Neurosciences

Question 1

what causes increased tone?

Answer 1

UMN - spasticity, velocity dependent, clasp knife

extrapyramidal - velocity independent, cog-wheel

Question 2

what is the cause of increased and abnormal reflexes?

Answer 2

clonus

Question 3

where is the location of UMN?

Answer 3

cerebral cortex
brainstem
spinal cord (myelopathy)

Question 4

where is the location of LMN?

Answer 4

anterior horn cell
nerve roots (radiculopathy)
peripheral nerve (neuropathy)
nerusomuscular junction
muscle (myopathy)

Question 5

what are the general UMN signs?

Answer 5

increase tone (clasp-knife feel)
weakness of upper limb extensors and lower limb flexors
increased reflexes
extensor plantar
ankle clonus
Hoffmans sign
no muscle wasting

Question 6

describe spinal cord UMN weakness

Answer 6

absent facial weakness

Question 7

describe motor cortex UMN weakness

Answer 7

facial weakness on the same side as limb weakness

Question 8

describe brainstem UMN weakness

Answer 8

facial weakness on opposite side of limb weakness

Question 9

describe mid pons UMN weakness

Answer 9

jaw jerk

motor component of trigeminal nerve

Question 10

what are the general LMN signs?

Answer 10

muscle wasting
decreased tone
reduced/absent reflexes
flexor plantar

Question 11

describe anterior horn cell LMN weakness

Answer 11

fasciculation

wasting

Question 12

describe nerve root (radiculopathy) LMN weakness

Answer 12

pain
weakness
numbness in a particular myotome/dermatome

Question 13

describe peripheral nerve (neuropathy) LMN weakness

Answer 13

often length dependent (distal becoming proximal)
reflexes lost early
wasting ++
often sensory and autonomic involvement

Question 14

describe neuromuscular junction LMN weakness

Answer 14

fatiguable weakness
no pain
diurnal fluctuation
extra ocular involvement; ophthalmoplegia, ptosis, diplopia
bulbar muscle involvement; chewing, swallowing, nasal speech
most common condition; myasthenia gravis

Question 15

describe myopathy LMN weakness

Answer 15

often proximal weakness
symmetrical weakness
can be painful
reflexes spared until late
\+/- wasting (late)
may have pseudohypertrophy

Question 16

describe the dorsal column

Answer 16

carriers sensory signals of proprioception and vibration

stays on the same side as the receptor and crosses at the medulla

Question 17

describe the spinothalamic tracts

Answer 17

carries sensory signal of pain, temperature and fine touch

crosses to the opposite site immediately when it enters the spinal cord

Question 18

describe Guillain-Barre syndrome

Answer 18

acute paralytic polyneuropathy
LMN
associated with campylobacter jejuni, cytomegalovirus, epstein barr virus

B cells create antibodies against the antigens on the pathogen
these may target proteins on the myelin sheath of the motor never cell or the nerve axon

Question 19

what are the symptoms and signs of Guillain-Barre syndrome?

Answer 19

reduced reflexes
symmetrical ascending weakness
peripheral loss of sensation
neuropathic pain
cranial nerve (facial) weakness

symptoms usually start within 4 weeks of the preceding infection

Question 20

what is the management of Guillain-Barre syndrome?

Answer 20

IV immunoglobulins/plasma exchange
supportive care
VTE prophylaxis
respiratory failure - intubation, ventilation, ICU admission

Question 21

describe posterior spinal cord lesion

Answer 21

dorsal column impairment
intact spinothalamic tracts
flexor muscle weakness in both legs
symmetrical
brisk reflexes

Question 22

describe a spinal cord lesion affecting the whole cord at about T10

Answer 22

increasing difficulty walking over the last few weeks
weakness of the flexor muscles in the legs
brisk reflexes
all sensory modalities impaired in the legs
light touch and pinprick sensation impaired onto the abdomen up to the umbilicus

Question 23

describe myasthenia gravis

Answer 23

neuromuscular junction disorder
autoimmune production of AChR antibodies which bind to the postsynaptic NMJ receptors - less effective stimulation of muscle with increased activity
muscle weakness that gets progressively worse with activity and improves with rest
typically women <40 and men >60
strong link with thymoma

Question 24

what antibodies are responsible for myasthenia gravis?

Answer 24

AChR
MuSK
LRP4

Question 25

what are the symptoms and signs of myasthenia gravis?

Answer 25

``` fatiguability; clinical hallmark diplopia ptosis ophthalmoplegia facial movement weakness dysphagia fatigue in the jaw when chewing slurred speech neck extension/flexion weakness proximal limb weakness finger extensor weakness no wasting, reflexes or sensory involvement progressive weakness with repetitive movements diurnal variation ```

Question 26

describe brown sequard syndrome

Answer 26

hemisection of half the spinal cord due to injury ipsilateral side - loss of vibration, motor function, deep touch, position sense contralateral side - loss of pain, temperature, fine touch

Question 27

name the primary headaches

Answer 27

migraine tension type trigeminal autonomic cephalgias

Question 28

name the secondary headaches

Answer 28

``` head and neck trauma cranial or cervical vascular disorders non-vascular intracranial disorders a substance or its withdrawal infection disorder of homeostasis disorder of neck, cranium, eyes, ears, nose, sinuses, teeth or mouth psychiatric disorder ```

Question 29

describe the symptoms of a tension type headache

Answer 29

``` bilateral pressing/tightening non-pulsating mild-moderate pain lasts 30 minutes continuous ```

Question 30

what is the difference between an episodic or chronic tension type headache or migraine?

Answer 30

episodic - <15 days/month | chronic - >15 days/month for over 3 months

Question 31

describe the symptoms of a migraine

Answer 31

unilateral or bilateral pulsating moderate-severe pain photosensitivity, hyperacusis, nausea, vomiting aura - flickering lights, spots, lines, partial loss of vision, numbness, pins and needles, speech disturbance lasts up to 72 hours

Question 32

what are the symptoms of a cluster headache?

Answer 32

``` unilateral (around the eye, above the eye and along the side of the head/face) can be sharp, boring, burning, throbbing or tightening severe-very severe pain restlessness agitation red eye watery eye nasal congestion runny nose swollen eyelid forehead and facial sweating constricted pupil drooping eyelid lasts 15-180 minutes ```

Question 33

what is the treatment of tension-type headaches?

Answer 33

aspirin paracetamol NSAID do not offer opioids chronic - 10 sessions of acupuncture over 5-8 weeks, amitriptyline 10-75mg

Question 34

what is the treatment of an episodic migraine?

Answer 34

fluids oral paracetamol 1g, naproxen 250mg, aspirin 900mg, metoclopramide 10mg, voltarol 100mg PR, triptan IV metoclopramide 10mg, paracetamol 1g, magnesium sulphate 500-1000mg, aspirin 900mg nerve blocks realistic expectations

Question 35

what is the treatment of a chronic migraine?

