Neurology 2 Flashcards

Question

What will the CSF electrophoresis be like in a patient with MS?

Answer 1

IgG oligoclonal bands are present in the CSF of MS patients.

Answer 2

``` SLE Sjogren's syndrome Lyme disease Syphilis AIDS ```

Answer 3

Treating relapses of MS symptoms (with steroid medicine) Treating specific MS symptoms Treatment to reduce the number of relapses (disease-modifying therapies)

Answer 4

Mild to moderate: oral medications Focal disabling: peripheral nerve blocks Severe: reversible and irreversible invasive procedures General rehabilitation: removal of trigger factors, physical treatments

Answer 5

``` Generally well-tolerated but side effects are: Injection site reactions Flu-like symptoms Mild intermittent lymphopenia Mild to moderate rises in liver enzymes ```

Answer 6

Oral medications - beta blockers, low-dose barbituates, Gabapentin, Izonizid, Carbamazepine, Clonazepam Orthotic devices - weighted wrist bands, computer-controlled mechanical damping devices Thalamic surgery - stereotactic thalamotomy, thalamic electrostimulation

Answer 7

General sexual counselling Erectile dysfunction - papaverine or prostaglandin E1, penile prosthesis, oral yohimbine Ejaculatory dysfunction - no specific treatment For women, lubricating gels, topical local anasthetics, relief of other symptoms

Answer 8

5-10% clinically milder with no disability 30% develop significant disability within 20-25 years Survival rate is linked to disability Death is usually due to secondary complications (respiratory or renal) Marburg variant can lead to coma or death within days

Answer 9

``` Male gender Late age of onset High number of attacks Short inter-attack interval Early residual disability Early motor, cerebellar and sphincter symptoms ```

Answer 10

Immunomodulatory therapy for acute repulses, frequent relapses, aggressive illness, progressive illness Management of symptoms Non-pharmacological treatments - physio, occupational health

Answer 11

Oral or intravenous Methylprednisolone can hasten recovery of acute exacerbation Plasma apheresis can be used if steroids are contraindicated or ineffective

Answer 12

Prevention of immuno-activation Modifying lymphocyte trafficking Lymphocytes depletion Immune reconstruction

Answer 13

``` Parasomnia e.g. narcolepsy TIA Dystonia Cardiogenic syncope Postural syncope Hypoglycaemia Migraine Benign paroxysmal positional vertigo Cataplexy Hyperventilation Non-epileptic seizure ```

Answer 14

Primary disturbance of brain function - Could be epileptic seizures (including genetic generalised epilepsy, unclassifiable epilepsy, structural or metabolic epilepsy) or dissociative/non-epileptic seizures. Secondary disturbance of brain function - related to the heart or low blood pressure.

Answer 15

Paroxysmal event in which changes of behaviour, sensation or cognitive processes are caused by excessive, hypersynchronous neuronal discharges in the brain. Too much voltage - groups of brain cells are depolarising at the same time (added together). Spike in the EEG.

Answer 16

Duration: 30-120 seconds ‘Positive’ ictal symptoms (excessive of something - hearing/seeing/feeling/being touched when it’s not actually happening) Postictal symptoms - weakness afterwards Stereotypical seizures/syndromal seizure types May occur from sleep - weakened by the state sleep (most vulnerable) May be associated with brain dysfunction Typical seizure phenomena - lateral tongue bite, deja vu (commonly the same each time for an individual patient).

Answer 17

Eye deviation to the right Head deviation to the right Right hand shaking Gradual spread to both sides TONIC: stiff, mouth open, eyes open, muscles are activated at the same time CLONIC: individual spikes of activation of movement, big fast movements, relaxation and then more big fast movements. Amplitude grows but frequency decreases. Staring into space, not really breathing properly.

Answer 18

both halves of the brain are doing the same thing (symmetrical). If it’s on both sides of the brain then both sides of the body are affected.

