PBL ILO’s Flashcards

Question

Question 2 A 67 year old man comes to the emergency department with sudden onset vertigo and nausea starting 3 hours ago. He feels uncoordinated walking and has a broad based uncoordinated gait and nystagmus. Which other clinical features are consistent with the presentation (choose three)? A. Chorea = uncontrollable jerky movements B. Dysarthria = speech muscles weak C. Dysdiadochokinesia D. Intentional tremor E. Resting tremor F. Rigidity

Answer 1

B. Dysarthria = speech muscles weak C. Dysdiadochokinesia D. Intentional tremor

Answer 2

E3 V4 M5 = 12 Opens eyes to sounds 3/4 Confused speech 4/5 Localised motor response 5/6

Answer 3

E1 V2 M5 = 8

Answer 4

E1 V1 M3 = 5

Answer 5

D. No contrast CT scan brain - can see haemorrhage

Answer 6

A. Alteplase - should be given within 4.5hrs of symptoms starting

Answer 7

A. Broca’s area

Answer 8

A. Direct factor Xa inhibitor

Answer 9

D. Prednisolone 30mg - if symptoms presented in the last 72hrs (NICE)

Answer 10

- Check tongue for swelling - in case of allergic reaction to thrombolytic agent used - Another CT scan - Neuro exam - Any dizziness, vomiting

Answer 11

The most likely diagnosis is bacterial meningitis. This patient has presented with meningeal symptoms, fever and confusion, which have progressed rapidly over the last 12 hours. The CSF is cloudy on inspection, the white cell count is significantly raised, and glucose levels are low. The history and CSF results strongly suggest bacterial meningitis, and he should be treated empirically whilst culture results are awaited.

Answer 12

The most likely diagnosis is viral meningitis. This patient has presented with a history of meningitic symptoms alongside coryzal symptoms, suggesting a viral illness. The CSF findings are more suggestive of viral meningitis, given the clear appearance of the CSF, the mildly raised WCC (consisting mainly of lymphocytes), raised protein level and normal glucose. Further investigations, including CSF PCR, would be useful in identifying the specific causative virus.

Answer 13

The most likely diagnosis is subarachnoid haemorrhage (SAH). The typical history of a sudden severe headache and meningitic symptoms (neck stiffness) strongly suggest SAH. CT head is the first-line investigation, however sensitivity decreases after six hours from symptom onset. As a result, lumbar puncture is used to rule out SAH. The CSF typically shows a persistently raised red cell count (due to blood in the CSF from the initial bleed). Within several hours, the red blood cells in the cerebrospinal fluid are broken down, releasing their oxygen-carrying molecule heme, which is metabolised by enzymes to bilirubin, a yellow pigment. This yellow pigment can be detected, and its presence is called xanthochromia.

Answer 14

Neurogenic atrophy is caused by an injury or disease affecting nerves that connect to your muscles. When these nerves are damaged, they can't trigger the muscle contractions that are needed to stimulate muscle activity.

Answer 15

Some symptoms common to neuromuscular disorders include: • Muscle weakness that can lead to twitching, cramps, aches and pains • Muscle loss • Movement issues • Balance problems • Numbness, tingling or painful sensations • Droopy eyelids • Double vision • Trouble swallowing • Trouble breathing

Answer 16

Types of neuromuscular disorders include: • Amyotrophic lateral sclerosis (ALS) • Charcot-Marie-Tooth disease • Multiple sclerosis • Muscular dystrophy • Myasthenia gravis • Myopathy • Myositis, including polymyositis and dermatomyositis • Peripheral neuropathy • Spinal muscular atrophy

Answer 17

Features of upper motor neurone lesions: Site or the lesion - cerebral hemisphere, cerebellum, brain stem, spinal chord Muscle weakness - quadriplegia, hemiplegia, diplegia, paraplegia Muscle tone - spasticity, rigidity Fasciculation - absent Tendon reflexes - hyperreflexia Abdominal reflexes - absent depending on the involved spinal level Sensory loss - cortical sensations Electromyography - normal nerve conduction, decreased interference pattern and firing rate

Answer 18

Site or the lesion - anterior horn cell, nerve roots, peripheral nerves, neuromuscular junction and muscles Muscle weakness - proximal (myopathy), distal (neuropathy) Muscle tone - hypotonia Fasciculations - present (particularly tongue) Tendon reflexes - hypo / areflexia Abdominal reflexes - present Sensory loss - peripheral sensations Electromyography - abnormal nerve conductions, large motor units, fasciculations and fibrillations

Answer 19

There is a classic triad of features in Parkinson’s disease: 1. Resting tremor (a tremor that is worse at rest) 2. Rigidity (resisting passive movement) 3. Bradykinesia (slowness of movement)

Answer 20

Pathophysiology The basal ganglia are a group of structures situated near the centre of the brain. They are responsible for coordinating habitual movements such as walking, controlling voluntary movements and learning specific movement patterns. Dopamine plays an essential role in the basal ganglia function. Patients with Parkinson’s disease have a slow but progressive drop in dopamine production.

Answer 21

Spinal cord disorders are conditions that cause damage and deterioration to the spinal cord. These conditions may include: Tumours Spinal stenosis Herniated discs Abscess Hematoma Vertebral fractures Degenerative disc disease

Answer 22

Features Tremor in Parkinson's is worse on one side and has a 4-6 hertz frequency, meaning it cycles 4-6 times per second. It is described as a “pill-rolling tremor” due to the appearance of rolling a pill between their fingertips and thumb. It is more noticeable when resting and improves on voluntary movement. It gets worse when the patient is distracted. Performing a task with the other hand (e.g., miming the act of painting a fence) exaggerates the tremor. Rigidity is resistance to the passive movement of a joint. Taking a hand and passively flexing and extending the arm at the elbow demonstrates tension in the arm that gives way to movement in small increments (like little jerks). The jerking resistance to movement is described as “cogwheel” rigidity. Bradykinesia describes the movements getting slower and smaller and presents in several ways: • Handwriting gets smaller and smaller (micrographia) • Small steps when walking (“shuffling” gait) • Rapid frequency of steps to compensate for the small steps and avoid falling (“festinating” gait) • Difficulty initiating movement (e.g., going from standing still to walking) • Difficulty in turning around when standing and having to take lots of little steps to turn • Reduced facial movements and facial expressions (hypomimia) Other features include: • Depression • Sleep disturbance and insomnia • Loss of the sense of smell (anosmia) • Postural instability (increasing the risk of falls) Cognitive impairment and memory problems

Answer 23

Myopathy is a general term referring to any disease that affects the muscles that control voluntary movement in the body.