Answer 35

``` amitriptyline 10-150mg propanolol 80-240mg topiramate 25-100mg candersartan 4-16mg pizotifen 0.5-4.5mg ``` botox - inhibits the release of peripheral nociceptive neurotransmitters, must have failed on 3 preventers electrical neuromodulation single-pulse transcranial magnetic stimulation non-invasive vagus nerve stimulation

Question 36

describe the features of trigeminal autonomic cephalgias

Answer 36

``` unilateral ptosis conjunctival injection lacrimation nasal congestion rhinorrhea restlessness severe pain ```

Question 37

describe the acute treatment and prevention of a cluster headache

Answer 37

acute treatment - sumatriptan sc, high flow oxygen, nerve blocks prevention - verapamil, topiramate, lithium

Question 38

what may be the causes of systemic symptoms associated with headaches?

Answer 38

``` fever weiht loss fatigue HIV cancer immunosuppression ``` infection inflammation metastatic cancer carcinomatous meningitis

Question 39

what may be the causes of neurologic symptoms/signs with headaches; altered consciousness, focal deficits?

Answer 39

encephalitis mass lesion stroke

Question 40

what may be the causes of a thunderclap or abrupt onset of headache?

Answer 40

SAH IPH RCVS

Question 41

what may be the cause of a new onset headache >50?

Answer 41

temporal arteritis

Question 42

what may be the cause of a positional headache, prior HA, papilloedema?

Answer 42

intracranial hypertension dysautonomia cervicogenic headache posterior fossa pathology

Question 43

what are the symptoms and risk factors for a SAH?

Answer 43

thunderclap headache ``` female seizures vomiting LOC focal neurology ```

Question 44

what are the secondary causes of a thunderclap headache?

Answer 44

``` intracerebral or subarachnoid haemorrhage vertebral dissections acute hypertension acute low CSF pressure cough micturition colloid cyst of the third ventricle reversible cerebral vasoconstrictive syndrome psychiatric conditions (panic disorder) ```

Question 45

what are the causes of meningitis?

Answer 45

``` bacterial viral fungal rickettsial (Rocky Mountain spotted fever) parasitic protozoal cancer physical injury certain drugs (NSAIDs) ```

Question 46

what are the symptoms and signs of meningitis?

Answer 46

``` mild fever headache with sensitivity to light/sound stiff neck loss of appetite muscle aches kernig's and brudzinski sign ```

Question 47

what are the symptoms and signs of a cervical artery dissection?

Answer 47

``` headache ataxia vertigo vomiting numbness on one side of face horner's syndrome ```

Question 48

describe a headache caused by a brain tumour

Answer 48

<1% of patients have only headache pain worse with valsalva manoeuvre, exertion, bending over often awaken from sleep

Question 49

describe the intracranial compartments

Answer 49

supratentorial - frontal, temporal, parietal, occipital lobes, hypothalamus, thalamus infratentorial - brainstem (midbrain, pons, medulla), cerebellum

Question 50

describe the craniospinofluid pathways

Answer 50

ventricular system | subarachnoid space

Question 51

what are the functions of the frontal lobe?

Answer 51

``` motor function speech high cognitive function micturition personality ```

Question 52

what are the functions of the parietal lobe?

Answer 52

sensory awareness spatial awareness visual tracts

Question 53

what are the functions of the temporal lobe?

Answer 53

vision receptive speech memory

Question 54

what is the primary function of the occipital lobe?

Answer 54

vision

Question 55

what is the primary function of the cerebellum?

Answer 55

coordination

Question 56

what is normal ICP in the resting state?

Answer 56

10mmHg

Question 57

describe an extradural haematoma

Answer 57

high density biconcave shape adjacent to the skull often associated with a lucid interval between initial impact and onset of neurological deterioration can be caused by focal trauma good prognosis

Question 58

describe a subdural haematoma

Answer 58

crescentic mass of increased attenuation adjacent to inner table of the skull rotational movement and tearing of the bridging veins between the brain and the dura

Question 59

describe skull fractures

Answer 59

open - scalp laceration depressed - lies below adjacent bone comminuted - multiple fragments

Question 60

describe gliomas

Answer 60

tumours of glial cell; astrocytoma, oligodendroglioma, ependymoma median survival 1 year following resection, radiotherapy and chemotherapy

Question 61

describe meningiomas

Answer 61

arise from meninges (arachnoid) primary goal - surgical excision recurrence - repeat surgery or radiation

Question 62

describe pituitary adenomas

Answer 62

macro and microadenomas | compression of adjacent structures; optic nerve (bitemporal hemianopia)

Question 63

name some examples of pituitary microadenomas

Answer 63

prolactinoma GH-secreting (acromegaly) ACTH (Cushing's)

Question 64

define a vestibular schwannoma

Answer 64

slow-growing benign nerve sheath tumours arising on the vestibular nerve

Question 65

what are the symptoms and signs of a vestibular schwannoma

Answer 65

hearing loss tinnitus balance problems associated with neurofibromatosis

Question 66

describe stereotactic radiosurgery

Answer 66

use stereotactic localisation to precisely focus a large dose of radiation onto a lesion, usually in a single treatment

Question 67

define hydrocephalus

Answer 67

imbalance in the normal CSF production vs resorption process

Question 68

describe communicating hydrocephalus

Answer 68

CSF circulation blocked at the level of arachnoid granulations 4th ventricle open CSF malabsorption

Question 69

describe obstructive/non-communicating hydrocephalus

Answer 69

blockage proximal to arachnoid granulations enlargement of ventricles proximal to blockage e.g. posterior fossa tumour

Question 70

what is the treatment of hydrocephalus?

Answer 70

ventriculoperitoneal shunt

Question 71

what is the treatment of a cerebral aneurysm?

Answer 71

endovascular occlusion using coils | open surgical approach of applying a clip across the aneurysm neck

Question 72

define an arteriovenous malformation

Answer 72

an abnormal collection of blood vessels wherein arterial blood flows directly into draining veins without the normal interposed capillary beds causes an intraventricular bleed

Question 73

what is the treatment of arteriovenous malformation?

Answer 73

surgery embolisation stereotactic radiosurgery

Question 74

what are the risk factors and symptoms of intracranial infection?

Answer 74

previous trauma recurrent sinus infection infected heart valves poor dental hygiene headache drowsiness seizures fever

Question 75

what are the pathologies of intracranial infection?

Answer 75

meningitis brain abscess subdural empyema

Question 76

what is the treatment of intracranial infection?

Answer 76

urgent surgical drainage/evacuation prolonged course of antibiotics regular clinical, serological and imaging surveillance until infection resolves

Question 77

define cauda equina syndrome

Answer 77

neurosurgical emergency due to sphincter dysfunction | compression of nerve roots in lumbosacral spine

Question 78

what are the symptoms and signs of cauda equina syndrome?