Answer 19

``` Different clinical features depending on which part of the brain is affected.. TEMPORAL Lip smacking Swallowing Slow movement of one hand Unconscious ``` FRONTAL Awareness fully retained No control of movements

Answer 20

Paroxysmal event in which changes in behaviour, sensation and cognitive processes are caused by an insufficient blood or oxygen supplied to the brain.

Answer 21

Situational Typically from sitting or standing Rarely from sleep Presyncopal symptoms - seeing stars over the whole visual field, vision may go black, distorted sound, dizzy and light-headed Duration 3-30 seconds Recovery within 30 seconds Cardiogenic syncope: less warning, history of heart disease

Answer 22

You faint because your body overreacts to certain triggers, such as the sight of blood or extreme emotional distress. It may also be called neurocardiogenic syncope. The vasovagal syncope trigger causes your heart rate and blood pressure to drop suddenly.

Answer 23

Paroxysmal event in which changes in behaviour, sensation and cognitive function caused by mental processes associated with psychosocial distress. People may not be able to recall what the distress was. May happen instead of a panic attack.

Answer 24

Situational Duration 1-20 minutes Dramatic motor phenomena or prolonged atonia Eyes closed Ictal crying and speaking Surprisingly rapid or slow postictal recovery History of psychiatric illness, other somatoform disorder e.g. IBS, fibromyalgia

Answer 25

Syncope - look for upright position, presyncopal symptoms, sweating, looking pale, nausea, rapid recovery Epilepsy - look for tongue biting, head turning, muscle pain, prolonged loss of consciousness, cyanosis, postictal confusion

Answer 26

Incomplete history, lack of witness account Misinterpretation of syncopal, myoclonic jerks Misinterpretation of EEG-changes

Answer 27

Associated with focal brain abnormality, may start at any age. Seizure types: - Partial seizures without impairment of consciousness e.g. Jacksonian seizures, deja vu - Partial seizures with impairment of consciousness e.g. psychomotor seizures - Secondary generalised seizures

Answer 28

Common cause: hippocampal sclerosis (mesial temporal seizure) caused by encephalitis or a prolonged febrile seizure. Starts in one place and gradually spreads.

Answer 29

First-line treatment: Carbamazepine or Lamotrigine

Answer 30

No associated brain abnormality, manifestation usually <30 years Seizure types: - Absence seizures e.g. childhood absence epilepsy, juvenile absence epilepsy - Myoclonic seizures e.g. juvenile myoclonic epilepsy - Primary generalised tonic clonic seizures e.g. grand mal on awakening

Answer 31

First-line treatment: Valproate or Lamotrigine

Answer 32

PRE-SYNAPTIC EXCITABILITY - Voltage-gated Na channel: Na increases excitability and drives action potentials. Inhibited by carbamazepine, lamotrigine, oxcarbazepine. - Voltage-gated K channel: K efflux reduces neuronal excitability, channel increased by retigabine. NEUROTRANSMITTER RELEASE - Voltage-gated Ca channels: Ca influx drives neurotransmitter release. Channel inhibited by pregabalin and gabapentin. - SV2A required for release of neurotransmitter from these vesicles. Inhibited by levetiracetem.

Answer 33

Target the GABA A receptor - reduces neuronal excitability. Increased activity by benzodiazepines, barbiturates, felbamate, topiramate. Target GABA transaminase - degrades GABA, inhibited by vigabtrin (to elevate GABA levels) Target the GABA transporter - removes GABA from the synapse, inhibited by tiagabine

Answer 34

Monotherapy: increase to lowest possible effective dose Monotherapy: increase to fully effective/maximum tolerated dose Consider alternative monotherapy/combination therapy Consider epileptic surgery (electrical stimulation therapies) Consider reduction to monotherapy in very refractory epilepsy

Answer 35

Curative intent: Resective surgery Hemispherectomy Palliative intent: Tractotomy Electrostimulation

Answer 36

Stimulates 30 seconds every 5 minutes When the heart rate goes up - given stimulation Can reduce seizure frequency

Answer 37

Cerebral hemisphere - unilateral dysfunction Spinal cord - bilateral dysfunction in the legs Cerebellum - loss of coordination Brain stem - any that doesn’t fit the pattern Peripheral nerves - mononeuropathies/polyneuropathies that don’t fit in dermatomes/myotomes

Answer 38

Where is the lesion? Single or multiple? What is the likely pathology? E.g. inflammation, infection, neoplasm, vascular, neurodegenerative Guided by the history and examination

Answer 39

Lesion at the optic chiasm you get bitemporal hemianopia - loss of temporal vision. Lesion between optic chiasm and Meyer’s loop you get homonymous hemianopia - loss of one side in both eyes Lesion at Meyer’s loop upper fibres you get inferior quadrantanopia - loss of bottom corner Lesion at Meyer’s loop lower fibres you get superior quadrantanopia - loss of top corner Lesion in the occipital pole (primary visual cortex) you get sparing of the macular

Answer 40

Nystagmus is a vision condition in which the eyes make repetitive, uncontrolled movements. These movements often result in reduced vision and depth perception and can affect balance and coordination. These involuntary eye movements can occur from side to side, up and down, or in a circular pattern.

Answer 41

Internuclear ophthalmoplegia (INO) is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction. When an attempt is made to gaze contralaterally (relative to the affected eye), the affected eye adducts minimally, if at all. The contralateral eye abducts, however with nystagmus.

Answer 42

Asymmetrical nystagmus may suggest internuclear ophthalmoplegia (may suggest MS)

Answer 43

Surgical 3rd nerve refers to the space-occupying lesion that is pressing on the 3rd nerve.The parasympathetic fibres are on the outside so they will be pushed on - dilated pupil. Can’t have a space-occupying lesion without pupillary involvement.

Answer 44

Microvascular lesions tend to be found in elderly patients, almost always diabetic. Have 3, 4, 6th nerve palsies but are well. Treat the diabetes (occlusion of the microvasculature). Most common cause of 3rd nerve palsy. Can also have this with pupillary involvement.

Answer 45

Venous sinus thrombosis, metastases, carotid rupture, inflammatory syndromes can occur in the cavernous sinus. Common area for pathology.

Answer 46

A rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, and the other can move only in outward direction. Characterized by "a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other". Nystagmus is also present when the eye on the opposite side of the lesion is abducted.

Answer 47

Eye movements not affected include convergence, vertical CNIII movements, unilateral partial adduction.

Answer 48

Horner’s syndrome - lesion in the sympathetic supply Characterized by miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face).

Answer 49

Single lesion ``` Ipsilateral side: Horner’s syndrome Limb ataxia Loss of facial sensation of pain and temperature on face, reduced corneal reflex Dysarthria Dysphagia ``` Contralateral side: Loss of pain and temperature sensation

Answer 50

Consider vertebral artery or carotid artery dissection. Carotid dissection is more common.

Answer 51

At the level of the lesion: Ipsilateral Spinothalamic (localising sign) Below the lesion: Ipsilateral corticospinal tract dysfunction Ipsilateral dorsal column dysfunction Contralateral spinothalamic tract dysfunction

Answer 52

TYPE OF INJURY Non-missile (most common) Missile head injury - penetration of skull or brain DISTRIBUTION OF LESION Focal (one particular area) Diffuse brain lesions (across the brain) TIME COURSE OF DAMAGE Primary – due to immediate biophysical forces of trauma Secondary – presenting some time after the traumatic event (physiologial responses, effects of hypoxia/ischaema, infection)

Answer 53

Scalp - contusions (damage to tissue), lacerations (tear in the tissue) Skull - Fracture Meninges - Haemorrhage, Infection Brain - Contusions, Lacerations, Haemorrhage, Infection

Answer 54

Diffuse axonal injury Diffuse vasucular injury Hypoxia-ischaemia Swelling

Answer 55

Implies considerable force ↑ risk haematoma, infection & aerocele Angled or pointed objects cause localized fractures that are often open or depressed Flat surfaces cause linear fractures Can extend to skull base (sometimes called contrecoup fractures)

Answer 56

``` ~ 10% severe head injuries, ~15% fatal ones Usually associated with skull Occur slowly over hours Usually a lucid interval Causes death by - brain displacement - raised intracranial pressure - herniation ```

Answer 57

Usually due to tears in bridging veins that cross subdural space from superior surface of brain to midsagittal sinus Dehydrated, elderly or demented patients are more vulnerable. Can occur slowly (‘chronic’) Usually surrounded by ‘membrane’ of granulation tissue

Answer 58

Contusions/lacerations Base of skull fracture (tear vessels) Vertebral artery rupture/dissection Intraventricular haemorrhage

Answer 59

Superficial: due to severe contusion Deep: related to diffuse axonal injury

Answer 60

At or just deep to the point of impact

Answer 61

Force to head causes differential movement of skull & brain. This can cause: - Impact of inner surface of skull on underlying brain causes contusion - Traction on bridging veins causes subdural haemorrhage Differential movement of brain tissue which causes shearing, traction and compressive stresses and damages blood vessels and axons

Answer 62

Superficial “bruises” of the brain “Coup” at the site of impact “Contre coup” – away from the site of impact At first – haemorrhagic Then become brown/orange and soft (days weeks) Then indented or cavitated after months years

Answer 63

When contusion is sufficiently severe to tear the pia mater

Answer 64

Axonal injury – a non specific term, can happen in a multitude of pathologies Traumatic axonal injury – can be focal or widespread Diffuse axonal injury A clinicopathological syndrome of widespread axonal damage (inc brainstem) But can be caused by a variety of processes Diffuse traumatic axonal injury – over the brain due to trauma

Answer 65

Corpus callosum Midbrain Pons

Answer 66

Usually involves acceleration and deceleration of the head Mild: recovery of consciousness ± long term, variable severity deficit Severe: unconscious from impact & remain so or severe disability

Answer 67

Enlargement of the ventricles Thinning of the corpus callosum Curvy, linear lesions in the superior frontal lobe (gliding contusion) Lose the myelination (pink colour)

Answer 68

Usually result in near immediate death | Multiple petechial haemorrhages throughout brain