Answer 24

The visual Pathway • The optic nerve transmits sensory visual information received from the retina to the visual cortex. • The optic nerve is formed by the convergence of axons from the retinal ganglion cells. These cells receive impulses from the rods and cones. Optic Tracts • Within the middle cranial fossa the optic nerves from each eye unite and cross over to form the optic chiasm. • Within the lateral halves the optic nerves remain ipsilateral. • Action potentials received from the retina travel along these pathways and go into the brain Each optic tract travels to its corresponding cerebral hemisphere and goes to the R and L Lateral Geniculate Nucleus. This is a relay system located within the thalamus The axons running from the LGN carry visual information knows as the optic radiation

Answer 25

Optic Radiation (remember everything is upside down until it reaches the brain) The optic radiation pathway can be divided into upper and lower optic radiation • Upper optic radiation – carries fibres from the superior retinal quadrants (corresponding to the inferior visual field quadrants). It travels through the parietal lobe to reach the visual cortex. • Lower optic radiation – carries fibres from the inferior retinal quadrants (corresponding to the superior visual field quadrants). It travels through the temporal lobe, via a pathway known as Meyers’ loop, to reach the visual cortex. • Once in the visual cortex the brain processes the visual data and responds appropriately i.e. by flipping it the right way round

Answer 26

Proprioception is transmitted through one of the ascending sensory pathways called The Dorsal Column-Medial Lemniscal Pathway The DMCL carries the sensory modalities of: • Fine touch • Vibration sense • Proprioception The name • In the spinal cord, information travels via the dorsal (posterior) columns. • In the brainstem, information is transmitted through the medial lemniscus. First second and third order neurones are involved in this pathway. First order The first order neurones carry sensory information regarding touch, proprioception or vibration from the peripheral nerves to the medulla oblongata. Arm = above T6 Leg = Below T6 • Signals from the upper limb (T6 and above) – travel in the fasciculus cuneatus (the lateral part of the dorsal column). They then synapse in the nucleus cuneatus of the medulla oblongata. • Signals from the lower limb (below T6) – travel in the fasciculus gracilis (the medial part of the dorsal column). They then synapse in the nucleus gracilis of the medulla oblongata. Second Order Neurones • The second order neurones begin in the cuneate nucleus or gracilis. The fibres receive the information from the preceding neurones, and delivers it to the third order neurones in the thalamus. • Within the medulla oblongata, these fibres decussate. They then travel in the contralateral medial lemniscus to reach the thalamus. Third Order Neurones • Lastly, the third order neurones transmit the sensory signals from the thalamus to the ipsilateral primary sensory cortex of the brain.

Answer 27

Dorsal column-medial lemniscus (DCML) The DCML pathway transports information about vibration, proprioception and fine touch. Information from these modalities is transported in the dorsal column, two large white matter tracts located between the dorsal grey horns of the spinal cord. These dorsal columns are divided into two regions: • Fasciculus gracilis (more medial): information from below T6-T8; • Fasciculus cuneatus (more lateral): information from above T6-T8 but below the head.

Answer 28

The central nervous system uses ascending and descending pathways to communicate with the external environment. Ascending pathways transport sensory information in afferent pathways from the body to the brain. Descending tracts carry motor information in efferent nerves from upper motor neurons of cortical structures like the cerebellum and cerebrum. The descending tracts transmit this information to lower motor neurons, allowing it to reach muscles. Each pathway has upper motor neurons and lower motor neurons, typically neurons in descending tracts are UMNs and those leaving the spinal cord are LMNs. Ascending: • Dorsal column and the medial lemniscus (DCML) • Spinothalamic tracts • Spinocerebellar tracts Descending: • Pyramidal/ extra pyramidal tracts • Corticospinal tracts

Answer 29

The dorsal columns are assessed by: • Vibration: 128 Hz tuning fork • Joint proprioception: small-joint movement (thumb, big toe) • Light touch: cotton wool

Answer 30

Spinothalamic tract (anterolateral system) The spinothalamic tract has two components, often referred to collectively as the anterolateral system. They are the anterior and lateral spinothalamic tracts (STT): • Anterior STT: crude touch and pressure • Lateral STT: pain and temperature The nerve fibres cross at the anterior grey commissure at the level of the spinal nerve, but sometimes continue to cross in the 2-3 spinal levels superior

Answer 31

The spinothalamic tracts are assessed by: • Pain: pin-prick • Temperature: cool and warm metal object

Answer 32

Spinocerebellar tracts The spinocerebellar tracts transmit proprioceptive signals from the body to the brain. They transmit information about muscle stretch and the rate of muscle stretch from golgi tendon organs (GTO) and muscle spindle (MS) complexes. There are four of them: • Dorsal (D) spinocerebellar: MS and some GTO from lower limb • Cuneocerebellar (C): MS and some GTO from upper limb • Ventral (V) spinocerebellar: GTO only from lower limb • Rostral (R) spinocerebellar: GTO only from upper limb There are three extra points to note with these pathways: • The ventral spinocerebellar tract decussates twice, terminating in the IPSILATERAL cerebellum • The other (spinocerebellar) tracts do not decussate and terminate in the IPSILATERAL cerebellum • Each of these tracts can be group into the ventral spinocerebellar tract (ventral and rostral spinocerebellar tracts) and a dorsal spinocerebellar tract (cuneocerebellar and dorsal spinocerebellar tract) The midbrain and superior cerebellar peduncle are only involved in the ventral spinocerebellar tract; the cuneocerebellar, and dorsal and rostral spinocerebellar all enter the cerebellum at the medulla through the inferior cerebellar peduncle

Answer 33

Pyramidal tracts The pyramidal tracts are named as such due to their course through the pyramids of the medulla oblongata. The pyramidal tracts are responsible for the conscious, voluntary control of the body and face muscles. They can be divided into two tracts that supply each of these areas: • Corticospinal tract: cortex to spine (body) • Corticobulbar tract: cortex to ‘bulb’ (Latin for ‘swelling or bulb of an onion’ like the head and neck)

Answer 34

Corticospinal tract (CST) The CST communicates with three major cortical areas: • Primary motor cortex: located in the precentral gyrus; execution of movements • Premotor cortex: responsible for the control of behaviour, particularly of the trunk muscles • Supplementary motor cortex: stabilisation and coordination of the body during bimanual movements The fourth cortical area the CST communicates with is the posterior parietal cortex for integration with and modulation of incoming sensory information. Neurons exiting the cerebral cortex in one of the three major regions above converge to form the white matter structure in the brain known as the internal capsule. The internal capsule is located between the basal ganglia and thalamus; two highly vascularised structures in the deep brain. After passing through the internal capsule, the fibres continue to pass down through the centre of the crus cerebri of the midbrain, before entering the pons and medulla. As the CST passes through the caudal medulla, it divides into the lateral and anterior corticospinal tracts: • Lateral CST: decussate in the pyramid of the medulla • Anterior CST: stay ipsilateral These tracts then descend into the spinal cord, terminating in the ventral horn of the spinal cord where they synapse onto LMNs to supply the peripheral musculature. The anterior CST remains ipsilateral and descends only to the cervical and upper thoracic spinal cord, where they decussate at the level of the nerve root they supply.

Answer 35

Management of an acute episode NICE guidelines state that a relapse/attack can be diagnosed if a patient presents with: • New symptoms or worsening of existing symptoms • Subacute onset >24hrs • Absence of fever/signs of active infection Not all relapses require medical intervention. If symptoms are severe, should consider medical intervention: • Treatment of an acute attack/relapse requires high dose steroid therapy with methylprednisolone ○ 500mg Po for 5 days or 1g Po for 3-5 days Plasmapheresis is also an option if the exacerbation is refractory to steroids • Plasmapheresis → replacement of blood plasma. Take it out the vein, separate plasma from other cells, replace with plasma solution. Flare ups may be caused by something other than an attack, such as an infection → treat underlying cause

Answer 36

Long-term management Management of demyelinating process Injectable disease-modifying agents: • Beta-interferon • Glatiramer acetate Oral disease-modifying therapies (for relapsing MS): • Dimethyl fumarate • Fingolimod • Cladribine Monoclonal antibody therapies (treatment of RRMS): • Alemtuzumab • Natalizumab ** monoclonal antibodies carry dangerous side effects: • Most notably, natalizumab can rarely cause progressive multifocal leukoencephalopathy via reactivation of the JC virus

Answer 37

Beta-interferons • Recommended for treatment of 'active' relapsing MS → two or more relapses in the last two years • How do beta-interferons work? ○ Your body makes its own interferons to dampen down inflammation. These are artificial interferons that reduce and my prevent inflammation that damages nerves in MS

Answer 38

Glatiramer acetate • In England you can have this drug if: ○ You've had two relapses in the last two years ○ You've had one relapse in the last two years and MRI scans show new signs your MS is active • How does glatiramer acetate work? ○ It is not clear how glatiramer acetate works. It seems to kill the immune cells that coat the myelin around nerves in the brain and spinal cord.