Answer 78

``` radicular pain parasthesia weakness perianal numbness bladder and sphincter disturbance ```

Question 79

what are the underlying pathologies of cauda equina syndrome?

Answer 79

tumours degenerative disease infection trauma

Question 80

define epilepsy

Answer 80

a chronic disease of the brain associated with 1 of the following; > 2 unprovoked seizures >24hrs apart a single unprovoked seizure with a >60% chance of having another within 10yrs a diagnosed epilepsy syndrome

Question 81

describe genetic, primary, or idiopathic epilepsy

Answer 81

``` known or presumed genetic etiology associated with; childhood onset good response to AEDs favourable prognosis "normal brain" ```

Question 82

describe structural/metabolic, secondary or symptomatic epilepsy

Answer 82

``` caused by a distinct structural or metabolic condition associated with; onset at any age variable response to AEDs variable prognosis brain pathology ```

Question 83

describe focal-onset seizures

Answer 83

originate within networks limited to one hemisphere of the brain may be discretely localised or widely distributed can be describe as dyscognitive can evolve to bilateral, convulsive seizures

Question 84

describe generalised-onset seizures

Answer 84

originate at some point with and rapidly engage bilaterally distributed networks can include cortical and subcortical structures but no the entire cortex can present as tonic, clonic, tonic-clonic, myoclonic, absences, atonic

Question 85

describe generalised tonic clonic seizures

Answer 85

start with a sudden loss of consciousness generalised stiffening of the body (tonic) followed by muscle contraction (clonic)

Question 86

describe absence seizures

Answer 86

``` usually during childhood or adolescence momentary loss of consciousness 3-15s brief staring spells eyelid flutter often undetected ```

Question 87

describe myoclonic seizures

Answer 87

starts in adolescence and persists throughout adulthood sudden, brief arrhythmic jerks of arms and other extremities associated with a fall no loss of consciousness shortly after awakening may be triggered by photic or sensory stimulation or sleep deprivation

Question 88

what is the treatment of juvenile myoclonic epilepsy?

Answer 88

1st line sodium valproate | consider lamotrigine, levetiracetam, topiramate if sodium valproate is unsuitable or not tolerated

Question 89

define status epilepticus

Answer 89

``` continuous or rapidly repeating seizures abnormally prolonged consider if seizures >5mins or 2 discrete seizures with no regaining of consciousness inbetween ```

Question 90

what is the treatment of status epilepticus?

Answer 90

0-10 mins; ABCDE, oxygen | 1-60 mins; monitoring, emergency AED, IV lines, investigation, glucose and thiamine?, treatment of acidosis?

Question 91

what is the treatment of convulsive status epilepticus?

Answer 91

``` early status (0-10/30mins); lorazepam 0.07mg/kg, repeated once after 20mins established status (10/30-60/120mins); phenytoin 20mg/kg at 50mg/min refractory status; general anaesthetic with either propofol, midazalom or thiopentone ```

Question 92

define MS

Answer 92

chronic, immune mediated, inflammatory, demyelinating disease of theCNS clinical and radiological episodes occurring over time

Question 93

what are the risk factors for developing MS?

Answer 93

``` EBV genetics smoking obesity female ```

Question 94

what are the functions of astrocytes?

Answer 94

secretion and absorption of neurotransmitters maintenance of the blood-brain barrier nutritional and metabolic support

Question 95

describe the pathogenesis of MS

Answer 95

inflammation demyelination axonal injury and neurodegeneration incomplete remyelination demyelinating ability decreased with age brain atrophy meningeal inflammation cortical demyelination

Question 96

what are the consequences of increased meningeal inflammation and cortical demyelination?

Answer 96

``` more severe disease course more rapid decline in cognitive function accumulation of disability younger age of death enter progressive stages sooner ```

Question 97

what are the features of an MS relapse?

Answer 97

an episode of new symptoms or a worsening of an existing MS symptoms over days/weeks usually followed by in/complete recovery over weeks/months

Question 98

what are the common symptoms of MS?

Answer 98

``` weakness sensory symptoms visual loss incoordination vertigo bladder; frequency, urgency, incontinence bowels; constipation, urgency, incontinence fatigue pins and needles neuropathic pain sexual dysfunction depression anxiety cognitive deficits ```

Question 99

what are the common clinical presentations in RRMS?

Answer 99

optic neuritis transverse myelitis brainstem symptoms differentiated in time and space

Question 100

describe the signs and symptoms of optic neuritis

Answer 100

unilateral visual (acuity) loss loss of colour vision retrobulbar eye pain, exacerbated on eye movement develops over days and starts to improve within 2 weeks papilloedema afferent pupillary defect (dilation rather than constriction) optic atrophy

Question 101

describe the signs and symptoms of transverse myelitis

Answer 101

area of inflammation within the spinal cord evolution of symptoms over days weakness sensory symptoms bladder/bowel symptoms Lhermitte's phenomenon; electric shock sensation down spine on neck flexion (cervical cord lesion)

Question 102

describe brainstem symptoms

Answer 102

``` internuclear ophthalmoplegia (INO); failure of eye adduction, lesion in medial longitudinal fasciculus diplopia vertigo facial/limb weakness/sensory loss trigeminal neuralgia nystagmus ataxia dysarthria dysphagia cranial nerve palsies ```

Question 103

what investigations are performed to diagnose MS?

Answer 103

blood tests; exclude other neuroinflammatory conditions (ANA, dsANA, anti-cardiolipin, ACE) CSF; unmatched oligoclonal bands, not MS specific MRI; typically juxtacortical, periventricular, infratentorial, spinal cord VERs; delayed in optic neuritis

Question 104

what are the aims of MS treatment?

Answer 104

prevention of relapses slowing rate of accumulating disability treatment of an acute relapse symptomatic treatment of fatigue, neuropathic pain, bladder/bowel dysfunction etc.

Question 105

what are the disease modifying treatments of RRMS?

Answer 105

active - interferon beta, glatiramer acetate, peginterferon beta highly active - natalizumab, ocrelizumab, alemtuzumab, cladribine

Question 106

describe the treatment approaches for progressive MS

Answer 106

targeting T and B lymphocytes; ocrelizumab, rituximab mitochondrion protective strategies; minocycline, chelating drugs, antioxidants anti-inflammatory strategies; riluzole, memantine, amantadine, siponimod, minocycline

Question 107

describe some of the treatment approaches for CNS remyelination

Answer 107

``` amiloride at ASIC1 lamotrigine at Na channels opicinumab at LINGO1 domeperidone at PRLR clementine, benztropine and quetiapine at M1 vitamin D at VDR clobetasol at GR liothyronine at THR ```

Question 108

describe polyneuropathies

Answer 108

``` longest nerves affected first (distal) symmetrical weakness +/- sensory loss reflexes absent distal wasting pes cavus ```

Question 109

describe motor neurone disease

Answer 109

degeneration of cortical pyramidal cells, cortico-spinal pathways, brainstem motor nuclei (V, VII, X, XII) and anterior horn cells painless progressive weakness and wasting normal eye movements normal sensation combination of UMN and LMN signs

Question 110

describe fasciculations

Answer 110

spontaneous firing os a single motor unit active denervation usually MND or a very proximal nerve lesion can be benign; physical, psychological stress, caffeine

Question 111

describe mononeuropathy

Answer 111

weakness affecting one nerve's muscles | sensory loss affecting one nerve

Question 112

describe peripheral neuropathy

Answer 112

absent/reduced reflexes distal, symmetrical sensory loss foot drop, high steppage gait e.g. Charcot marie tooth

Question 113

describe MND findings

Answer 113

increased reflexes despite other LMN signs | no sensory loss

Question 114

describe myopathy

Answer 114

reduced/normal reflexes no sensory loss trendelenburg/waddling gait symmetrical proximal weakness

Question 115

what are the causes of high CK?