Answer 69

``` Occurs in ~75% patients Leads to ↑ intra cranial pressure Caused by: - Congestive brain swelling Vasodilation and ↑ cerebral blood volume - Vasogenic oedema Extravasation of oedema fluid from damaged blood vessels - Cytotoxic oedema Increased water content of neurons and glia ```

Answer 70

Due to bleeding or swelling

Answer 71

Subfalcine herniation of the cingulate gyrus Transtentorial herniation of medial temporal lobe Transforaminal herniation of cerebellar tonsil

Answer 72

HI often causes infarction and hypoxic ischaemic damage Likely in patients who have had - Clinically evident hypoxia - Hypotension with systolic BP<80mmHg for ≥15 min - ↑ intracranial pressure It can be widespread or confined to vulnerable regions - Susceptible neurones - Border zones between major cerebral territories

Answer 73

Cortical laminar necrosis - pyramidal neurones which have long axons so high metabolic demand Hippocampus cell populations Purkinje cells in the cerebellum

Answer 74

Seen following repetitive mild traumatic brain injury Initially irritability, impulsivity, aggression, depression, memory loss Then dementia, gait and speech problems, parkinsonism Some have motor neurone disease-like symptoms

Answer 75

Atrophy: neocortex, hippocampus, diencephalon & mamillary bodies Enlarged ventricles with fenustrated cavuum septum Tau-positive neurofibrillary and astrocytic tangles in frontal and temporal cortex and limbic regions, especially around depths of sulci and limbic regions and blood vessels

Answer 76

``` Motor neurone disease (aka ALS) Heretidary spastic paraplegia Spinal muscular atrophy Kennedy’s disease Post-polio syndrome Motor neuropathies Hyperactivity disorders of the motor neurons e.g. stiff person syndrome ```

Answer 77

AKA paresis | Impaired ability to move a body part in response to will

Answer 78

The ability to move a body part in response to will is completely lost

Answer 79

AKA incoordination | Willed movements are clumsy, ill-directioned or uncontrolled.

Answer 80

Spontaneous movement of a body part, independently of will.

Answer 81

Disorder of consciously organised patterns of movement or impaired ability to recall acquired motor skills.

Answer 82

Upper motor neurons which have cell bodies sitting in the precentral gyrus and axons which travel down the brainstem and into the spinal cord via the corticospinal tract. The lower motor neurons come from the motor nuclei in the brainstem and down the ventral horn of the spinal grey matter.

Answer 83

Idea of a movement - association areas of cortex Activation of the upper motor neurons in the precentral gyrus Impulses travel to the lower motor neurons and their motor units via the corticospinal (pyramidal) tracts Modulating activity from the cerebellum and the basal ganglia Further modification of movement depending on sensory feedback

Answer 84

The pathway by which the CNS controls voluntary movement Lower motor neurons have their cell bodies in the nuclei in the brainstem and spinal cord. Their axons join with other axons to form nerve roots and peripheral nerves. It makes contact with muscle fibres at the neuromuscular junction.

Answer 85

Basic functional unit of muscle activity. Lower motor neuron, axon and several supplied muscle fibres.

Answer 86

Stretch receptors in muscle (muscle spindles) are innervated by Y motor neurons. When the muscle is stretched, afferent impulses from muscle spindles are sent to the spinal cord which cause a reflex partial contraction of the muscle. Disease states e.g. spasticity and extrapyramidal rigidity alter muscle tone by altering the sensitivity of this reflex.

Answer 87

``` Motor nuclei of cranial nerves Motor neurons in the spinal cord Spinal ventral roots Peripheral nerves Neuromuscular junction Muscle ```

Answer 88

Muscle tone is normal or reduced (flaccid) Muscle wasting e.g. in tongue, upper/lower limb, respiratory muscles Fasciculation - visible spontaneous contraction of motor units Reflexes depressed or absent

Answer 89

``` Motor neuron disease Spinal muscular atrophy Poliomyelitis Syringomyelia/syringobulbia Spinal cord/brainstem compression Vascular disease ```

Answer 90

Prolapsed intervertebral disc Cervical or lumbar spondylosis Tumours e.g. neurofibroma/ependymoma Malignant infiltration

Answer 91

Axonal degeneration or axonal demyelination Symmetrical polyneuropathy - distal weakness in limbs, glove and stocking patterns in sensory loss, decreased/absent reflexes Mononeuritis multiplex pattern of weakness and sensory loss conforms to the distribution of particular peripheral nerves

Answer 92

Myasthenia gravis Lambert Eaton myasthenic syndrome Congenital disorders

Answer 93

Many causes Weakness tends to affect proximal muscles Tendon reflexes preserved until late

Answer 94

Know the anatomy of the innervation of muscles and reflexes Are there any coexisting sensory signs as well as motor signs Is there any history of diurnal variability/fatigability? Fasculications? Suggest motor neuron problem Pattern of weakness - proximal limb and axial in muscle disease, distal in peripheral neuropathy

Answer 95

Neurophysiology nerve conduction studies/electromyography Neuro-imaging MRI scan head/spine Blood tests e.g. muscle enzymes, peripheral neuropathy screen, auto-antibodies Lumbar puncture

Answer 96

Reflex arc Corticospinal or pyramidal pathway (upper motor neuron system) Extrapyramidal or basal ganglia system Cerebellum

Answer 97

A major descending pathway connecting the upper and lower motor neurons, important in the control of voluntary movements. Made up of a number of anatomical stations including the motor cortex, the corona radiata, internal capsule, cerebral peduncle in the midbrain, pyramidal bundle in the pons, pyramid in the medulla. In the medullary pyramid 85% of the fibres cross to form the lateral corticospinal tract in the contralateral spinal cord. 15% of fibres remain uncrossed and form the anterior corticospinal tract.

Answer 98

Muscle tone is increased (spasticity) Tendon reflexes/jaw jerk are brisk Plantar responses extensor (and Babinski sign) Characteristic pattern of limb muscle weakness (pyramidal pattern) - upper limbs extensor muscles are weaker than flexors, lower limbs flexors weaker than extensors, finer more skillful movements are most severely impaired Emotional lability may be present

Answer 99

A pathological sign whereby a patient is unable to maintain their arms in a stretched out raised supine position. Presentation of this suggest spasticity due to an upper motor neuron lesion.

Answer 100

Contralateral, often circumscribed weakness | Dispersion of cortical representation over a wide area

Answer 101

Complete contralateral hemiparesis | Descending fibres grouped closely together in a very small area

Answer 102

Often bilateral weakness due to an involvement of both descending corticospinal tracts Involvement of the cranial nerve nuclei Bulbar involvement often present

Answer 103

Often bilateral corticospinal tract lesions Cervical - will involve upper and lower motor neurons (tetraparesis) Below cervical - weakness of both legs (paraparesis) Sensory level may help in localising the site of the spinal lesions

Answer 104

Vascular disease e.g. Cerebrovascular accident Inflammatory e.g. MS Compression of brain or spinal cord e.g. by a tumour or a degenerative spinal disease (spondylosis) Infiltration of the corticospinal pathway e.g. tumour Neurodegenerative disease of upper motor neurons +/- lower motor neurons in motor neuron disease, hereditary spastic paraplegia

Answer 105

Over 150 different types Divided based on cell or location Meninges - Meningioma Sellar region - Craniopharyngiomas, usually benign, cystic tumours Germ cell tumours - rare paediatric, usually cancerous tumours in the pituitary/pineal region Intrinsic brain - Gliomas: oligodendroglial cells, astrocytic cells, ependymal cells, neuronal tumours Cranial nerves (schwannoma) e.g. eighth nerve - acoustic neuroma Haematopoietic, lymph nodes (lymphoma) Metastatic spread from lung, breast, colorectal, testicular, renal cell, malignant melanoma