Answer 39

Dimethyl fumarate • Recommended for people with 'active' relapsing MS. It won't work if you don’t get relapses so you won't be offered it if you have primary or secondary progressive MS • How does dimethyl fumarate work? ○ Unsure on exact function, but it dampens down inflammation. May be helpful in reducing the inflammation that causes damage in the brain and spinal cord of people with MS

Answer 40

Fingolimod • In England and Northern Ireland you can have it if: ○ You have the same or an increased number of relapses despite treatment with beta interferons, glatiramer acetate, dimethyl fumarate or teriflunomide • How does fingolimod work? ○ T and B cells cause a lot of damage in MS. They normally kills viruses and bacteria that get into your body but in MS they damage your nerves. ○ Fingolimod stops them leaving you lymph nodes → means fewer get to the brain and spinal cord where they would attack the myelin

Answer 41

Cladribine • You can take cladribine if: ○ You have 'highly active' relapsing MS → two or more disabling relapses in the past year and MRI scans show you have more, or bigger lesions. ○ Despite taking a DMT, you've had a relapse in the past year and new or bigger lesions can be seen on your MRI scans • How does cladribine work? ○ Kills T and B cells made by the immune system ○ Cladribine stops B cells especially from getting into the brain and spinal cord, so they cannot damage nerves there

Answer 42

Alemtuzumab • Across the UK you can take alemtuzumab if you have relapsing MS and: ○ Despite already taking a DMT, you've still had a recent relapse and MRI scans show new signs that your MS is active OR ○ You've had two or more relapses in the last year and you have new lesions on your MRI scans • How does alemtuzumab work? ○ Kills T and B cells that attack the myelin in MS ○ Stops the cells from getting into the brain and spinal cord before they can damage the nerves there

Answer 43

Natalizumab • In England you can have this drug if: ○ You have relapsing MS and you've had at least two relapses in the last year and MRI scans show new signs that your MS is active • How does natalizumab work? ○ Sticks to T cells and stops them from getting into the brain and spinal cord where they would attack the myelin around the nerves there.

Answer 44

Symptom control in advanced Multiple Sclerosis (MS) can be challenging due to the progressive nature of the disease and the wide range of symptoms that individuals may experience. Here are some common problems associated with symptom control in advanced MS: 1. Mobility Issues: As MS progresses, mobility can become severely impaired. Individuals may experience difficulty walking or may become wheelchair-bound. This can lead to a loss of independence and increased reliance on caregivers. Exercise can help to maintain activity and strength 2. Spasticity: Spasticity, or muscle stiffness and spasms, is a common symptom in advanced MS. It can be painful and limit range of motion, making daily activities more challenging. Spasticity and oscillopsia may be managed with baclofen or gabapentin 3. Fatigue: Fatigue is a pervasive symptom of MS and can become even more debilitating in advanced stages. Managing energy levels and finding ways to cope with extreme fatigue can be difficult. Fatigue may be managed with amantadine, modafinil or SSRI's 4. Pain: Pain can be a significant problem in advanced MS, and it may be neuropathic pain or related to musculoskeletal issues caused by mobility limitations. Neuropathic pain may be managed with medication (eg amitriptyline or gabapentin) 5. Bladder and Bowel Dysfunction: Advanced MS can lead to bladder and bowel control problems, including urinary urgency, incontinence, and constipation. These issues can be distressing and affect overall quality of life. Urge incontinence may be managed with antimuscarinic medications (eg solifenacin) 6. Cognitive Impairment: Cognitive changes, such as memory problems and difficulty with concentration and decision-making, can worsen in advanced MS, impacting daily functioning and independence. 7. Depression and Anxiety: Dealing with the progressive nature of MS and the challenges it presents can lead to increased rates of depression and anxiety in individuals with advanced MS. Depression may be managed with antidepressants, such as SSRI's 8. Swallowing Difficulties: Dysphagia, or difficulty swallowing, can occur in advanced MS, making it challenging to eat and increasing the risk of aspiration pneumonia. 9. Respiratory Issues: Weakness in the respiratory muscles can lead to breathing difficulties, especially during sleep. This can result in sleep apnea or a decreased ability to clear secretions from the airways. 10. Pressure Sores: Immobility and the inability to change positions easily can lead to pressure sores (bedsores), which can be painful and lead to serious infections. 11. Emotional and Social Isolation: The progressive nature of advanced MS can lead to social isolation and emotional distress due to limitations in mobility and participation in social activities. 12. Medication Management: Managing a complex medication regimen, including disease-modifying therapies, symptom management, and potential side effects, can be challenging. 13. Financial and Caregiver Burden: The cost of care for advanced MS and the strain it places on caregivers can be overwhelming for both individuals with MS and their families.

Answer 45

A lumbar puncture is usually required to obtain a sample of CSF for analysis. The most common indication for a diagnostic lumbar puncture is to investigate cases of suspected CNS infection (e.g. meningitis). CSF interpretation is also used to diagnose important non-infective pathologies, including subarachnoid haemorrhage.

Answer 46

Normal CSF To understand CSF abnormalities in certain disease states, it is important to understand normal CSF composition. Normal CSF is acellular. However, up to 5 white blood cells (WBCs) and 5 red blood cells (RBCs) per microlitre (µL) are considered normal after lumbar puncture. White blood cell analysis in CSF usually separates WBCs into lymphocytes and polymorphonuclear leukocytes (PMNs). PMNs include neutrophils, eosinophils, basophils and mast cells. In ‘normal’ CSF, WBCs should be predominantly lymphocytes. The presence of PMNs in the CSF, particularly neutrophils, suggests bacterial meningitis. The blood-brain barrier is effective against large molecules (e.g. protein) but allows the passage of smaller molecules (e.g. glucose). As such, CSF is generally a low-protein fluid with copious glucose. Normal CSF ranges (adults) Appearance: clear and colourless White blood cells (WBC): 0 – 5 cells/µL, predominantly lymphocytes. Red blood cells (RBC): 0 – 5/µL Protein: 0.15 – 0.45 g/L (or <1% of the serum protein concentration) Glucose: 2.8 – 4.2 mmol/L (or ≥ 60% serum glucose concentration) Opening pressure: 10 – 20 cm H2O

Answer 47

Bacterial meningitis Bacterial meningitis typically presents with headache, fever, neck stiffness and photophobia. A characteristic non-blanching (petechial) rash is often present in meningococcal disease. Patients are often systemically unwell and require urgent treatment with IM/IV antibiotics following local antibiotic guidelines.3 Treatment should not be delayed to obtain CSF analysis. The presence of turbid CSF with high opening pressure, elevated polymorphonuclear leukocytes and low serum glucose is highly suggestive of bacterial infection. Gram stain and CSF culture / bacterial PCR confirm the diagnosis. Gram staining The appearance of causative bacterial organisms on Gram stain:3 • Gram-positive diplococci: pneumococcal infection (Streptococcus pneumoniae) • Gram-negative diplococci: meningococcal infection (Neisseria meningitidis) • Gram-positive rods / coccobacilli: listerial infection (Listeria meningitidis)

Answer 48

Viral meningitis Viral meningitis is typically more insidious in onset than bacterial infection but causes similar symptoms (headache, fever, neck stiffness and photophobia). Patients are less likely to be systemically unwell at the time of presentation. The most common causative agents are herpes viruses (HSV / VSV) and enteroviruses.5 Treatment is with an intravenous antiviral agent, most commonly aciclovir. CSF with a markedly raised lymphocyte count and elevated protein but normal glucose is highly suggestive of viral infection. Diagnosis can be confirmed with a positive CSF viral PCR.