Answer 115

``` black/male post-high intensity exercise rhabdomyolysis muscular dystrophy denervation sepsis renal failure malignancy ```

Question 116

what LMN disorders do not have sensory loss?

Answer 116

myopathy MND myasthenia

Question 117

what are the signs and symptoms of Charcot Marie tooth disease?

Answer 117

``` pes cavus distal muscle wasting; champagne bottle weakness of lower legs weakness of hands reduced tendon reflexes reduced muscle tone peripheral sensory loss ```

Question 118

what are the causes of Charcot Marie tooth disease?

Answer 118

genetic ``` alcohol B12 deficiency cancer and CKD diabetes and drugs (isoniazid, amiodarone, cisplatin) every vasculitis ```

Question 119

what are the symptoms and signs of amyotrophic lateral sclerosis?

Answer 119

``` cognitive and behavioural impairment dysphagia dysarthria respiratory insufficiency muscle cramps spasticity muscle weakness and atrophy ``` motor neurone disease

Question 120

what are the acquired causes of myopathy?

Answer 120

autoimmune endocrine; thyroid, parathyroid, Addison's, cushing's toxic; corticosteroids, colchicine, chloroquine, alcohol, statins viral; HIV, HCV, influenza metabolic; Ca2+/K+ critical illness myopathy

Question 121

describe dermatomyositis

Answer 121

``` inflammatory myopathy gottron papules periorbital oedema heliotrope rash V sign shawl sign mechanics hands acute/subacute proximal weakness myalgia CK 10-50 x normal associated with malignancy; lung, breast, ovarian, lymphoma perifascicular and perimysial atrophy on biopsy ```

Question 122

what is the treatment of dermatomyositis?

Answer 122

oral prednisolone 0.5-1 mg/kg/day acute and severe - IVMP for 3-5 days long-term immunosuppressants, used in parallel with steroids; methotrexate, azathioprine, mycophenolate if not tolerated/effective; IVIG, ciclosporin 3rd line; rituximab, cyclophosphamide

Question 123

describe inclusion body myositis

Answer 123

late onset progressive myopathy (50-60yrs) chronic sporadic cause unknown m>f usually wheelchair use within 10yrs of onset does not affect life expectancy

Question 124

what are the symptoms and signs of inclusion body myositis?

Answer 124

``` predominantly proximal weakness and wasting symmetrical reflexes spare some thigh atrophy dysphagia common (aspiration pneumonia) no response to steroids CK increased but <12x normal ``` biopsy; endomysial inflammatory infiltrates, partial invasion of fibres, rimmed vacuoles. amyloid deposits on electron microscopy

Question 125

what are the causes of neuromuscular junction dysfunction?

Answer 125

``` myasthenia gravis Lambert eaton myasthenia syndrome organophosphate poisoning botulinum toxin congenital myasthenic syndromes ```

Question 126

describe myasthenia gravis

Answer 126

autoimmune disorder affecting NMJ 80% AChR antibodies 10-15% MuSK antibodies 5-10% double seronegative MG degradation and endocytosis of AChRs, degeneration of the motor end-plate and loss of postsynaptic folds reduced muscle membrane depolarisation increased in the action potential threshold

Question 127

what are the clinical features of myasthenia gravis?

Answer 127

``` young women and old men fatiguability diurnal variation; fluctuating nature ptosis ophthalmoplegia neck extension/flexion weakness proximal limb weakness finger extensor weakness no wasting reflexes preserved no sensory involvement ```

Question 128

what investigations are required to diagnose myasthenia gravis?

Answer 128

AChR antibody MuSK antibody ice pack test tensilon test edrophonium IV; assess objective improvement in muscle strength neurophysiology; repetitive nerve stimulation, single fibre EMG CT chest; exclude thymoma

Question 129

what is the management of myasthenia gravis?

Answer 129

``` pyridostigmine steroids steroid-sparing agents; azathioprine, ciclosporin, methotrexate IVIg plasmapheresis (PLEX) rituximab ```

Question 130

what factors exacerbate myasthenia gravis?

Answer 130

``` infection stress; trauma, post-operative cholinesterase inhibitors withdrawal rapid introduction/increase of steroids electrolyte imbalance anaemia antibiotics penicillamine anaesthetic agents quinidine beta blockers calcium channel blockers lithium chlorpromazine phenytoin botox chemotherapy; cisplatin ```

Question 131

describe myasthenia crisis

Answer 131

``` increasing muscle weakness diplopia respiratory failure oxygen saturation and blood gases can be normal until later FVC; >1L or 15ml/kg requires ICU support ```

Question 132

what is the management of myasthenia crisis?

Answer 132

IVIg or plasma exchange ventilatory support NBM NG feeding

Question 133

define polyneuropathies

Answer 133

length dependent (distal( symmetrical weakness/sensory loss

Question 134

what is the differential diagnosis of polyneuropathy?

Answer 134

``` diabetes vitamin deficiencies (copper, B12, D, E) hyper/hypothyroidism toxins, lead, nitrous oxide drugs; cisplatin hereditary infectious; HIV, EBV, CMV, lyme disease inflammatory SLE, sjogren's, behcet's paraneoplastic; myeloma, MGUS acute/chronic immune demyelination polyneuropathy ```

Question 135

define Guillain Barre syndrome

Answer 135

acute inflammatory demyelinating polyneuropathy

Question 136

what are the triggers for developing Guillain barre syndrome?

Answer 136

CMV mycoplasma pneumoniae campylobacter jejuni

Question 137

what are the clinical features of GBS?

Answer 137

``` progressive weakness; hours-days areflexia peak <4 weeks relatively symmetrical motor - sensory involvement pain autonomic dysfunction facial involvement bulbar ```

Question 138

what investigations are required to diagnose GBS?