Answer 106

Increasing - could be genuinely an increase or due to better diagnosis Not as common as other cancers like breast cancer but more common in young adults 55% malignant Common differential diagnosis - frequent concern is that a headache will be a sign of a brain tumour

Answer 107

Don’t used TNM system Use the WHO system - based on the cell that the tumour comes from Grade using morphology into four grades of malignancy Now also using molecular genetic features

Answer 108

Most common primary brain tumour Tumour of glial cells - astrocytes, oligodendrocytes, ependymal cells WHO grade I and II ‘low’ WHO grade III and IV ‘high’ Grade II aren’t ‘benign’ because they are prone to transforming Grades determine the prognosis Characterised histologically - cellularity/mitotic activity/vascular proliferation/necrosis

Answer 109

Slow growing but will undergo anaplastic transformation Astrocytomas 3.5 years Oligodendrogliomas 7-10 years Median age 35 years Survival time longer than de novo high grade gliomas

Answer 110

``` Histology type Age Size of tumour Rate of growth Location Cross midline Presenting features Performance status ```

Answer 111

Most common type of brain tumour (85% of new malignant brain tumours) Either as a primary tumour or from a pre-existing low-grade Median onset is 45 for III, 60 for IV

Answer 112

``` Majority - no cause found Ionising radiation 5% family history: associated genetic syndromes, neurofibromatosis, tuberous sclerosis, Von Hippel-Lindau disease Immunosuppression (CNS lymphoma) No evidence to link to mobile phone use ```

Answer 113

Varied presentation dependent on tumour type, grade and site | Symptoms can include headaches, seizures, focal neurological symptoms, other non-focal symptoms.

Answer 114

Patient is woken by the headache, worse in the morning, worse lying down, associated with nausea and vomiting, exacerbated by coughing, sneezing, drowsiness.

Answer 115

21% presenting symptom 80% associated with low-grade rather than high-grade First fit 2-6% = brain tumour

Answer 116

Limb jerking, head or eye deviation - frontal lobe Sensory disturbance, spreading, tingling - parietal lobe Deja vu, jamais vu, memories, feelings of dread, rising feeling - temporal lobe Positive visual disturbance (coloured balls) - occipital lobe

Answer 117

Focal - weakness, sensory loss, visual/speech disturbance, ataxia Non-focal - personality change, memory disturbance, confusion

Answer 118

Papilloedema | Focal neurological deficit - hemiparesis, hemisensory loss, visual field defect, dysphagia

Answer 119

Headache with features of raised intracranial pressure (pailloedema) or focal neurology New onset focal seizure Rapidly progressing focal neurology (without headache) Past history of other cancer

Answer 120

Low-grade: typically present with seizures (can be incidental finding) High-grade: rapidly progressive neurological deficit. Symptoms of intracranial pressure.

Answer 121

Brain imaging - CT (with contrast) or MRI MRI is best If high-grade, you would see an irregular mass with vasogenic oedema. Enhancement often seen (disruption of BBB).

Answer 122

``` Cerebral blood volume MR Spectroscopy (chemical composition) Rate of Growth Enhancement Brain biopsy - histology and molecular markers ```

Answer 123

Depends on tumour type, grade and site | Treatment is non-curative (unless it’s grade I)

Answer 124

Steroids - reduce oedema Surgery - biopsy or reduction for tissue diagnosis, reduction of ICP, prolongation of survival Radiotherapy - mainstay (radical vs. palliative) Chemotherapy - Temozolamide, PCV Prognosis: 6 months without treatment, 18 months with treatment

Answer 125

Surgery - early resection or biopsy Radiotherapy alone - delays disease transformation not overall survival Radiotherapy and chemotherapy - evidence suggests long-term survival

Answer 126

If the tumor or the area of your brain where your seizures occur (epileptic focus) is near the parts of the brain that control vision, movement or speech, the patient can be woken up during surgery to be asked questions to monitor responses. The procedure also lowers the risk of damage to functional areas of your brain that could affect your vision, movement or speech..

Answer 127

Sits in the pituitary fossa Almost always benign and usually curable. Excessive hormone production Local effects of the tumour - bilateral hemianopia due to compression of optic chiasm Inadequate hormone production by the remaining gland

Answer 128

A decline in cognitive function that is gradual onset and progressive. A set of symptoms including memory loss, problem-solving or language. Alzheimer's disease is the most common.

Answer 129

Post-mortem: cerebral cortex was thinner, senile plaques, neurofibrillary tangles. You can stage dementia into I-II, III-IV, V-VI. Begins in the amygdala and spreads into the medial temporal lobe, the cingulate gyrus before becoming more widespread.

Answer 130

Up to 45% normal elderly people will meet the criteria for dementia had they been demented. Known as the ‘high-pathology non-demented controls’ or individuals with asymptomatic AD’.

Answer 131

Presentation of an early and significant episodic memory impairment. Can be isolated or associated with gradual and progressive change in memory function reported by the patient for over 6 months. Objective evidence of an amnestic syndrome of the hippocampal type (based on failure in episodic memory test) - problems with storing memories.

Answer 132

Decreased Aβ1-42 together with increased T-tau or P-tau in CSF Increased tracer retention on amyloid PET AD autosomal dominant mutation present in PSEN1, PSEN2, APP

Answer 133

``` HISTORY Sudden onset Early occurrence of the following symptoms: gait disturbances, seizures, major and prevalent behavioural changes. CLINICAL FEATURES Focal neurological features Early extrapyramidal signs Early hallucinations Cognitive fluctuations ```

Answer 134

Major depression Cerebrovascular disease Toxic, inflammatory and metabolic disorders MRI FLAIR or T2 changes in the medial temporal lobe that are consistent with infectious or vascular insult.

Answer 135

Criteria for asymptomatic risk for AD: Absence of specific clinical phenotype Evidence of AD pathology - decreased Aβ1-42 together with increased T-tau or P-tau in CSF OR increased tracer retention on amyloid PET

Answer 136

Criteria for presymptomatic AD Absence of specific clinical phenotype Proven AD autosomal dominant mutation present in PSEN1, PSEN2, APP or other genes including trisomy 21 (Down’s syndrome)

Answer 137

Depression - onset and decline is often rapid (trigger/life event), subjective complaints of memory loss (obvious early on), patients distressed/unhappy, variability in cognitive performance, ‘don’t know’ answers. Dementia - vague, insidious onset, unaware or attempting to hide problems (symptoms may go unnoticed), confusion in the evening, mood might be labile, cognitive performance is consistent, attempts all questions.

Answer 138

Subtle behaviour changes: inattentiveness, mild cognitive dulling, social withdrawal, emotional withdrawal and agitation Apathy (most frequent change), disengagement Psychotic symptoms: delusions (usually of theft) or hallucinations Agitation or anxiety

Answer 139

Hearing (superior temporal lobe) Language comprehension (superior temporal lobe) Semantic knowledge (anterior temporal lobe) Memory (hippocampus) Emotional/affective behaviour (limbic system)

Answer 140

Alzheimer’s disease is thought of as a posterior form of dementia. Typical amnesic variant. Early degeneration of medial temporal lobe before degeneration spreads to temporal neocortex, frontal and parietal association areas.

