Neurology Flashcards

Question

acute management: ischemic stroke

Answer 1

abcde non-contrast ct 3) hyper-acute treatments - If presenting within **4.5 hours of symptom onset - Thromboysis** with **Alteplase** (tissue plasminogen activator) - contraindications: recent head trauma, GI or intracranial haemorrhage, recent surgery, BP>185/110, platelet count, INR > 1.7, elevated PTT, glucose <50mg, stroke or head trauma in the past 3 months - Otherwise, **Mechanical Thrombectomy** - in patients with **anterior circulation strokes** within **6 hours of symptom onset** - in **posterior circulation strokes** up to **12 hours after onset** 4) Aspirin 300 mg orally once daily for two weeks - If hyper-acute treatments are offered, aspirin is usually started **24 hours after the treatment** **following a repeat CT Head** that excludes any new haemorrhagic stroke. 5) Consider **decompressive hemicraniectomy (which should be performed within 48 hours of symptom onset)** for people with acute stroke who meet all of the following criteria: - Clinical deficits that suggest **infarction** in the territory of the **middle cerebral artery (**with a score >15 on the National Institutes of Health Stroke Scale (NIHSS)) - Decreased level of consciousness, - Signs on CT of an infarct of at least 50% of the middle cerebral artery territory

Answer 2

abcde non-contrast CT 3) Neurosurgical and neurocritical care evaluation due to the potential for surgical intervention (e.g. decompressive hemicraniectomy). - Craniotomy is done to relieve pressure and stereotactic aspiration to aspirate blood using CT scan for guidance and relieve pressure. 4) Admission to the neuro ICU or stroke unit (the patient may require intubation and ventilation or invasive monitoring of ICPs). 5) Aim to keep blood pressure <140/80 as poor blood pressure control in the acute stage is associated with poorer outcomes later on - labetalol or nimodipine 6) Urgently reverse abnormalities of clotting, particularly in patients taking anticoagulants

Answer 3

Warfarin/ vit K antagonist: prothrombin complex concentrate/fresh frozen plasma and phytomenadione (vit K) Dabigatran: idraucizumab Factor Xa inhibitor reversal: prothrombin complex concentrate

Answer 4

Investigations in the post-acute phase aim to further define the cause of the stroke and to quantify vascular risk factors. Further investigations to determine the cause of the stroke include, for example: - In ischaemic stroke: - carotid doppler ultrasound (to identify critical carotid artery stenosis) - CT/MR angiography (to identify intracranial and extracranial stenosis) - 12h ECG & echocardiogram (if a cardio-embolic source is suspected) - In young patients further investigation e.g. a vasculitis screen or thrombophilia screen may be necessary. - In haemorrhagic stroke: - serum toxicology screen (sympathomimetic drugs e.g. cocaine are a strong risk factor for haemorrhagic stroke). - to quantify vascular risk factors include: - serum glucose (all patients with stroke should be screened for diabetes with a fasting plasma glucose or oral glucose tolerance test) - serum lipids (to check for raised total cholesterol/LDL cholesterol).

Answer 5

The key steps in secondary stroke prevention can be remembered by the mnemonic HALTSS: - Hypertension: - Anti-hypertensive therapy should, however, be initiated 2 weeks post-stroke. - no benefit in lowering the blood pressure acutely (as this may impair cerebral perfusion) unless there is malignant hypertension (systolic blood pressure >180 mmHg). - Antiplatelet therapy: - patients should be administered Clopidogrel 75 mg once daily for long-term antiplatelet therapy (after 2 weeks of aspirin). - If clopidogrel CI, then aspirin &dipyrimidole - In patients with ischaemic stroke secondary to atrial fibrillation, and chadVASc ≥2, warfarin (target INR 2-3. or a direct oral anticoagulant (such as Rivaroxaban or Apixiban) is initiated 2 weeks post-stroke. - Lipid-lowering therapy: - patients should be prescribed high dose atorvastatin 20-80 mg once nightly (irrespective of cholesterol level this lowers the risk of repeat stroke). - Do not start statin treatment immediately. There is consensus that it is safe to start statins after 48 hours - Tobacco: - offer smoking cessation support. - Sugar: - patients should be screened for diabetes and managed appropriately. - Surgery: - patients with ipsilateral carotid artery stenosis more than 50% should be referred for carotid endarterectomy. - (carotid endarterectomy also indicated for non-stroke patients with 70% occlusion) - Feeding assessment and management - swallow - All patients presenting with acute stroke must be screened for safe swallowing function prior to further oral intake, as dysphagia is common after stroke - if unsafe: NG tube feeding, if not tolerated → nasal bridle tube/gastrostomy instead