Answer 49

Tuberculosis meningitis Tuberculous meningitis should be suspected in those exposed to a patient with pulmonary tuberculosis, or those with known risk factors for TB (e.g. people from areas of high disease prevalence, homelessness, immunosuppression etc). CSF typically shows high protein, low glucose levels, and elevated WCC with lymphocyte predominance. In early TB meningitis, there may be PMN predominance, and thus CSF analysis can appear very similar to bacterial meningitis. To confirm the diagnosis, CSF is sent for acid-fast-bacilli smear and culture alongside TB polymerase chain reaction (PCR). Appearance: opaque, if left to settle it forms a fibrin web Opening pressure: elevated WBC: elevated (typically lymphocyte predominance) Glucose level: low Protein level: elevated (1-5 g/L)

Answer 50

Fungal meningitis Fungal meningitis is rare, most commonly seen in cases of profound immunosuppression, such as untreated HIV. CSF analysis can vary depending on the pathogen involved, and diagnosis can be confirmed by CSF fungal culture or specific antigen tests (e.g. cryptococcal antigen). Appearance: clear or cloudy Opening pressure: elevated WBC: elevated (typically more modest elevations seen) Glucose level: low Protein level: elevated

Answer 51

Subarachnoid haemorrhage (SAH) Appearance: blood-stained initially, with xanthochromia (yellowish) >12 hours later Opening pressure: elevated WBC: elevated (WBC to RBC ratio of approximately 1:1000) RBC: elevated Glucose level: normal Protein level: elevated

Answer 52

Multiple sclerosis (MS) Appearance: clear Opening pressure: normal WBC: 0 – 20 cells/µL (primarily lymphocytes) Glucose level: normal Protein level: mildly elevated (0.45 – 0.75 g/L) CSF electrophoresis: oligoclonal bands present

Answer 53

Guillain-Barré syndrome (GBS) Appearance: clear Opening pressure: normal or elevated WBC: normal Glucose level: normal Protein level: markedly elevated (>5.5 g/L) Guillain-Barré syndrome is characterised by rapidly progressive ascending weakness. CSF interpretation is usually performed to aid diagnosis and exclude alternate pathologies. CSF generally shows a normal cell count and raised protein in cases of GBS. However, this may not be seen in the first week of the illness.

Answer 54

Bacterial meningitis Appearance: Cloudy/turbid White blood cells: Elevated, >100/µL Primarily PMNs Protein: Elevated, >0.5g/L Glucose: Low <40% serum glucose Opening pressure: Elevated (>25cm H2O)

Answer 55

Appearance: Usually clear White blood cells: Elevated, >100/µL Primarily lymphocytes Protein: Elevated, >0.5g/L Glucose: Normal >60% serum glucose Opening pressure: Normal or elevated

Answer 56

Hearing loss Seizures Motor deficit Cognitive impairment Hydrocephalus Visual disturbance

Answer 57

• Tension headaches • Migraines • Cluster headaches • Sinusitis • Giant cell arteritis → (temporal arteritis). Form of vasculitis commonly causing inflamed temporal arteries • Glaucoma → acute angle closure glaucoma. Due to reduced drainage of aqueous humor in the eye - leads to raised intraocular pressure • Intracranial haemorrhage • Venous sinus thrombosis → blood clot in dural venous sinuses. May happen during pregnancy. • Subarachnoid haemorrhage • Hormonal headache • Cervical spondylosis • Trigeminal neuralgia • Raised intracranial pressure • Brain tumours • Meningitis • Encephalitis • Brain abscess • Pre-eclampsia → high blood pressure and protein in urine

Answer 58

1) Olfactory nerve (sensory) ○ Function: smell ○ Provides special visceral afferent fibres for the sense of smell ○ Connects to the brain, passes through cribriform plate of the skull

Answer 59

2) Optic nerve (sensory) ○ Function: vision ○ Provides special somatic afferent fibres for vision ○ Afferent limb for the pupillary light reflex ○ Connects to the brain, passes through optical canal of the skull

Answer 60

3) Oculomotor nerve (motor) ○ Function: eye movement ○ Provides general somatic efferent and general visceral efferent fibres to extraocular and pupillary constrictor muscles. ○ Efferent limb for pupillary light reflex ○ Connects to midbrain, passes through superior orbital fissure of skull

Answer 61

4) Trochlear nerve (motor) ○ Function: eyeball movement ○ General somatic efferent to extraocular superior oblique muscle. Assists in depressing and abducting the eye ○ Connects to midbrain ○ Only cranial nerve to leave the pontomesencephalic junction posteriorly, passes through superior orbital fissure of skull

Answer 62

5) Trigeminal nerve (sensory + motor) ○ Function: facial movement, chewing, temperature, touch, pain ○ 3 sensory nuclei: § Mesencephalic - proprioception § Principal - light touch and discrimination § Spinal - pain, temperature, crude touch ○ Afferent limb of corneal reflex, CN 7 in bilateral efferent limb ○ Emerges from the pons and has 3 divisions

Answer 63

6) Abducens (motor) ○ Function: eyeball movement ○ General somatic efferent fibres for eye abduction, innervates lateral rectus muscle ○ Originates in pontomedullary region, passes through superior orbital fissure of the skull

Answer 64

7) Facial nerve (sensory + motor) ○ Function: taste, saliva, tears, facial movement i.e. facial expressions ○ Originates in pontomedullary region, passes though internal auditory meatus and exists through stylomastoid foramen ○ Facial nerve loops around abducens nucleus ○ Lower motor neurone facial nerve lesions cause upper and lower facial paralysis ○ Upper motor neurone facial nerve lesions causes lower facial paralysis only

Answer 65

8) Vestibulocochlear nerve (sensory) ○ Function: hearing, equilibrium ○ Special somatic afferent fibres for hearing and balance ○ Originates in pontomedullary region, passes through auditory meatus, does not leave the skill ○ Cochlea transmits sound waves to mechanical ossicle movements to electrochemical action potentials ○ Vestibular apparatus detects changes in head motion

Answer 66

9) Glossopharyngeal nerve (sensory + motor) ○ Function: swallowing, monitoring blood pressure/oxygen/CO2 ○ Originates in the medulla oblongata, passes through jugular foramen ○ Draws fibres from solitary nucleus (taste) and nucleus ambiguus (motor) ○ Draws more fibres from inferior salivatory nucleus (parotid gland) and dorsal motor nucleus

Answer 67

10) Vagus nerve (sensory + motor) ○ Function: smooth muscle control, digestive enzyme secretion ○ Originates in medulla oblongata and passes though jugular foramen with CN IX and XI ○ Key role is parasympathetic innervation of the viscera ○ Recurrent laryngeal nerve loops under right subclavian artery and left aortic arch

Answer 68

11) Accessory nerve (motor) ○ Function: swallowing, head/shoulder movement ○ Originates in medulla oblongata and superior cervical cord region ○ Exists the spinal cord in the neck and enters skull though foramen magnum ○ General somatic efferent fibres to trapezius and sternocleidomastoid

Answer 69

12) Hypoglossal nerve (motor) ○ Function: tongue movement, speech, swallowing ○ General somatic efferent fibres for controlling tongue muscles ○ Originates in medulla oblongata and exists skull though hypoglossal canal