Answer 141

``` Selective amnesia, semantic and language impairments Complex attention (divided, selective, attention switching) Global deficits ```

Answer 142

Episodic memory: frequent intrusions and repetition errors and high numbers of false positive errors in recall Complex attention/executive function Disproportionate impairment of category fluency as compared to phonemic fluency

Answer 143

Visual variants Posterior occipitoparietal, occipitotemporal or more rarely primary visual cortex Visual deficits, dyspraxia, dysgraphia, simultanagnosia Linguistic variant: Lateral temporal regions Progressive aphasic syndrome

Answer 144

- Vascular/mixed dementia: sub-cortical-frontal pattern (attention difficulties, motor/cognitive slowing and executive problems) and visuo-spatial difficulties - Dementia with lewy bodies: fluctuating cognition, pronounced disturbances in attention and concentration, working memory and early visuoperceptual deficits, visual hallucinations and spontaneous Parkinsonism - Depressive pseudodementia

Answer 145

Studies suggest that patients with early onset AD are less likely to have memory-related symptoms and more likely to have non-memory symptoms like apraxia/visuospatial dysfunction.

Answer 146

``` Apraxia/visuospatial dysfunction Language dysfunction Aphasic-apraxic-agnostic syndrome Posterior cortical atrophy Dysexecutive syndrome ```

Answer 147

Memory impairment is typically the first marker of AD Poor performance on episodic memory tests General cognitive function is preserved, activities of daily living are intact and there is no evidence of dementia Score above 24/30 on MMSE (Mini-Mental State Examination) High risk for developing AD

Answer 148

Take a history from patient/family Assess cognitive function (6CIT) - what year is it? What month is it? Give a 5 part address, count 20-1, say months of the year in reverse, repeat address Blood tests (vitamin deficiencies, thyroid dysfunction) Structural scan (can be CT but preferably MRI) used to exclude other cause e.g. stroke, tumour. PET scan can be used for amyloid imaging. FDG PET looks at glucose metabolism.

Answer 149

But a large number of healthy elderly people have amyloid in their brain because the deposition increases with age. Not very good at discriminating.

Answer 150

Prevention: Healthy diet, healthy body weight, not smoking, reduced drinking and increased physical exercise can reduce your risk of dementia. Support - socially and cognitively active, treat mood and anxiety Carers - safeguarding Advice Medications - Acetylcholine esterase inhibitors - Memantime (anti-glutamate) - moderate to severe AD

Answer 151

A collective term used to denote the complex of nervous and mental disorders of soldiers in modern wartime societies E.g. spasticity, tics, tremors etc Treatment was psychosocial e.g. hypnosis, relaxation techniques, re-education, occupation

Answer 152

Symptoms are broadly classified as organic or functional. Organic - structural problem that is causing the symptoms that can be identified through physical examination or investigation Functional - disorder of function (structurally intact) E.g. IBS, chronic fatigue syndrome, fibromyalgia

Answer 153

A psychological defense mechanism in which negative feelings are transferred from the original source of the emotion to a less threatening person or object.

Answer 154

The manifestation of psychological distress by the presentation of physical symptoms.

Answer 155

Dissociation is a way the mind copes with too much stress. People who dissociate may feel disconnected from themselves and the world around them. Periods of dissociation can last for a relatively short time (hours or days) or for much longer (weeks or months). It can sometimes last for years, but usually if a person has other dissociative disorders.

Answer 156

``` Appearance Behaviour Speech Mood Thoughts Perception Cognition Insight ```

Answer 157

Neuroacanthocytosis is a general term for a group of rare progressive disorders characterized by the association of misshapen, spiny red blood cells (acanthocytosis) and neurological abnormalities, especially movement disorders. The 'core' neuroacanthocytosis syndromes, in which acanthocytes are a typical feature, are chorea acanthocytosis and McLeod syndrome.

Answer 158

Change in personality | Apathy, irritability, disinhibition, inability to plan/organise/execute behaviour.

Answer 159

ECT - pass electricity through the brain Intention is to bring about a tonic-clonic seizure therapeutic for depression Can work within a week Can affect episodic memory

Answer 160

Memory impairment in Alzheimer’s - acetylcholine depletion Depression in Parkinson’s - serotonin, noradrenaline, dopamine depletion Psychosis in Parkinson’s treatment - excess dopamine

Answer 161

Age e.g. Huntinton’s is a disease of the middle age Sex Educational attainment e.g. higher education is protective for dementia progression Socio-economic status Premorbid personality traits e.g. Psychiatric history

Answer 162

Personality traits - repeated patterns of thinking, feeling and behaving. Some traits are helpful but some are unhelpful. Helpful traits should outweigh the unhelpful, allowing us to cope with the challenges of daily life.

Answer 163

Cerebellum contains a complete motor and sensory representation of the whole body. Dysfunction causes ataxia (lack of order). Made up of an outer molecular layer, a single layer of large purkinje cells and then an inner granular layer. The structure is the same throughout - same population of cells repeating itself.

Answer 164

``` Slurring of speech -often the volume of the speech is inappropriate to compensate for the slurring. Dysphagia Oscillopsia/blurring vision Clumsiness due to loss of precision of movement Tremor Unsteadiness when walking Falls Cognitive ```

Answer 165

Dysarthria Nystagmus - fast movement to one direction and a slow movement to the other Limb ataxia Action (intention) tremor Truncal ataxia - very rare, concerns about cancer if sudden onset Gait ataxia

Answer 166

A form of involuntary eye movement that is part of the vestibulo-ocular reflex (VOR), characterized by alternating smooth pursuit in one direction and saccadic movement in the other direction.

Answer 167

Tested by asking the patient to touch their nose and then touch your finger. Should be able to do that with precision. Ask the patient to do a repetitive movement to test for Dysdiadochokinesia (impaired ability to perform rapid, alternating movements). Heel-to-shin test in a straight line.

Answer 168

Congenital ataxia Diseases where ataxia is only one of many features and may not be the most prominent one e.g. leukodystrophies Familial ataxias, presumed genetic split into dominant and recessive Sporadic (acquired) ataxias Ataxias due to structural damage to the cerebellum are not included in this classification.