Answer 138

``` CSF; increased protein NCS; demyelination MRI spine; rule out structural cause CK; rule out muscle disease TFTs K bone B12 folate ```

Question 139

what is the treatment of GBS?

Answer 139

``` discuss with neurology IVIG/PLEX supportive care with airway support MDT; SALT, physio enoxaparin nursing care ``` 10% mortality; neuromuscular respiratory failure

Question 140

define Charcot marie tooth disease

Answer 140

general term for a group of inherited sensorimotor neuropathies dominant (CMT1A), recessive or X-linked

Question 141

what are the clinical features of Charcot Marie tooth disease?

Answer 141

gradual, progressive, length dependent weakness and wasting foot deformity; from childhood, pes cavus Champagne bottle deformity upper limb involvement eventually no cardiac or respiratory involvement no current treatment

Question 142

describe motor neurone disease

Answer 142

fatal degenerative condition degeneration of cortical pyramidal cells, corticosteroids-spinal pathways, brainstem motor nuclei (V, VII, X, XII) and anterior horn cells

Question 143

what are the subtypes of MND?

Answer 143

amyotrophic lateral sclerosis; most common, mixture of UMN and LMN signs progressive muscular atrophy; LMN signs only progressive bulbar palsy; bulbar dysfunction primary lateral sclerosis; UMN signs only

Question 144

what are the clinical features of MND?

Answer 144

``` pain progressive weakness and wasting normal eye movements jaw weakness brisk jaw jerk focal weakness gag reduced/exaggerated bulbar/pseudobulbar palsy; bad prognostic sign tongue fasciculations head drop limbs UMN/LMN; triceps weak and wasted, fasciculations, brisk reflex cachexia normal sensation cognition; frontal involvement ```

Question 145

what is the differential diagnosis of MND?

Answer 145

multifocal motor neuropathy cervical myeloradiculopathy MG; MND has no ocular involvement inherited motor neuropathies; MND more rapidly progressive

Question 146

what investigations are required to diagnose MND?

Answer 146

``` MRI spine nerve conduction studies electromyography exclude metabolic, endocrine, infectious causes AChR antibodies genetic testing; FHx ```

Question 147

what is the management of MND?

Answer 147

``` SALT nurse specialist PEG/RIG; gastroenterology, dietician NIV; respiratory palliative; symptom control riluzole ```

Question 148

describe the lateral cord of the median nerve

Answer 148

made up of C6-7 fibres sensation; thenar eminence, thumb, index and middle fingers motor; proximal median muscles of the forearm

Question 149

describe the medial cord of the median nerve

Answer 149

made up of C8-T1 fibres sensation; lateral 1/2 of ring finger motor; median muscles of distal forearm and hand

Question 150

describe carpal tunnel syndrome

Answer 150

most common entrapment neuropathy f>m C6-7 nerve root lesion brachial plexus lesion proximal median nerve lesion

Question 151

what are the symptoms and signs of carpal tunnel syndrome?

Answer 151

``` paraesthesia in the median distribution worse on wakening motor symptoms loss of dexterity 55-65% bilateral on first presentation mostly dominant hand more severely affected ```

Question 152

what are the risk factors for developing carpal tunnel syndrome?

Answer 152

``` small carpal tunnel sex fractures DM inflammatory conditions; RA hypothyroidism renal disorders occupational ```

Question 153

what are the findings on an examination on a patient with carpal tunnel syndrome?

Answer 153

thenar eminence may be spared; innervation from the palmar cutaneous sensory branch positive tinels and phalens test wasting of thenar eminence weakness of thumb abduction and opposition

Question 154

what is the differential diagnosis of carpal tunnel syndrome?

Answer 154

``` C6/7 root lesion brachial plexus lesion proximal median nerve PN MND ```

Question 155

what are the clinical features of C6-7 nerve lesion?

Answer 155

reduced reflexes reduced power; EF, EE, arm pronation reduced sensation in palm and forearm

Question 156

what are the clinical features of a brachial plexus lesion?

Answer 156

similar weakness to cervical radiculopathy more widespread weakness reflex changes

Question 157

what are the clinical features of a proximal median nerve lesion?

Answer 157

very uncommon | weakness of proximal muscles; distal thumb flexion, arm pronation, WF

Question 158

what does the anterior interosseous nerve supply?

Answer 158

motor; flexor policus longus, flexor digitorum profundus (digits 2 and 3), pronator quadratus sensory; deep sensory fibres to wrist and interosseous membrame

Question 159

what are the causes of anterior interosseous neuropathy (AIN)?

Answer 159

fracture mid-shaft of radius penetrating injury to forearm excessive exercise brachial neuritis

Question 160

describe the pathway of the ulnar nerve

Answer 160

C8-T1 roots enters the ulnar groove between medial epicondyle and olecranon process travels under the two heads of flexor carpi ulnaris muscle

Question 161

what are the clinical features of ulnar neuropathy at the elbow?

Answer 161

``` decreased grip and pinch hypothenar eminence atrophy intrinsic muscles of hand atrophy wartenberg's sign; abducted 5th finger benediction posture sign sensory deficits on ulnar side ```

Question 162

what is the differential diagnosis of ulnar neuropathy?

Answer 162

C8/T1 radiculopathy lower trunk brachial plexopathy PN MND

Question 163

what are the clinical features of C8-T1 cervical radiculopathy?

Answer 163

sensory disturbance into forearm | weakness involving APB

Question 164

what are the clinical features of peripheral neuropathy?

Answer 164

bilateral wasting lower limbs PMHx DM

Question 165

what are the causes of a wrist drop?

Answer 165

``` CNS lesion C7/8 radiculopathy brachial plexopathy spiral groove PIN ```

Question 166

describe the radial nerve

Answer 166

receives innervation from all 3 trunks | innervates; triceps, brachioradialis, long head extensor carpi radials, supinator

Question 167

what are the clinical features of radial neuropathy?

Answer 167

``` wrist and finger drop mild weakness supination mild weakness EF EE spared ulnar power reduced sensory disturbance on lateral dorsum of hand ```

Question 168

what are the causes of radial nerve palsy?

Answer 168

Saturday night palsy | humeral fracture

Question 169

what are the clinical features of axillary nerve palsy?

Answer 169

tricep involvement; EE weakness wrist drop supinator weakness sensory loss in the distribution of proximal cutaneous branches

Question 170

define parkinsonism

Answer 170

the combination of bradykinesia, rigidity and impaired motor control

Question 171

define hemidallismus

Answer 171

flinging movements

Question 172

define dyskinesia

Answer 172

involuntary movement | fragmentary/incomplete

Question 173

define dystonia

Answer 173

prolonged spasms of muscle contraction

Question 174

define akathisia

Answer 174

unpleasant sensation of restlessness | inability to sit still or remain motionless

Question 175

what are the hypo kinetic movement disorders?

Answer 175

akinetic rigid syndrome idiopathic Parkinson's disease atypical Parkinsonism; MSA, PSP, CBD drug-induced Parkinsonism

Question 176

what are the hyperkinetic movement disorders?

Answer 176

``` dyskinesias essential tremor dystonia chorea hemiballismus tics myoclonus drug-induced dyskinesias ```

Question 177

describe the pathophysiology of PD

Answer 177

loss of dopamine containing neurones result in a pre-synaptic dopaminergic deficit presence of lewy bodies composed of abnormal protein deposits loss of pigmented neurones in the substantial nigra

Question 178

what are the clinical features of Parkinson's disease?