Answer 6

used for AF stroke risk CHF +1 Hypertension +1 Age >= 75 +2 Diabeter +1 Stroke/TIA/VTE +2 Vasc disease +1 Age 65-74 +1 Sex (female) +1 1- oral anticoagulant should be considered 2 or oral - anticoagulant is recommended

Answer 7

complete = complete loss of function (motor power, sensory input, reflexes)→ may enter spinal shock - caused by complete transection of the cord or compression - loss of involuntary movement & sensation if parts enervated by segment - spinal shock - irreversible Incomplete = some preservation of function below level of injury - often from contusions produced by pressure on the cord - clinical picture unpredictable - some function is present below site.

Answer 8

1. Brown-Sequard Syndrome 2. Anterior Cord Syndrome 3. Posterior Cord Syndrome 4. Central cord syndrome 5. Cauda Equina Syndrome 6. Conus Medullaris Syndrome

Answer 9

a lesion affecting T6 or higher. triggered by excessive autonomic response to stimuli below the level of the lesion, such as a faecal impaction or blocked catheter. The abnormal response produces autonomic imbalance with sympathetic overactivity. results in: unbalanced physiological response: **extreme hypertension, flushing and sweating above the level of the cord lesion** - agitation - in untreated cases severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke.

Answer 10

history - Nature and mechanism - is it Complete, incomplete, absent SCI at scene - was Methylprednisoloone given/not given/ - Concomitant injuries. Examination 1. Initial Assessment **A** Airway and cervical spine immobilisation. **B** Breathing: adequacy of oxygenation and ventilation. **C** Circulation: Fluid resuscitation(in trauma patients hypotension should always be considered to be secondary to blood loss, not spine shock). **D** Disability: GCS, pupil size. 2. Associated injuries 3. Level of spinal injury: Radiological and clinical. (level= Most distal uninvolved nerve root segment with normal function) complete or incomplete tetraplegia or paraplegia?

Answer 11

Paraplegia is a paralysis starting in the thoracic (T1-T12), lumbar (L1-L5) or sacral (S1-S5) area, while tetraplegia is caused by damage in the cervical area (C1-C8). Persons with paraplegia possess good functioning of the arms and hands.

Answer 12

- Routine **Bloods** and ABG- FBC, U&Es, **calcium**, ESR, immunoglobulin electrophoresis (multiple myeloma) - **Radiology** - Trauma series for trauma: First CT cervical spine, then CT of whole spine to look for injury somewhere else in the spine. Imaging of cervical spine is especially important - Refer urgently to a neurosurgeon or spinal surgeon any patient with clinical signs of a **spinal cord injury** (i.e., an abnormal neurological exam). An MRI of the spine is indicated (in addition to the CT) in these patients. ( T2 weighted saggital MRI scan ) - Patient with clinical features suggestive of spinal cord compression or cauda equina syndrome should have an urgent WHOLE spine MRI, with an aim (in appropriate cases) to surgically decompress within 48 hours. - **Monitor**: pulse oximetry, ecg, BP, twice daily VC, UO with catheter. ICU monitoring is needed. - **Urine** - look for Bence Jones proteins (multiple myeloma)