Answer 169

Congenital vs. early onset (genetic) vs late onset (acquired) Rate of progression - slow is more likely to be genetic, fast is more likely to be due to degeneration or immune-associated ataxia Episodic - recovery/relapse Pure ataxia or ataxia plus (leg stiffness, sensory symptoms, deafness) - are there additional features? Oscillopsia Speech affected early or not? Urinary, postural, nocturnal symptoms (autonomic features)

Answer 170

Nystagmus, opsoclonus (involuntary movement in an unpredictable direction), slow saccades, ophthalmoplegia, oculomotor apraxia (eye not moving properly so need to turn head to see target) Palatal tremor, deafness Gait more affected than limb ataxia Truncal ataxia Pure or ataxia plus (autonomic, neuropathy, spasticity) Other clues (telangiectasia, xanthomata)

Answer 171

``` Posterior circulation stroke affecting the cerebellum Primary tumours (hemangioblastoma, acoustic neuromas, medulloplastoma) Secondary tumours (metastasis) Hydrocephalus, Chiari malformation (a condition in which brain tissue extends into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward.) Multiple sclerosis (primary progressive) ```

Answer 172

The MRI appearance of the pons in a variety of neurodegenerative diseases. T2 hyperintensity forms a cross on axial images through the pons, representing selective degeneration of pontocerebellar tracts. It has been described in multiple system atrophy (MSA)

Answer 173

Familial/genetic (⅓) - most common is Friedreich's ataxia Gluten ataxia Idiopathic ataxia Alcohol MSA-C (multiple system atrophy in the cerebellum)

Answer 174

Accounts for a quarter of all sporadic ataxias Heterogenous group Likely some will be genetic Less likely to be degenerative

Answer 175

47% had other associated immune diseases like thyroid problems, type 1 diabetes etc HLA DQ2 present which is associated with autoimmune diseases Anticerebellar antibodies more likely to be detected.

Answer 176

Presence of anti-GAD - antibodies to glutamic acid decarboxylase Often associated with additional autoimmune disease (Insulin-dependent diabetes mellitus , hypothyroid, PA) Variable progression with periods of stability Better to treat early with immunosuppressants Monitored with MR spectroscopy

Answer 177

Sequencing Language Executive function Visuospatial abilities

Answer 178

A condition that follows from lesions (damage) to the cerebellum of the brain.These cognitive impairments result in an overall lowering of intellectual function.

Answer 179

A clinical syndrome caused by a cerebral infarction or haemorrhage, typified by rapidly developing signs of focal and global disturbance of cerebral functions lasting more than 24 hours or leading to death.

Answer 180

An acute loss of cerebral or ocular function with symptoms lasting less than 24 hours caused by an inadequate cerebral or ocular blood supply as a result of low blood flow, ischaemia or embolism associated with disease of the blood vessels, heart or blood.

Answer 181

Very common Causes morbidity and mortality Stroke/TIA are urgent/emergency Primary and secondary prevention are essential parts of care (medicines/lifestyle changes)

Answer 182

85% of strokes Blood vessel in the brain is blocked Usually atherosclerotic plaque or a clot in a larger artery ruptures, travels downstream, gets trapped in a narrower artery in the brain. Embolic strokes are common complications of atrial fibrillation and atherosclerosis of carotid arteries.

Answer 183

15% of strokes Bleeding from a blood vessel within the brain High blood pressure is the main cause of intracerebral hemorrhagic stroke.

Answer 184

Suspect a stroke in anyone presenting with acute onset, ongoing focal neurological deficit that cannot be explained by hypoglycaemia or other stroke mimics.

Answer 185

Usually unilateral Facial weakness (MCA) Unilateral weakness of the upper and/or lower limb (MCA/ACA) Unilateral sensory loss of the upper and/or lower limb (MCA/ACA) Speech problems (MCA, dominant hemisphere - most commonly left) Visual defects (PCA) Disorders of perception (PCA) Disorders of balance (posterior circulation) Coordination disorders (posterior circulation)

Answer 186

Hypoglycaemia Labyrinthine disorders (labyrinthitis/vestibular neuronitis, Benign paroxysmal positional vertigo (BPPV), Meniere’s disease) Migraine with aura - Typical (visual symptoms, sensory symptoms, dysphasia) - Typical (motor weakness, ‘hemiplegic migraine’, other) Mass lesions (subdural haematoma, cerebral abscess, tumours) Postictal weakness (aka Todd’s paralysis) Simple partial seizures Functional hemiparesis

Answer 187

Arrange emergency admission to a specialist stroke unit | 999 (if by telephone if clinically appropriate) or 1 hour admission

Answer 188

Assess risk of stroke in the next 7 days | High risks require urgent referral (within 24 hours)

Answer 189

Assess risk used ABCD score: A - Age: 60 years or more (1 point) B - BP (at presentation): 140/90 mmHg or greater (1 point) C - Clinical features: unilateral weakness (2 points), speech disturbance without weakness (1 point) D - Duration: 60mins or longer (2 points), 10-58mins (1 point) D - Diabetes (1 point)

Answer 190

ABCD score of 4 or more Atrial fibrillation More than one TIA in one week TIA whilst on anticoagulant

Answer 191

None of the above | Present more than a week after their last symptoms have resolved

Answer 192

First point of contact for healthcare For new problems (including urgent and emergency) and for ongoing problems Places: GP practices, walk-in centres, minor injuries unit, dentists, opticians, 999? ED? Wide range of practitioners So primary care is not the same as general practice

Answer 193

Healthcare provided by GPs GPs are doctors and members of the RCGP Training: med school, foundation training, GP specialist training Summarised as expert medical generalism Independent contractors to the NHS (the GP contract), owned and run by GP partners Own buildings and employ staff Reception, admin, nurses, healthcare assistants, physician associates, salaried GPs.

Answer 194

Provision of healthcare to all patients with any healthcare need. It’s focused on the person and not the disease ‘whole person medicines. Includes: physical and mental health, end of life care, scheduled/routine care and emergencies, easy access, long term relationship, continuity, coordination, gatekeeping, evidence-based, patient education, public health, symptoms, medicines management.

Answer 195

Depth of knowledge in lots of high-prevalence diseases e.g. asthma, COPD, diabetes, hypertension, headache. Don't have deep knowledge of all conditions, especially rare conditions. Requires decision-making without subject specific expertise. Emphasis on pragmatism, adaptability, resilience. Clinical skills and clinical reasoning and the ability to manage uncertainty. Specialist input is often required. Working in teams is an essential part of general practice - practice-teams, community teams, hospital teams.

Answer 196

Brain tumours are fairly uncommon but have a high morbidity and mortality. 20% of childhood cases (majority occur in the posterior fossa) In adults, majority supratentorial

Answer 197

``` Astrocytic (most common) Oligodendroglial Ependymal Neuronal and neuro-glial Pineal Embryonal – primitive looking phenotype Choroid plexus ```

Answer 198

Cranial and spinal nerve tumours Meningeal tumours Lymphomas – no lymphoid tissue but these can occur in older people and immunosuppressed (EBV) Germ cell tumours – like the ones that can occur in the gonads, seen in young people Metastatic tumours

Answer 199

Loss of function – depends on location Seizures/epilepsy Raised ICP

Answer 200

``` Diffuse Astrocytomas (share a lot of pathological and clinical features) diffuse astrocytoma WHO grade 2 anaplastic astrocytoma, grade 3 glioblastoma, grade 4 ``` ``` Other Types (biologically distinct) eg Pilocytic ```

Answer 201

Infiltrate diffusely into brain – not very well circumscribed This means that these tumours are not curable by surgical resection. Propensity to undergo progressive anaplasia (aggressive progression)

Answer 202

Become progressively more molecular abnormal Cell of origin Astrocytoma (Hypercellularity pleomorphism) Anaplastic astrocytoma (Mitoses) Glioblastoma (Necrosis and microvascular proliferation) Note: Patients can present with a glioblastoma straight away (usually older patients).

Answer 203

Most common 4/5th decades May have seizures WHO grade II Anaplastic Variant Calcification recognised on scan Co-deletion 1p19q – can be demonstrated by FISH – prognostic and predictive marker. Mutations in IDH1 (isocitrate dehydrogenase) are present in low grade (II/III) astrocytomas and oligodendrogliomas. Antibody for IDH1 can be used to identify whether the tumour has the mutation.

Answer 204

IDH1 mutant 1p19q del, ATRX preserved - Oligodendroglioma IDH1 mutant 1p19q non-del, ATRX lost - Astrocytoma, secondary Glioblastoma IDH1 wildtype - Glioblastoma de novo

Answer 205

