the Epstein-Barr virus
- CT: single or multiple ring enhancing lesions
-
Tuberculosis
- Much less common
- CT: Single enhancing lesion
Myotonic Dystrophy?
- Inherited myopathy - develops at around 20-30 yrs
- Affects skeletal, cardiac and smooth muscle
- Autosomal Dominant
- Trinucleotide repeat disorder
DM1
- DMPK gene on chromosome 19
- Distal weakness more prominent
DM2
- ZNF9 gene on chromosome 3
- Proximal weakness more prominent
Features of myotonic dystrophy?
General
- Myotonic facies (long, ‘haggard’ appearance)
- Frontal balding
- Bilateral ptosis
- Cataracts
- Dysarthria
Other Features
- Myotonia (tonic spasm of muscle)
- Weakness of arms and legs (distal initially)
- Mild mental impairment
- Diabetes mellitus
- Testicular atrophy
- Cardiac involvement: heart block, cardiomyopathy
- Dysphagia
Management of cluster headache?
- Acute: 100% oxygen, subcutaneous sumatriptan (5-HT1D receptor agonist), nasal lidocaine
- Prophylaxis: verapamil, prednisolone
- Consider specialist referral
Normal CSF values?
- *Pressure** = 60-150 mm (patient recumbent)
- *Protein** = 0.2-0.4 g/l
- HIGH protein = TB, fungal, bacterial meningitis; viral encephalitis; Guillain Barre syndrome, Spinal block (Froin’s syndrome?
- *Glucose** = > 2/3 blood glucose
- *Cells**: red cells = 0, white cells < 5/mm³
- HIGH lymphocytes = viral meningitis/encephalitis, TB meningitis, partially treated bacterial meningitis, Lyme disease, Bechet’s, SLE, lymphoma, leukaemia
Features of post-LP headache? Factors which contribute?
- Usually develops within 24-48 hours following LP but may occur up to one week later
- May last several days
- Worsens with upright position
- Improves with recumbent position
Management
- Supportive initially (analgesia, rest)
If pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural hematoma - Blood patch, epidural saline, intravenous caffeine