Answer 70

Pathophysiology • DIC is in response to an illness or disease which results in dysregulated blood clotting • There is both bleeding and thrombosis simultaneously 1. A trigger leads to intravascular activation of the coagulation cascade throughout the body a. Sepsis and trauma -> lead to a release of pro-inflammatory cytokines in a systemic inflammatory response b. Other conditions -> expression of certain pro-coagulant factors is upregulated 2. Microvascular thrombosis results due to formation of fibrin webs and the activation and aggregation of platelets within small vessels a. These small thrombi can lead to multi-organ failure due to tissue ischaemia 3. However, widespread activation of coagulation leads to a reduction in conc of circulating coagulation factors a. This is known as consumptive coagulopathy, where clotting factors are consumed by intravascular thrombosis 4. The fall in conc of clotting factors leads to a risk of bleeding and as all platelets are being used within the circulation there is thrombocytopenia which also increases risk of bleeding

Answer 71

Shock Sepsis/severe infection Major trauma or burns Malignancies Obstetric emergencies Severe immune-mediated reactions Severe organ dysfunction

Answer 72

Complications • Multi-organ failure • Life-threatening haemorrhage • Cardiac tamponade • Haemothorax • Intracranial haemorrhage • Gangrene and loss of digits

Answer 73

Investigations FBC Thrombocytopenia seen due to excessive consumption Coagulation screen • PT is a measure of extrinsic and common pathways of coagulation and is usually prolonged in DIC • APTT measures the intrinsic and common pathways of coagulation and is usually prolonged in DIC Clauss Fibrinogen Usually decreased as fibrinogen is converted to fibrin in intravascular thrombosis D-Dimer Typically raised providing evidence of degradation of fibrin clots around the body

Answer 74

Management 2 stages of treatment: • Treat underlying disorder to stop the triggering process • Supportive treatment to restore normal coagulation Transfusion Protocols • Platelet transfusion if the patient is bleeding • Fresh frozen plasma if PT and/or APTT is prolonged • Concentrated solution of clotting factors • Fibrinogen concentrate if fibrinogen is severely low • Therapeutic doses of heparin if thrombosis is prominent • Prophylactic doses of heparin if patient is non-bleeding and at risk of VTE

Answer 75

Presentation History: For a diagnosis to be made there must be some evidence of a precipitating factor. This can be found in a history: • Acute illness or major trauma • Symptoms of infection • Symptoms of malignancy: weight loss, anaemia, night sweats, lymphadenopathy or masses • Past medical history: underlying organ dysfunction (especially hepatic cirrhosis or pancreatitis) • Recent blood transfusion: acute haemolytic transfusion reaction • Obstetric history: symptoms of pre-eclampsia (headache, oedema, visual disturbance), fetal movements, abdominal pain or vaginal bleeding Symptoms: • Bleeding from unusual sites: ○ ears, nose, gastrointestinal tract, genitourinary tract, respiratory tract or sites of venepuncture or cannulation. ○ Bleeding from three unrelated sites is highly suggestive of DIC. • Widespread or unexpected bruising without a history of trauma • New confusion or disorientation: a sign of microvascular thrombosis affecting cerebral perfusion Clinical examination: • Signs of haemorrhage: bleeding from cannula sites/venepuncture sites, melaena, haematemesis, rectal bleeding, epistaxis, haemoptysis, haematuria • Petechiae or purpura • Livedo reticularis: a mottled lace-like patterning of the skin • Purpura fulminans: widespread skin necrosis • Localised infarction and gangrene for instance of the digits • Confusion • Oliguria, hypotension and/or tachycardia: signs of circulatory collapse, which is associated with DIC

Answer 76

The paranasal sinuses are air-filled extensions of the nasal cavity. There are four paired sinuses – named according to the bone in which they are located – maxillary, frontal, sphenoid and ethmoid. Each sinus is lined by a ciliated pseudostratified epithelium, interspersed with mucus-secreting goblet cells. The function of the paranasal sinuses is a topic of much debate. Various roles have been suggested: Lightening the weight of the head Supporting immune defence of the nasal cavity Humidifying inspired air Increasing resonance of the voice The paranasal sinuses are formed during development by the nasal cavity eroding into the surrounding bones. All the sinuses therefore drain back into the nasal cavity – openings to the paranasal sinuses can be found on the roof and lateral nasal walls.

Answer 77

There are four paired sinuses – named according to the bone in which they are located – maxillary, frontal, sphenoid and ethmoid.

Answer 78

Cranial nerve 3 Oculomotor nerve

Answer 79

Cranial nerve 9 Glossopharyngeal

Answer 80

Cranial nerve 7 Facial nerve

Answer 81

Relapsing remitting MS

Answer 82

• ?Stroke, ?encephalitis, ?GBS, ?Dehydration, ?Drugs • Simple bedside investigation for confusion - glucose for hypoglycaemia

Answer 83

Ceftriaxone is a broad-spectrum antibiotic recommended for empirical treatment of bacterial meningitis. By intravenous infusion, intravenous injection, or by deep intramuscular injection: • 2–4 g daily, doses at the higher end of the recommended range used in severe cases.

Answer 84

• Supportive treatment as symptoms are usually milder than bacterial meningitis • Most people recover completely in 7 to 10 days • Aciclovir can be used to treat suspected or confirmed HSV or VZV infection

Answer 85

Parkinson’s plus syndromes: What is progressive supranuclear palsy (PSP)? • Progressive supranuclear palsy (PSP) is a condition that causes both dementia and problems with movement. • It is a progressive condition that mainly affects people aged over 60. • The word ‘supranuclear’ refers to the parts of the brain just above the nerve cells that control eye movement. For a person with PSP, this means: ○ they may find it difficult to move their eyes in the direction they want to look – particularly up or down ○ their eyes may not co-ordinate with each other, which causes blurred or double vision ○ their eyelids may not open normally and may become stuck shut.

Answer 86

Parkinson’s plus syndrome: Dementia with Lewy bodies is a type of dementia associated with features of Parkinsonism. It causes a progressive cognitive decline. There are associated symptoms of visual hallucinations, delusions, REM sleep disorders and fluctuating consciousness.

Answer 87

Parkinson’s plus syndrome • Multiple system atrophy is a rare condition where the neurones of various systems in the brain degenerate, including the basal ganglia. • The degeneration of the basal ganglia leads to a Parkinson’s presentation. • Degeneration in other areas leads to autonomic dysfunction (causing postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia).

Answer 88

Corticobasal degeneration • A rare condition in which areas of your brain shrink and your nerve cells degenerate and die over time. • The disease affects the area of the brain that processes information and brain structures that control movement. • This degeneration results in growing difficulty in movement on one or both sides of your body. Aetiology • Corticobasal degeneration (corticobasal syndrome) can be caused by several underlying pathologies. • Most commonly, corticobasal degeneration is characterized by a buildup of tau in brain cells, which may lead to their deterioration and the symptoms of corticobasal degeneration. • Half of the people who have signs and symptoms of corticobasal degeneration have corticobasal degeneration. • The second most common cause of corticobasal degeneration is atypical Alzheimer's disease. • Other causes include progressive supranuclear palsy, Pick's disease and Creutzfeldt-Jakob disease.

Answer 89

• Underneath the cerebral cortex - “little brain” • Has many roles but its main one is primarily associated with c o ordinating movement by detecting errors which occur and correcting them so the movement appears fluid and achieves its goal. • It is also involved in motor learning and storing learnt behaviour ie driving a car/ riding a bike

Answer 90

Cerebrocerebellum: Receives input from the cerberal cortex and involved in planning and initiating movements. Spinocerebellum: Receives information about limb position and touch and pressure sensations from the spinal cord and uses this information for accurate proprioception. If there is a discrepancy, the spinocerebellar can modify motor signals to correct this. Vestibulocerebellum: Maintains equilibrium, balance and posture. Vermis: Runs along the midline of the cerebellum. Involved with posture, limb movement and eye movements.