``` Hematoxylin and eosin stain Immunohistochemistry Molecular/cytogenetics e.g. 1p 19q IDH1 mutation MGMT methylation status – predicts responsiveness to alkylating agents ```

Answer 206

``` More prevalent in children Present in the posterior fossa – cerebellum (common site) Also at other sites - Optic nerves - Hypothalamic/midline Often cystic Good prognosis ```

Answer 207

Embryonal tumour Densely cellular Primitive “small blue cell” tumour of the cerebellum Found in childhood Highly malignant but may respond to excision/radiotherapy/chemo (potentially curable)

Answer 208

Morphology - Desmoplastic lower risk in infants - Large cell anaplastic higher risk Molecular subtyping - Wnt, Shh, Non-Wnt/Shh groups

Answer 209

Dural based Push into brain Most are grade I but there are more aggressive variants (grade II and III)

Answer 210

``` May present with brain metastasis before the primary tumour presents. Lung (45%) Breast (25%) Melanoma (12%) GI Tract Kidney ```

Answer 211

Brain is in a hard box – no space to expand Initially there is a compensatory phase – compression of the ventricles Only last so long Pressure begins to rise Exponential rise in pressure –bigger and bigger change in pressure Starts to push on adjacent structures Pressure gradients – things start to move across dividers (internal herniation)

Answer 212

Oculomotor nerve is responsible for the innervation of the extraocular muscles of the eye except the superior oblique and the lateral rectus (when compressed – down and out phenotype). Also responsible for the parasympathetic innervation of the pupil – lose parasympathetic tone (unbalance sympathetic tone) – ipsilateral pupil dilatation.

Answer 213

Bleeding into the brain stem (into the midbrain and the pons) due to the tear of vessels coming from the basilar artery. Classically in the midline. Prevent any brainstem input into the cerebrum. Causes death.

Answer 214

Tonsils pushed out through the foramen magnum. Presses on the medulla (cardio-respiratory centres). Causes death. Lesion sometimes referred to as coning.

Answer 215

Local deformity and shift of structures Decreased volume of CSF although there may be expansion of the ventricles in some cases. Pressure gradients lead to internal herniation Lateral tentorial - lethal Cerebellar tonsillar - lethal Central transtentorial Subfalcine cingulate

Answer 216

Cells death: PD and Huntington’s | Dysfunctional brain circuits: Essential tremor, dystonia, tourette’s

Answer 217

Consider how quickly a symptom develops. Time patterns are very important in considering neurological conditions. In PD it is usually gradual onset. Consider how the symptoms affect the areas of the body. In PD there is usually one side which is worse than the other.

Answer 218

Brady/Akinesia: problems with doing up buttons, keyboard, writing smaller, walking deteriorated, small stepped, dragging one foot etc. Tremor: at rest, may be unilateral Rigidity: pain, problems with turning in bed

Answer 219

Small stepped gait with stooped posture, reduced arm swing L>R Increased tone = rigidity, tone increased over entire radius of joint movement Rest tremor: often asymmetrical, also some postural tremor Decreasing amplitude/accuracy of repetitive movements - much better than the beginning, gradual worsening

Answer 220

A tool used to confirm the diagnosis of Parkinson's disease. It is a specific type of single-photon emission computed tomography (SPECT) imaging technique that helps visualize dopamine transporter levels in the brain. In Parkinson's, there is a steady loss of dopamine transporters in the brain.

Answer 221

Unknown Can be familial, but more likely to be spontaneous. Can have genetic susceptibility factors. Environmental factors - toxin-induced risk factors Oxidative stress and mitochondrial dysfunction can also have an impact.

Answer 222

``` The disease is slowly progressive It does not change its nature overnight No cure No disease-modifying treatment Plenty of symptomatic treatment options Aim is to compensate for loss of dopamine ```

Answer 223

L-Dopa is the precursor of dopamine Best drug for PD Can support the action of L-Dopa by combining it with other drugs, like dopamine agonists. Act at the post-synaptic receptors. COMT/MAO-B inhibitors can also be useful: Catechol-O-Methyl-Transferase Monoamino-oxidase Reduce the breakdown of dopamine, increasing its levels. Anticholinergics are really not ideal because they have side effects on cognition, confusion, systemic.

Answer 224

Wearing off: the medication doesn’t work as it did before On-dyskinesias: hyperkinetic, choreiform movements whenever drugs work Off-dyskinesias: fixed, painful dystonic posturing, typically of feet, when drugs don’t work Freezing: unpredictable loss of mobility

Answer 225

Nobody knows Major push from drug companies to get patients started on treatment early Factors to take into consideration: severity of motor impairment/QoL, side effects, myths, L-dopa phobia, neuroprotection As always look at each individual patient and try to decide together

Answer 226

``` MAO-B inhibitor (Rasagiline - Selegiline): not very powerful but does help some patients, neuroprotective effect DA agonist (Ropininirole, pramipexole, Rotigotine - patches): reduced risk of dyskinesias in the short-term, first line treatment of younger PD patients, significant ‘soft tissue’ effects: tiredness, gambling, hypersexuality etc L-Dopa: most powerful drug, the higher the dose, the greater the risk of side effects ```

Answer 227

Dispersible L-Dopa: kick start in the morning Standard release: day time medication Slow release: night time medication

Answer 228

Depression is very common in PD (20%) Other psychiatric problems are also very common: phobias, anxiety, psychosis Dementia is also very common in PD (20%) Can also have autonomic problems: constipation, increased urinary frequency, urinary incontinence is untypical.