Answer 178

``` tremor rigidity brady/akinesia postural instability trembling of head and extremities forward tilt of trunk reduced arm swinging shuffling gait short steps no weakness; extrapyramidal disorder ```

Question 179

describe the tremor of Parkinson's and how it is examined

Answer 179

``` pill rolling reduces on action increases when distracted/stressed re-emergent initially unilateral jaw, eyelid, chin, foot; suggestive of PD head tremor; uncommon in PD ``` hands in lap outstretched in front of face finger-to-nose

Question 180

describe the rigidity of Parkinson's and how it is examined

Answer 180

``` stiffness through a range of movement; EF, EE like a lead pipe clasp knife usually asymmetrical + tremor = cogwheel rigidity ``` at wrist, elbow and other joints

Question 181

describe the akinesia of Parkinson's and how it is examined

Answer 181

decrement in amplitude of repetitive movement mask-like facies reduced spontaneous blinking lack of arm swing finger taps playing piano heel taps

Question 182

describe the postural instability of Parkinson's and how it is examined

Answer 182

sitting; motionless, slumped to one side gait; stopped/bent, flexed at hips, shuffling steps positive pull test falls 'en bloc' able to stand with arms crossed gait pull test

Question 183

define atypical parkinsonism

Answer 183

Parkinsonism + additional features which distinguish them from Parkinson's disease; MSA PSP CBS

Question 184

what are the clinical features of multiple system atrophy (MSA)?

Answer 184

``` parkinsonism ataxia/unsteadiness; cerebellar degeneration autonomic failure; postural hypotension, impotence, urinary frequency, urgency, incontinence stridor sleep breathing problems jerky postural tremor high pitched quivering dysarthria polyminimyoclonus disproportionate anterocollis UMN signs deep involuntary sighs or gasps ```

Question 185

what is the treatment of multiple system atrophy (MSA)?

Answer 185

``` symptomatic management only physio OT SALT BP treatment ```

Question 186

what are the clinical features of progressive supranuclear palsy (PSP)?

Answer 186

``` Parkinsonism; symmetrical atremulous early falls; classically backwards axial rigidity; severe neck stiffness inability to move eyes vertically; staring pseudobulbar palsy dementia; frontal lobe ```

Question 187

what are the clinical features and treatment of progressive supra nuclear palsy (PSP)?

Answer 187

loss of balance falls slurred speech difficulty swallowing amantadine; may improve symptoms

Question 188

what are the clinical features corticobasal degeneration (CBD)?

Answer 188

``` parkinsonism alien limb phenomenon apraxia myoclonus dementia ```

Question 189

what are the causes, clinical features and treatment of drug-induced Parkinsonism?

Answer 189

dopamine antagonists; haloperidol, maxolon, metoclopramide, stemetil, prochlorperazine parkinsonisan syndrome slowness rigidity tremor; less often stop offending drug poor response to L-dopa

Question 190

describe an essential tremor

Answer 190

``` rhythmic oscillation of body part common often inherited; AD slowly progressive fairly symmetrical flexion-extension voice worse with anxiety, stress, beta agonists improves with alcohol, beta blockers shaky handwriting ```

Question 191

describe dystonia

Answer 191

involuntary sustained contractions of opposing muscle groups twisting movements abnormal postures

Question 192

what is the clinical presentation of dystonia?

Answer 192

abnormal posture pain cramps may be associated with tremor

Question 193

describe adductor and abductor laryngeal dystonia

Answer 193

adductor; vocal cords slam together and stiffen, speech sounds strangled, words cut off abductor; cords fold open and do not vibrate, weak and breathy voice

Question 194

what is the diagnosis and treatment of laryngeal dystonia?

Answer 194

laryngoscopy | botulinum toxin injections

Question 195

describe generalised dystonia

Answer 195

``` primary dystonia onset <25yrs typically lower limb onset 50% have DYT1 mutation; AD craniocervical onset; DYT6 ```

Question 196

describe chorea

Answer 196

rapid jerky movement that flit from one part of the body to another often generalised may be confined to one body part

Question 197

what are the causes and treatment of chorea?

Answer 197

huntington's disease rheumatic fever; sydenham's chorea drugs; OCP, L-dopa pregnancy dopamine receptor blockers; tetrabenazine

Question 198

describe the pathology and clinical features of huntingtons disease

Answer 198

loss of nerve cells in caudate nucleus and putamen reduction in neurotransmitter GABA ``` inherited progressive chorea behavioural disturbance dementia jerky uncontrollable movements unsteady gait difficulty with speech, chewing, swallowing anxiety depression insomnia ```

Question 199

what is the treatment and prognosis of Huntington's disease?

Answer 199

none to cure/slow the disease tetrabenzine; reduce chorea steady progression of chorea and dementia death; 10-20yrs after onset

Question 200

describe the clinical features, causes and treatment of hemiballismus

Answer 200

severe proximal chorea; violent flinging movement of one side of the body causes; infarction, haemorrhage in the contralateral sub thalamic nucleus treatment; may settle spontaneously, dopamine blockers, surgery

Question 201

what are the primary causes of tics?

Answer 201

simple transient tics of childhood | gilles de la Tourette's syndrome

Question 202

what are the secondary causes of tics?

Answer 202

neurodegenerative disease; HD, wilsons learning disabilities; Down's syndrome, rett's syndrome, autism infection; Sydenham's, chorea, PANDAS

Question 203

describe myoclonus

Answer 203

brief involuntary twitch of a muscle or group of muscles may be a normal physiological symptoms; hypnic jerks, hiccups feature in Parkinson's disease, epilepsy, CJD contraction; positive relaxation; negative

Question 204

what are the causes of drug-induced dyskinesia?

Answer 204

levodopa in parkinsons patients neuroleptic/anti-psychotic medication tremor; salbutamol, lithium, valproate

Question 205

what are the clinical features of drug-induced dyskinesia?

Answer 205

tardive; mouthing, smacking of lips, grimaces with contortion of face and neck, after several years of neuroleptics acute dystonic reactions; spasmodic torticollis, trismus, oculogyric crises akathisia

Question 206

how is a parkinsonian syndrome diagnosed?

Answer 206

bradykinesia + at least one; muscular rigidity 4-6 Hz rest tremor postural instability

Question 207

define bradykinesia

Answer 207

slowness of initiation of voluntary movement with progressive reduction in speed and amplitude of repetitive movements

Question 208

describe the gait of Parkinson's disease

Answer 208

``` reduced arm swing reduced stride length and foot lift clock face or en-bloc turning narrow base gait freezing postural instability; pull test ```

Question 209

what are the non-degenerative causes of Parkinsonism-like symptoms?