Answer 91

The cerebellum communicates with the rest of the nervous system via three large pathways known as the cerebellum peduncles: Superior Middle Inferior

Answer 92

When information is sent to the cerebellum it takes an indirect path through to extensively branched cells called Purkinje cells. These cells then project to a group of nuclei in the centre of the cerebellum called the deep cerebellar nuclei. The deep cerebellar nuclei then sends information to various areas in the brain stem and thalamus that then can influence motor areas of the cortex or descending motor tracts to modify movement.

Answer 93

The basal ganglia (along with the substantia nigra and subthalamic nucleus) connect the rest of the cortex with the motor/premotor cortex •They ‘gate’ appropriate movements and prevent abnormal movements

Answer 94

Most of the information the basal ganglia receives, comes from the cerebral cortex and travels first to the: Caudate and Putamen - the main input nuclei of the basal ganglia The globus pallidus and substantianigra are the main output nuclei, and they send projections out from the basal ganglia to the cerebral cortex monthly via the thalamus and brain stem.

Answer 95

○ Pain. ○ Fatigue. ○ Low blood pressure. ○ Restless legs. ○ Bladder and bowel problems. ○ Skin and sweating. ○ Sleep. ○ Eating, swallowing and saliva control.

Answer 96

Bradykinesia means slowness of movement and speed (or progressive hesitations/halts) as movements are continued. The pathophysiology of bradykinesia is not well understood, but it is thought to result as a consequence of less dopamine, which leads to the inhibition of the thalamic targets and failure of basal ganglia output to reinforce the cortical mechanisms that prepare and execute the commands to move.

Answer 97

Rigidity is when muscles become stiff or inflexible. If this happens to the muscles in your face, it's called facial masking or a Parkinson's mask. Rigidity is caused by dysfunction of extrapyramidal pathways, most commonly the basal ganglia, but also as a result of lesions of the mesencephalon and spinal cord. Given that rigidity frequently arises in diseases of the basal ganglia, a tremor can coexist, giving rise to an interrupted “cogwheeling” effect.

Answer 98

Tremors Intention tremors occur when a person makes a direct and purposeful body movement. Resting tremors occur when a person is relaxed. PD tremor involves several neurotransmitters, including dopamine but also acetylcholine, serotonin and noradrenalin. PD tremor is the result of the interplay between basal ganglia and a cerebello-thalamo-cortical motor loop.

Answer 99

Dyskinesia: involuntary, erratic, writhing movements of the face, arms, legs or trunk. • Usually begin after a few years of treatment with Levodopa • Other drugs can also worsen dyskinesias e.g. COMT and MAO-B inhibitors

Answer 100

Chorea: movement disorder that causes sudden, unintended, and uncontrollable jerky movements of the arms, legs, and facial muscles. Can interfere with speech, swallowing, posture and gait, disappears in sleep. · Ongoing random-appearing sequence of one or more discrete involuntary movements. • Cause: overactivity of dopamine in the areas of the brain that control movement ○ Functional dysregulation of the basal ganglia motor circuit, where the final thalamic-cortical output is increased --> increased movement and chorea. ○ Disruption of basal ganglia: structural damage, selective neuronal degeneration, neurotransmitter receptor blockade, metabolic derangements, or autoimmune conditions. • Most common cause of Chorea is Huntington's disease

Answer 101

Huntingtons disease

Answer 102

Athetosis: abnormal muscle contraction which causes involuntary writhing movements. • Slow, continuous, involuntary writhing movement that prevents maintenance of stable posture. · Affects some people with cerebral palsy, impairing speech and use of hands. · Cause: damage to the corpus striatum (caudate and putamen) in basal ganglia which is responsible for motor control.

Answer 103

Hemiballismus: hyperkinetic involuntary movement disorder characterized by intermittent, sudden, violent, involuntary, flinging, or ballistic high amplitude movements involving ipsilateral arm and leg (one side of the body). · Is a type of chorea · Arm is affected more than leg · Cause: dysfunction of CNS of contralateral side. Lesion in the contralateral subthalamic nucleus (STN). STN important modulator of basal ganglia output.

Answer 104

Dystonia: neurological movement disorder. Involuntary muscle spasms and contractions. · Movements usually slow, repetitive and cause unusual, awkward and sometimes painful postures · Tremor often associated · Cause: abnormality/damage to basal ganglia.

Answer 105

Parkinson's disease is characterised by the progressive loss of dopaminergic neurons in the substantia nigra, leading to a reduction in dopamine levels in the basal ganglia - results in the characteristic motor symptoms of Parkinson's disease. The therapeutic approach to managing Parkinson's disease primarily aims to compensate for the depleted dopamine levels and restore the balance of dopaminergic transmission within the brain.

Answer 106

How does it work? • Works as a dopamine precursor that can cross the BBB and is converted to dopamine in the brain • By increasing dopamine levels it helps alleviate motor symptoms. • However, when taken alone, levodopa is rapidly converted to dopamine in the bloodstream, leading to significant side effects and reduced effectiveness. • Drugs such as Carbidopa and benzerazide work by inhibiting an enzyme called Dopa decarboxylase, which is responsible for the conversion of levodopa to dopamine outside of the brain. • By preventing this conversion in the bloodstream, carbidopa and benzerazide allow more levodopa to reach the brain, where it is converted to dopamine and helps alleviate the symptoms of Parkinson's disease. Examples: Carbidopa + levodopa = Sinemet and Atamet. Benzerazide and Levopoda = Madopar Disadvantages: Long-term use can lead to motor fluctuations and dyskinesias.

Answer 107

• Works by directly stimulate dopamine receptors in the brain • Mimic's the action of dopamine and help alleviate symptoms. Pramipexole Ropinirole Rotigotine.

Answer 108

• Work by blocking the breakdown of dopamine in the brain • Helps to increase and maintain dopamine levels, providing symptomatic relief and slowing disease progression Selegiline Resagiline

Answer 109

• Work by preventing the breakdown of levodopa in the peripheral tissues. • By prolonging the availability of levodopa in the brain, these drugs enhance the therapeutic effect of levodopa and reduce motor fluctuations. Entacapone Tolcapone

Answer 110

• Help balance the levels of acetylcholine and dopamine in the brain • Can be beneficial in managing tremors and other motor symptoms Trihexyphenidyl Benztropine Orphenadrine Procyclidine Biperiden

Answer 111

• Hyperactive delirium – increased sensitivity, agitation, restlessness, sleep disturbance, hypervigilance, restlessness, wandering • Hypoactive delirium – lethargic, reduced mobility/movement, lack interest in daily activities, reduced appetite, quiet, withdrawn Mixed – a combination of hyperactive and hypoactive subtypes

Answer 112

Risk Factors for Delirium: • Older age >65 (30% older medical patients experience delirium during hospitalisation) • Cognitive impairment - dementia • Malignancy • HIV • Recent surgery/trauma • Visual/hearing problems • Psychoactive drug use • Sever co-morbidity • Male gender

Answer 113

Causes of Delirium: Pain – post-op Infection – TI, LRTI Nutrition – thiamine, B12, nicotinic acid deficiency, alcohol/substance misuse Constipation/faecal impaction Hydration – urinary retention, dehydration Medication – benzodiazepines, opioids, anticholinergics, steroids Environment

Answer 114

Management of Delirium: 1. Treat underlying causes of delirium: a. Sepsis b. Drugs that are potentially causing it c. Treat deranged bloods d. Attend to exacerbating factors such as pain urine/faeces retention 2. Support the patient: a. Ensure good lighting levels, glasses/hearing aids b. Calm environment, avoiding restraint c. Encourage family visits 3. Sedation is avoided unless absolutely necessary for essential investigations/treatment, avoiding injury or relieving severe agitation. If required NICE recommends haloperidol as 1st line treatment.