Answer 229

``` Incontinence Dementia Symmetry Early falls None of these should really be present in PD (at least not at the beginning). ```

Answer 230

Intermittent pressure increase Increased ventricles Can be treated by surgery - a shunt can result in the improvement of dementia, incontinence, walking problems

Answer 231

Very common No structural pathology Treatment Beta-blockers - up to 100mg daily, contraindicated in asthmatics or diabetics Primidone - start off with low doses Others - gabapentin and clonazapam

Answer 232

Everything outside the brain and the spinal cord | Includes the receptors at the skin, peripheral nerves, spinal nerves, dorsal and ventral roots.

Answer 233

A⍺ fibres: proprioception - where our limbs stand in space, large in diameter, myelinated Aꞵ fibres: light touch, pressure, vibration - large in diameter, myelinated Aδ fibres: pain and cold sensation - small in diameter, myelinated C fibres: pain and warm sensation - small in diameter, unmyelinated

Answer 234

Mononeuropathy: problem with one nerve Polyneuropathy: problem with many nerves Demyelination - loss of myelin, reduction in conduction velocity Axonal damage - lesion in the axon itself

Answer 235

``` Carpal tunnel syndrome (median nerve) Ulnar neuropathy (entrapment at the cubital tunnel) Peroneal neuropathy (entrapment at the fibular head) Cranial mononeuropathies (III or VII cranial nerve palsy) - can be idiopathic, immune mediated, ischemic ```

Answer 236

Small fibre neuropathies Large fibre neuropathies (more common) Can be divided into: - Acute (less than 6 months): axonal or demyelinating - Chronic (more than 6 months): axonal or demyelinating

Answer 237

A⍺ fibres: ataxia, no balance, unable to tell where our limbs are Aꞵ fibres: unable to perceive light touch, pressure, vibration Aδ fibres: unable to perceive pain and cold sensation C fibres: unable to perceive pain and warm sensation

Answer 238

Poor balance TWO TYPES: sensory (loss of proprioception) or cerebellar dysfunction When the cause is sensory, the ataxia gets worse when it is dark or the eyes are closed.

Answer 239

Muscle cramps Weakness Fasciculations (muscle twitches) Atrophy - can lead to high arched feet (pes cavus) when atrophies are excessive

Answer 240

``` Symmetrical sensorimotor (most common) - length-dependent because the more distal nerves are affected first, initially sensory but eventually sensorimotor Asymmetrical sensory - less common, patchy distribution of sensory symptoms, dorsal root ganglia are affected, think of associated diseases: cancer, Sjogren’s, gluten sensitivity/coaelic disease Asymmetrical sensorimotor (least common) - multiple mononeuropathies, may not very affected all at the same time, variable, think of vasculitis ```

Answer 241

``` History Clinical examination Reduced or absent tendon reflexes Sensory deficit Weakness - muscle atropies Neurophysiological examination (i.e.. Nerve conduction studies/QST) to identify the severity and the type (axonal or demyelinating) ```

Answer 242

Associated with systemic diseases Inflammatory - immune mediated (mainly acute) Infectious (i.e. hepatitis, HIV, lyme) Ischaemic (i.e. vasculitis) Metabolic (i.e. Fabry’s, porphyria) Hereditary (CMT, HLPP) Toxins (pharmaceuticals, environmental toxins, B6)

Answer 243

``` Diabetes Vitamin deficiency (commonly B12) Gluten sensitivity/coeliac disease Chronic renal disease Excessive alcohol drinking Hypothyroidism Amyloidosis Connective tissue disease Paraproteinemia Paraneoplastic Critical illness polyneuropathy ```

Answer 244

Chronic - develops over at least 6 months Idiopathic - no aetiology can be identified despite extensive and appropriate investigations Axonal - axons are affected, most commonly in proportion to their length (length dependent)

Answer 245

Charcot Marie tooth disease | Hereditary sensory and autonomic neuropathy

Answer 246

3 types: demyelinating/axonal motor/axonal sensorimotor Can relapse Clinically: Rapidly ascending paralysis and sensory deficits (begins as tingling, weakness and then complete paralysis) Infection (i.e. GI or respiratory) might precede the disease - campylobacter Needs immediate treatment (IVIG - plasma exchange)/ITU to support respiratory function

Answer 247

Symptomatic Pain (i.e. amitriptyline, gabapentin, pregabalin etc) Cramps (i.e. quinine) Balance (i.e. physiotherapy/walking aids) Aim is to identify any reversible causes and prevent progression if possible.

Answer 248

Anterior circulation stroke - blockage/bleed from a artery in or coming from the circle of Willis Posterior circulation stroke - blockage of the basilar/vertebral artery

Answer 249

Leg weakness Sensory disturbance in the legs (due to sensory and motor loss) Gait apraxia - truncal apraxia Incontinence (sensory supply to the bladder) Drowsiness Akinetic mutism - decrease in spontaneous speech, stuporous state (can be almost unconscious with it)

Answer 250

CT scans utilize X-rays to produce images of the inside of the body while MRI (magnetic resonance imaging) uses powerful magnetic fields and radio frequency pulses to produce detailed pictures of organs and other internal body structures. CT looks at density of tissue - when an area of the brain’s blood supply is blocked, the cells there start to lyse and they lose their cell membranes and draw more water in, causing a change in density. MRI looks at water content

Answer 251

``` Contralateral arm and leg weakness Contralateral sensory loss Hemianopia (optic tracts pass through the area which is supplied by MCA) Aphasia (speech areas supplied by MCA) Dysphasia (motor cortex damage) Facial droop ```

Answer 252

Rapid neurological deterioration due to the effects of space occupying cerebral oedema following middle cerebral artery (MCA) territory stroke. Can cause herniation.

Answer 253

Contralateral homonymous hemianopia Cortical blindness with bilateral involvement of the occipital lobe branches Visual agnosia Prosopagnosia Dyslexia, anomic aphasia, colour naming and discrimination problems Only one where there may be a presenting unilateral headache

Answer 254

Mainly affect the cerebellum and the brain stem. Motor deficits such as hemiparesis or tetraparesis and facial paresis Dysarthria and speech impairment Vertigo, nausea and vomiting Visual disturbances Altered consciousness

Answer 255

Lacunar strokes - blockages of smaller vessels (branches), either get sensory or motor changes Warning syndromes - blockages of small vessels of the cortex, stuttering and starting of areas of the body before progressing towards a stroke TIA - blocked and then re-opens or finds another way round. Precursor for a bigger ischaemic event.

Answer 256

Aspirin and rehabilitation Break up the blood clots with Actilyse or other thrombolytic (has a window that it can work in: 4-6 hours) Clot retrieval - interventional radiology to remove clots from big arteries e.g. AMP/CA, basilar artery (most effective) Interaterial thrombolysis - inject the thrombolysis directly into the clot Decompressive craniectomy - usually in MCA stroke

Answer 257

Up to 4.5 hours post onset of symptoms | Given intravenously to break down the clot

Answer 258

``` Recent surgery in the last 3 months Recent arterial puncture History of active malignancy Evidence of brain aneurysms Patient on anticoagulation Severe liver disease Acute pancreatitis Clotting disorder Severe hypertension ```

Answer 259

- Platelet treatments: aspirin and dipyridamole of Clopidogrel - Cholesterol treatments: statins - Atrial fibrillation treatments: Warfarin and NOACs (don’t need as much monitoring but don’t have a reversible drug) - Blood pressure treatments: antihypertensives

Answer 260

A commonly used scale for measuring the degree of disability or dependence in the daily activities of people who have suffered a stroke or other causes of neurological disability.

Answer 261

Neurofibromatosis is a genetic disorder that causes tumors to form on nerve tissue. These tumors can develop anywhere in your nervous system, including your brain, spinal cord and nerves. Neurofibromatosis is usually diagnosed in childhood or early adulthood.

Answer 262

A pathognomonic sign, may be visible in the cornea of the eyes, either directly or on slit lamp examination as deposits of copper in a ring around the cornea. They are due to copper deposition in Descemet's membrane. They do not occur in all people with Wilson's disease.