Answer 209

``` repeated strokes or head injury Hx of encephalitis metoclopramide prochlorperazine lithium calcium channel blockers valproate ```

Question 210

what are the red flags for degenerative atypical parkinsonian disorders (MSA, PSP, CBD)?

Answer 210

``` poor/unsustained levodopa response early recurrent falls early speech/swallowing difficulty early cognitive decline cerebellar, pyramidal signs, dyspraxia or supra nuclear gas palsy early autonomic dysfunction ```

Question 211

which neurological diseases have a normal DAT (dopamine transporter) scan?

Answer 211

``` essential tremor drug induced Parkinsonism dystonic tremor vascular Parkinsonism dopa-responsive dystona psychogenic alzheimer's FXTAS ```

Question 212

which neurological disease have an abnormal DAT scan?

Answer 212

``` parkinson's disease MSA PSP dementia with LBs CBD wilson's disease spinocerebellar ataxia ```

Question 213

what are the supportive criteria for Parkinson's disease?

Answer 213

>3 for clinically definite PD ``` unilateral onset persistent asymmetry rest tremor progressive excellent levodopa response levodopa induced dyskinesia >5yr levodopa response visual hallucinations clinical course >10yrs ```

Question 214

describe the presentation of focal onset seizures

Answer 214

with preserved awareness or with impaired awareness

Question 215

describe the effects of a focal onset seizure

Answer 215

``` focal tonic-clonic limb movements adverse/contraversive movements grimacing autonomic auditory visual somatosensory ```

Question 216

what is the treatment of generalised tonic-clonic seizures

Answer 216

1st line; lamotrigine, levetiracetam, sodium valproate | 2nd line; lacosamide, zonisamide, brivaracetam, topiramate, phenytoin, carbamazepine

Question 217

what is the treatment of focal seizures?

Answer 217

1st line; lamotrigine, levetiracetam, carbamazepine, sodium valproate 2nd line; zonisamdide, gabapentin, oxcarbazepine, topiramate, phenytoin

Question 218

what is the treatment of absence seizures?

Answer 218

1st line; ethosuximide, sodium valproate | 2nd line; lamotrigine, clonazepam

Question 219

what are the side effects of levetiracetam?

Answer 219

irritability/behaviour change somnolence headache

Question 220

what are the sides effects of lamotrigine?

Answer 220

rash stevens-johnson syndrome toxic epidermal necrolysis (especially in children) insomnia

Question 221

what are the side effects of carbamazepine?

Answer 221

agranulocytosis SIADH OP stevens-johnson syndrome

Question 222

what are the side effects of sodium valproate?

Answer 222

``` hepatotoxicity highly teratogenic weight gain pancreatitis thrombocytopenia ```

Question 223

what are the side effects of phenytoin?

Answer 223

``` teratogenicity hirsutism weight gain peripheral neuropathy gum hypertrophy ```

Question 224

what are the side effects of topiramate?

Answer 224

``` cognitive problems speech disturbance behavioural abnormalities kidney stones weight loss ```

Question 225

describe the clinical features of epilepsy

Answer 225

``` tonic/clonic motor activity synchronous limb movements eyes usually open pupillary reflexes may be absent usually <90s possible injury tongue bitten high, deep and lateral 1-30mins to reorientation incontinence common ```

Question 226

describe the clinical features of syncope

Answer 226

``` often no movement multifocal synchronous limb movements eyes usually open intact pupillary response usually <60s seconds-minutes to reorientation ```

Question 227

describe the clinical features of NEAD

Answer 227

``` rhythmical tremulous movement, thrashing/violent pelvic thrusting eyes usually closed resistance to eye opening intact pupillary response can last several hours injury possible seconds-hours to reorientation occasional incontinence side to side head movements common ```

Question 228

define the different types of status epilepticus

Answer 228

impending status epilepticus/early status; <5 mins status epilepticus; >5 mins refractory status epilepticus; >30 mins super refractory status epilepticus; >24hrs

Question 229

what investigations should be performed to diagnose status epilepticus?

Answer 229

``` ABG bloods CTB drug levels ECG EEG ```

Question 230

what are the causes of status epilepticus?

Answer 230

stroke (haemorrhagic) low AED levels alcohol withdrawal drug intoxication; theophylline, imipenem, isoniazid, beta lactams, clozapine, bupropion drug withdrawal; benzodiazepine, barbiturate, baclofen anoxic brain injury metabolic disturbances infection; meningitis, encephalitis, brain abscess, sepsis traumatic brain injury brain neoplasm febrile seizures remote brain injury.congenital malformations idiopathic

Question 231

what are the symptoms and signs of early status epilepticus?

Answer 231

compensation phase/sympathetic overdrive; generalised convulsions increased CO, BP, BG, blood lactate increased cerebral blood flow, glucose and oxygen, brain tissue oxygenation, brain glucose

Question 232

what are the symptoms and signs of refractory/malignant status epilepticus?

Answer 232

``` decompression/homeostatic failure; reduced CO, BG, blood lactate, blood oxygen cardiorespiratory collapse electrolyte imbalances rhabdomyolysis and delayed tubular necrosis hyperthermia multiple organ failure increased brain damage ```

Question 233

what is the management of a post-epileptic event?

Answer 233

EEG monitoring essential if post-octal for a prolonged period maintenance medication; consider higher dose speak with neuro and ICU

Question 234

describe epilepsy in pregnancy

Answer 234

increased risk of obstetric complications; C-section, premature labour, bleeding AEDs associated with neural tube defects, cleft palate. cardiac abnormalities, hypospadias (especially valproate, phenytoin, carbamazepine) AED concentrations fluctuate during pregnancy; try to balance optimal seizure control with minimal foetal AED exposure folate supplements recommended

Question 235

what investigations are required to diagnose a LMN lesion?

Answer 235

``` CK; in all patients with neuromuscular weakness neurophysiology muscle biopsy MRI spine; exclude other causes muscle MRI genetic testing ```

Question 236

what are the muscle biopsy features in inclusion body myositis?

Answer 236

endomysial inflammatory infiltrates partial invasion of fibres rimmed vascuoles amyloid deposits on electron microscopy

Question 237

what functions are the extrapyramidal tracts responsible for?