Answer 115

Complications of Delirium: • Increased risk of death • Increased length of hospital stay • Nosocomial infections • Increased risk of admission to long-term care or re-admission to hospital • Increased incidence of dementia • Falls • Pressure sores • Continence problems • Malnutrition • Functional impairment • Distress for person, family and carers

Answer 116

• BPSD include: ○ Agitation ○ Aberrant motor behaviour ○ Anxiety ○ Elation ○ Irritability ○ Depression ○ Apathy ○ Disinhibition ○ Delusions ○ Hallucinations ○ Sleep or appetite changes.

Answer 117

Dementia → irreversible, progressive decline and impairment of more than one aspect of higher brain function (concentration, memory, language, personality, emotion) It is not a normal part of ageing, however, increasing age is the biggest risk factor for developing dementia Dementia can only be proven post-mortem with histological evidence. Diagnosis is usually made clinically from the history (patient and collateral), cognitive tests (6-CIT, GPCOG, MMSE etc) and formal neuropsychological assessment

Answer 118

NICE Diagnosis → initial assessment in non-specialist settings • Take a history (including, cognitive, behavioural and psychological symptoms, and the impact symptoms have on their daily life): ○ From the person with suspected dementia and ○ From someone who knows the person well • If dementia is still suspected after initial assessment: ○ Conduct a physical examination and ○ Undertake appropriate blood and urine tests to exclude reversible causes of cognitive decline and ○ Use cognitive testing • When using cognitive testing, use a validated brief structured cognitive instrument: ○ The 10-point cognitive screener (10-CS) ○ 6-item cognitive impairment test (6CIT) ○ 6-item screener ○ Memory impairment screen (MIS) ○ Mini-cog ○ Test your memory (TYM) • Refer to a specialist dementia diagnostic service if needed…

Answer 119

Alzheimer's dementia • Usually develops after the age of 60 (though, there are early-onset cases, most of which involve genetics) • Can affect ALL areas of the brain: many functions and abilities impacted and eventually lost • Most common presenting symptom → memory loss. Evidence of varying changes in planning, reasoning, speech and orientation

Answer 120

Vascular dementia • Single infarct vascular disease classically presents cognitive impairment following event • Mood disturbances and mood disorders are common • Psychosis, delusions, hallucinations and paranoia can often be seen in later stages • Patients should be screened for depression and signs of psychomotor retardation

Answer 121

Lewy body dementia • Involves visual hallucinations and Parkinson-like symptoms • If physical symptoms precede cognitive decline by more than a year, diagnosis is often Parkinson's with superimposed cognitive decline • At presentation, problems multitasking and performing complex cognitive actions are commonly the primary issue (rather than memory)

Answer 122

Frontotemporal dementia (including Pick's disease) • Can present with one of three clinical pictures: ○ Behavioural presentation: § Altered emotional responsiveness, apathy, disinhibition, impulsivity § Progressive decline noted in interpersonal skills § Changes in food preference, more childlike amusements § Obsessions and rituals may also be noted ○ Semantic presentation: § Progressive decline in understanding of word meanings § Speech may still be fluent, but there is difficulty in name-retrieval and use of less precise terms § Are unable to determine the meanings of common words when asked § Tends to develop into the inability to recognise objects or familiar faces (prosopagnosia) ○ Non-fluent presentation: § Progressive breakdown in the output of language § Speech takes effort and is not fluent § Speech apraxia (poor articulation) or disorders of speech sound § Also tends to be impaired comprehension of sentences and an impact on literacy skills

Answer 123

Mild cognitive impairment is a neurocognitive disorder that involves both memory and non-memory cognitive impairments that are not significant enough to affect the patients ADL. This condition was reclassified in 2022 in the DSM-5 and ICD-11 to be called mild neurocognitive disorder. Could be seen as the transition between normal cognitive state and a type of dementia

Answer 124

Neuropathology of MCI and Alzheimer's Similar to Alzheimer's dementia MCI/MND is most likely due to the formation of intracellular beta amyloid plaques in the brain. Or extrracellular neurofibrillary tangles (Tau), these deposits in the brain interrupt neuronal transmission and cause excitable and necrotic cell death, leading to brain atrophy and reduced cognitive function over time.

Answer 125

The cause of the presentation i.e. cognition problems , is thought to be due to cholinergic hypothesis, this is is what many of the treatments, such as anticholinesterases based on. The cholinergic hypothesis is based on the fact that there is reduced synthesis of acetylcholine in Alzheimer's brains and a loss of cholinergic neurones in the limbic system and cerebral cortex. The amyloid hypothesis suggests that the buildup of plaques is due to dysfunctions in the apolipoproteins that are responsible for clearing protein buildup in the brain. This is what is thought to contribute to the death of these cholinergic neurones.

Answer 126

Vascular dementia is characterised by multiple small cerebrovascular infarcts that mostly affect the white matter, thalamus, grey nuclei and striatum. The risk factors are typical of all microvascular diseases such as hypertension, smoking, hyperlipidaemia obesity and hypercholesterolaemia.

Answer 127

Lewy body dementia is the most rapidly progressing form of dementia, usually disease progression results in death within 7 years post diagnosis. The neuropathology of lewy body dementia is the formation of spherical protein deposits called lewy bodies (alpha synuclein). These are present in parkinsons disease but are localised to the substantia nigra, but these are more widespread in lewy body dementia.

Answer 128

Frontotemporal This is less common but affects patients younger than the other types, and many patients are diagnosed at around 65 There are several types of frontotemporal dementia but a prevalent one is called PICKS SYNDROME. This is where neuronal cell death occurs in the frontal and temporal lobes, this is also due to the deposition of proteins in the brain, mainly Tau. Has semantic presentation results in: • Progressive decline in understanding of word meanings • Difficulty in name retrieval and use of precise terms • Unable to determine the meaning of common words • Propagnosia Also has a non fluent presentation: Progressive breakdown in the output of language Poor fluency and impact on comprehension of language and writing Speech takes effort Apraxia

Answer 129

1. Alzheimer's Disease: This is the most common type of dementia, characterized by the accumulation of beta-amyloid plaques and tau tangles in the brain. The course of Alzheimer's disease is generally gradual, with symptoms worsening over time. Prognosis can vary, but the average life expectancy after diagnosis is typically around 3 to 9 years, although some individuals may live for up to 20 years or more.

Answer 130

2. Vascular Dementia: This type of dementia is caused by reduced blood flow to the brain, often due to stroke or small vessel disease. The progression of vascular dementia can be stepwise, with symptoms worsening in a step-by-step manner after each new stroke, often showing a period of stability at one level of functioning, before an acute decline progression, followed by another period of stability. The prognosis depends on the severity and number of strokes, as well as the individual's overall health.

Answer 131

3. Lewy Body Dementia: This type of dementia is associated with abnormal protein deposits in the brain, known as Lewy bodies. The course of Lewy body dementia can be fluctuating, with symptoms varying in severity from one day to the next. It can progress rapidly and prognosis is often linked to the development of motor symptoms similar to those of Parkinson's disease and can vary based on the individual's response to treatment. Mortality is often within the first 7 years post diagnosis.

Answer 132

4. Frontotemporal Dementia: This type of dementia is characterized by the degeneration of the frontal and temporal lobes of the brain, leading to changes in personality, behaviour, and language. The course of frontotemporal dementia can vary widely, but it often progresses more rapidly than Alzheimer's disease. Prognosis depends on the specific subtype of the disease and the individual's overall health. Average life expectancy is 8 years post diagnosis.

Answer 133

Epilepsy is an umbrella term for a condition where there is a tendency to have seizures. Seizures are transient episodes of abnormal electrical activity in the brain. There are many different types of seizure.

Answer 134

Generalised Tonic-Clonic Seizures Focal Seizures Absence Seizures Atonic Seizures Myoclonic Seizures Infantile Spasms Febrile convulsions

Answer 135

Generalised Tonic-Clonic Seizures These are what most people think of with an epileptic seizure. There is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) movements. Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or low. Management of tonic-clonic seizures is with: First line: sodium valproate Second line: lamotrigine or carbamazepine

Answer 136

Focal Seizures Focal seizures start in the temporal lobes. They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present: Hallucinations Memory flashbacks Déjà vu Doing strange things on autopilot One way to remember the treatment is that the choice of medication is the reverse of tonic-clonic seizures: First line: carbamazepine or lamotrigine Second line: sodium valproate or levetiracetam

Answer 137

Absence Seizures Absence seizures typically happen in children. The patient becomes blank, stares into space and then abruptly returns to normal. During the episode they are unaware of their surroundings and won’t respond. These typically only lasts 10 to 20 seconds. Most patients (more than 90%) stop having absence seizures as they get older. Management is: First line: sodium valproate or ethosuximide

Answer 138

Atonic Seizures Atonic seizures are also known as drop attacks. They are characterised by brief lapses in muscle tone. These don’t usually last more than 3 minutes. They typically begin in childhood. They may be indicative of Lennox-Gastaut syndrome. Management is: First line: sodium valproate Second line: lamotrigine

Answer 139

Myoclonic Seizures Myoclonic seizures present as sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy. Management is: First line: sodium valproate Other options: lamotrigine, levetiracetam or topiramate

Answer 140

Infantile Spasms This is also known as West syndrome. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free. It can be difficult to treat but first line treatments are: Prednisolone Vigabatrin

Answer 141

Febrile Convulsions Febrile convulsions are seizures that occur in children whilst they have a fever. They are not caused by epilepsy or other underlying neurological pathology (such as meningitis or tumours). By definition, febrile convulsions occur only in children between the ages of 6 months and 5 years. Febrile convulsions do not usually cause any lasting damage. One in three will have another febrile convulsion. Having febrile convulsions slightly increases the risk of developing epilepsy in the future.

Answer 142

Management of status epileptics in the hospital (take an ABCDE approach): Secure the airway Give high-concentration oxygen Assess cardiac and respiratory function Check blood glucose levels Gain intravenous access (insert a cannula) IV lorazepam, repeated after 10 minutes if the seizure continues If the seizures persist the final step is an infusion of IV phenobarbital or phenytoin. At this point intubation and ventilation to secure the airway needs to be considered, along with transfer to the intensive care unit if appropriate. Medical options in the community: Buccal midazolam Rectal diazepam

Answer 143

Status Epilepticus Status epilepticus is an important condition you need to be aware of and how to treat. It is a medical emergency. It is defined as a seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.

Answer 144

An electroencephalogram (EEG) shows typical patterns in different forms of epilepsy and supports the diagnosis. An EEG is a test that measures electrical activity in the brain using small, metal discs (electrodes) attached to the scalp. EEG's can help diagnose a number of conditions including epilepsy, sleep disorders and brain tumours. MRI brain is used to diagnose structural pathology (e.g. tumours) that may be the cause of seizures or be associated with epilepsy. Additional investigations can be considered to exclude associated pathology: • ECG • Serum electrolytes, including sodium, potassium, calcium and magnesium • Blood glucose for hypoglycaemia and diabetes • Blood cultures, urine cultures and lumbar puncture where sepsis, encephalitis or meningitis is suspected

Answer 145

Factors that can affect altered states of consciousness in epilepsy 1. Seizure type - Generalised seizures can cause complete loss of consciousness - Focal seizures might result in altered awareness or consciousness without complete loss 2. Seizure severity / length 3. Seizure Frequency - Increased frequency can impact function - lead to chronic alterations in consciousness 4. Medication effects - Some antiepileptic medications can contribute to altered consciousness such as drowsiness, confusion or cognitive impairment 5. Underlying brain abnormalities - Tumours or lesions can magnify consciousness level 6. Postictal State (state after a seizure) - Duration and intensity of postictal state can vary depending on the seizure type and severity

Answer 146

ROLE OF THE PREFRONTAL CORTEX The prefrontal cortex helps people set and achieve goals. It receives input from multiple regions of the brain to process information and adapts accordingly. The prefrontal cortex contributes to a wide variety of executive functions, including: • focusing one’s attention • predicting the consequences of one’s actions; anticipating events in the environment • impulse control; managing emotional reactions • planning for the future • coordinating and adjusting complex behaviours (“i can’t do a until b happens”)

Answer 147

ROLE OF THE LIMBIC SYSTEM • It combines higher mental functions and primitive emotion into a single system often referred to as the emotional nervous system. It is not only responsible for our emotional lives but also our higher mental functions, such as learning and formation of memories. The limbic system is the reason that some physical things eg eating, seem so pleasurable to us, and the reason why some medical conditions, such as hypertension, are caused by mental stress. • There are several important structures within the limbic system: the amygdala, hippocampus, thalamus, hypothalamus, basal ganglia, and cingulate gyrus. • The limbic system is among the oldest parts of the brain in evolutionary terms: it can be found in fish, amphibians, reptiles and mammals. • The pleasure centre is located in the limbic system. It is involved in sexual arousal and in the "high" derived from certain recreational drugs.

Answer 148

The Amygdala • Small almond-shaped structure; there is one located in each of the left and right temporal lobes. Known as the emotional centre of the brain, the amygdala is involved in evaluating the emotional valence of situations (e.g., happy, sad, scary). It helps the brain recognise potential threats and helps prepare the body for fight-or-flight reactions by increasing heart and breathing rate. The amygdala is also responsible for learning on the basis of reward or punishment. • Due to its close proximity to the hippocampus, the amygdala is involved in the modulation of memory consolidation, particularly emotionally-laden memories. Emotional arousal following a learning event influences the strength of the subsequent memory of that event, so that greater emotional arousal following a learning event enhances a person’s retention of that memory.

Answer 149

The Hippocampus • Found deep in the temporal lobe, and is shaped like a seahorse. It consists of two horns curving back from the amygdala. Psychologists and neuroscientists dispute the precise role of the hippocampus, but generally agree that it plays an essential role in the formation of new memories about past experiences.

Answer 150

The Cingulate Gyrus • Located in the medial side of the brain next to the corpus callosum. Its frontal part links smells and sights with pleasant memories of previous emotions. This region also participates in our emotional reaction to pain and in the regulation of aggressive behaviour.

Answer 151

The Thalamus and Hypothalamus • Both associated with changes in emotional reactivity. The thalamus (sensory “way-station” for the rest of the brain) is primarily important due to its connections with other limbic-system structures. The hypothalamus is a small part of the brain located just below the thalamus on both sides of the third ventricle. Lesions of the hypothalamus interfere with several unconscious functions (such as respiration and metabolism) and some so-called motivated behaviours like sexuality, combativeness, and hunger. The lateral parts of the hypothalamus seem to be involved with pleasure and rage, while the medial part is linked to aversion, displeasure, and a tendency for uncontrollable and loud laughter.

Answer 152

The Basal Ganglia • A group of nuclei lying deep in the subcortical white matter of the frontal lobes that organises motor behaviour.

Answer 153

• The formation of visual memories, including long-term memories. In conjunction with the amygdala and hippocampus, two structures of the limbic system, the temporal lobe is vital for the formation of conscious memories. • Wernicke's area: This brain region is associated with the understanding and processing of speech. • Broca's area: This brain region aids in the production of speech, though some evidence suggests that, when Broca's area is damaged, nearby regions may compensate. Together with Wernicke's area, Broca's area aids communication.

Answer 154

The insula's role in somatic states means that it provides an emotional context for physiological experiences. In other words, it gives meaning to bodily states by, for example, helping you experience pain as unpleasant.

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