Answer 237

posture | complex coordination of movement

Question 238

define UMN lesions

Answer 238

damage of upper motor neurone in the higher centre or above the level of the alpha motor neurone; motor cortex internal capsule brain stem spinal cord contralateral weakness; above the decussation in the brainstem

Question 239

describe hemiplegia (UMN)

Answer 239

``` contralateral weakness or paralysis spasticity of the skeletal muscle due to increased supra spinal facilitation exaggerated tendon jerk and clonus positive babinskis sign no atrophy ```

Question 240

define LMN lesions

Answer 240

damage of the lower motor neurons or any component of the reflex arc; spinal cord motor nerve axon neuromuscular junction muscle sensory nerve ipsilateral weakness, localised to muscles of the affected nerves

Question 241

what are the structural effects of LMN lesions?

Answer 241

nerve injury retrograde degeneration muscle atrophy regeneration

Question 242

what are the functional effects of LMN lesions?

Answer 242

``` all reflexes lost very focal weakness muscle wasting fasciculations; appears a few days/weeks after denervation weakness reduced tone ```

Question 243

define fasciculations

Answer 243

synchronous visible contraction of the motor unit supplied by the injured axon result from spontaneous generation of action potential in distal segment of the injured axon

Question 244

name examples of a generalised LMN process

Answer 244

generalised neuropathy; diabetic, GBS myopathy; dermatomyositis, DMD NMJ disorder; MG

Question 245

what is the aetiology of parkinsons disease?

Answer 245

sporadic/idiopathic genetic; juvenile PD, young-onset PD, early onset <50yrs drug induced; MPTP post-infectious; encephalitis lethargica chronic traumatic encephalopathy; boxing, rugby, football

Question 246

what are the features of Lewy body dementia?

Answer 246

fluctuating cognition visual hallucinations Parkinsonism

Question 247

what is the difference between LBD and PD?

Answer 247

LBD; Lewy bodies in cerebral cortex | PD; in substantis nigra

Question 248

describe MSA

Answer 248

MSA-P; Parkinsonism type MSA-C; cerebellar type alpha-synuclein progresses more rapidly than PD life expectancy 6-9 years from symptom onset

Question 249

describe PSP

Answer 249

accumulation of Tau protein | death usually within 7 years

Question 250

describe CBS

Answer 250

less common than MSA or PSP pathology; Tau onset usually around 63yrs death within 8 years

Question 251

describe the classification of dystonia

Answer 251

area affected; focal or generalised age of onset; juvenile or late secondary to other degenerative disease

Question 252

describe motor tics

Answer 252

simple; blinking, pouting, facial grimacing | complex; kicking, jumping, hopping

Question 253

describe vocal tics

Answer 253

simple; grunting, sniffing, coughing | complex; echolalia, coprolalia

Question 254

what is the difference between spasticity and rigidity?

Answer 254

spasticity; velocity dependent, clasp knife, ground in UMN/pyramidal disease rigidity; stiffness throughout the movement, lead pipe

Question 255

define akathisia

Answer 255

unpleasant sensation of restlessness | manifests as inability to sit still or remain motionless

Question 256

describe the causes, treatment and complications of bells palsy?

Answer 256

causes; HSV, VZV infection of the facial nerve treatment; acyclovir, steroids complications; persistent weakness, contracture, synkinesis

Question 257

describe synkinesis

Answer 257

voluntary muscle movement causes the simultaneous involuntary contraction of other muscles crocodile tears

Question 258

describe the course of the hypoglossal nerve

Answer 258

arises from the hypoglossal nucleus in the medulla oblongata passes laterally over the posterior cranial fossa exits the cranium via the hypoglossal canal passes inferiorly to the angle of the mandible moves anteriorly to enter the tongue

Question 259

what are the causes of hypoglossal nerve palsy?

Answer 259

head and neck malignancy penetrating trauma injuries internal carotid artery dissection

Question 260

what are the clinical features of trigeminal neuralgia?

Answer 260

sudden, short lasting, shooting/shock pain unilateral worse when eating and drinking can't bear to touch it treatment; carbamazepine

Question 261

what are the locations and features of cranial nerve damage?

Answer 261

CN nucleus within the brainstem nerve axons form nucleus still within the brainstem outside the brainstem weakness ataxia sensory loss

Question 262

what are the symptoms of anterograde amnesia?

Answer 262

``` forgetting recent personal/family events losing things in the house repeating questions, conversations problems following TV or noels reliance on lists ```

Question 263

what are the symptoms of retrograde amnesia?

Answer 263

past events; jobs, homes, illnesses, major news items | getting lost in familiar surrounds

Question 264

what are the functions of the temporal lobes?

Answer 264

memory; episodic semantic

Question 265

what are the functions of the perisylvian areas?

Answer 265

langauge

Question 266

what are the functions of the frontal lobes?

Answer 266

behavioural change | executive function

Question 267

what are the functions and pathologies of the parietal lobes?

Answer 267

praxis visuoconstructive tactile agnosia gerstmann's syndrome

Question 268

what are the functions and pathologies of the occipital lobes?

Answer 268

visual agnosia | ballint's syndrome

Question 269

what are the functions of the orbitofrontal lobes?

Answer 269

``` personality and behaviour; disinhibition eating behaviours lack of empathy personal neglect poor judgement ``` motivation; apathy passivity

Question 270

describe gerstmann's syndrome

Answer 270

``` damage to the left angular gyrus acalculia agraphia left/right disorientation finger agnosia ```

Question 271

what is the definition and causes of apraxia?

Answer 271

inability to perform learned skilled movements despite normal motor, sensory function, coordination, comprehension and attention dominant parietal, inferior frontal lesions or anterior corpus callosum

Question 272

what is the definition and causes of unilateral neglect?

Answer 272

failure to report, respond or orient to stimuli presented contralateral to a brain lesion not due to elementary sensory or motor disorder right parietal lesions also thalamus, basal ganglia, cingulate usually affects LHS

Question 273

define visual agnosia

Answer 273

failure of recognition despite normal visual percepts

Question 274

describe associative visual agnosia

Answer 274

preserved ability to perceive detail and copy accurately but unable to recognise what they have drawn left; words right; faces bilateral; objects

Question 275

describe Alzheimer's disease

Answer 275

>30yrs onset, usually >60yrs on average 8 years duration medial temporal lobes; amnesia temporo-parietal lobes; visuospatial deficits, language treatment; cholinesterase inhibitors (donepezil)

Question 276

describe frontotemporal dementia

Answer 276

``` usually 45-65yrs onset 2-20yrs duration frontal lobes; behavioural onset perisylvian areas; progressive aphasia anterior temporal lobes; semantic memory ```

Question 277

describe semantic dementia

Answer 277

a subtype of frontotemporal dementia language problems impaired word comprehension naming errors

Question 278

describe Lewy body dementia

Answer 278

``` usually >40yrs onset, mostly >65yrs 1-12yrs duration medial temporal lobes and temporoparietal; similar to AD, more visuospatial difficulty substantia nigra; Parkinsonism visual hallucinations, fluctuations neuroleptic sensitivity ```

Question 279

describe vascular dementia

Answer 279

``` usually >40yrs onset, mostly >65yrs variable, up to 12 yrs duration features determined by infarct location stepwise decline pseudo bulbar palsy pyramidal signs extensor plantars ```