Answer 13

1. ABCDE - Maintain SpO2 >98% and if C3 injury or above then do invasive ventilation/tracheostomy. IF T6 and above do non-invasive ventilation or bird respirator. - Maintain cardiovascular system and prevent/treat spinal shock - prevent hypotension (SBP >90mmHg). - Patient may need ICU monitoring (cardiac, haemodynamic, respiratory monitoring to detect any dysfunction) and they should be transferred to specialist centres. 2. full in-line spinal immobilisation if needed 3. Pain management: - In acute phase of trauma: intravenous morphine as the first-line analgesia. If intravenous access has not been established, consider diamorphine or ketamine via the intranasal route (follow local protocols) - For chronic spinal cord injury - Nociceptive pain: physiotherapy and simple analgesia - Neuropathic: Gabapentin/ pregabalin/ amitriptyline (first line). Oxycodone/ tramadol/ venlafaxine (second line) 4. If progressive neurologic deficit: urgent assessment. - Surgical decompression in 48 hours if needed- if there is presence of retained foreign objects, CSF leakage, infection or signs of extrinsic spinal cord compression. - If progressive neurology is secondary ischemia or inflammation- no surgery. 5. Treat underlying autonomic dysreflexia if present, treated by resolving: urinary retention (catheter) , rectal examination (check for and remove faecal impaction. If doesn't resolve vasodilator to lower BP (sublingual nifedipine or glyceryl trinitrate ) 6. Supportive care: resp, pressure sores, bladder & bowel management - spinal shock is autonomic function loss - Blood pressure should be maintained - DO NOT give steroids in acute stage - in patients where malignancy is demonstrated on MRI, or in patients where clinical suspicion is high, administration of dexamethasone 16 mg daily in divided doses (with PPI cover) is indicated. - Immoblisation to prevent further injury - refer to specialised centres 7. Physiotherapy and mobilisation

Answer 14

lateral hemisection or loss of half of the spinal cord trauma (knife or gunshot), tumour 1. Lateral corticospinal tract 2. Dorsal columns 3. Lateral spinothalamic tract Below the lesion: - ipsilateral weakness below lesion (UMN motor signs= spastic paresis ) - ipsilateral loss of proprioception and vibration sensation - contralateral loss of pain and temperature sensation At the level of the lesion: → Ipsilateral: complete sensory, (vibration, pain, proprioception, temperature) and motor loss (LMN lesion sign) if lesion is above T1 → Horner's syndrome: Constricted pupil, eyelid droop

Answer 15

2nd most common - Usually caused by **cervical flexion injury** , which occludes anterior spinal artery and/ or damages anterior spinal column - damage to the aorta, including both aneurysm repair and dissection. Lateral corticospinal tracts Lateral spinothalamic tracts 1. Bilateral spastic paresis (motor function lost) 2. Bilateral loss of pain and temperature sensation 3. Autonomic dysfunction (loss of sympathetic) can manifest as **hypotension** (either orthostatic or frank hypotension), sexual dysfunction, and/or bowel and bladder dysfunction (Vibration, proprioception intact )

Answer 16

Most common spinal cord injury - hyperextension injury - usually in elderly with existing stenosis - syringomyelia or intramedullary cord tumors - Impairment of the upper limb pain and temperature sensation. U Limb> LL - patients will walk with bowel/ bladder function preservation - Usually spinothalmic tract affected

Answer 17

compression by tumor or infarct of posterior spinal artery - loose function of dorsal column → proprioception, two point discrimination are lost so keep falling over - Preservation of motor function, pain and temperature sensation

Answer 18

Compression of the Cauda Equina, which is a continuation of the spinal nerves inferiorly beyond the Conus medullaris, L2-sacrum lumbosacral nerve roots more gradual and unilateral presentation more severe Massive disk rupture Trauma, tumour dysfunction of posterior longitudinal ligament, central cord prolapse Function of Cauda Equina nerve roots - Motor and sensory to lower extremity - Pelvic floor/ sphincter innervation So in Cauda Equina syndrome - severe low back pain - severe bilateral sciatica - Saddle anaesthesia - loss of anocutaneus reflex (when you touch skin outside anal sphincter, contraction or muscle) - bilateral sensory anal loss - Bowel and bladder dysfunction- prevents later - Normal babinski (Cauda Equina nerves are peripheral nerves so **LMN** findings seen not UMN findings) - both knee and ankle jerks affected -> arreflexia

Answer 19

L1-L2 sacral cord segment and roots acute and bilateral presentation symmetrical, less marked hypperreflexia - perianal anaesthesia bilaterally, as opposed to entire saddle area - Impotence more frequent - More acute presentation

Answer 20

M>F Mean age of onset: 55 yrs - 5-10% have a **family history** with autosomal dominant inheritance (genes including SOD1, FUS and C9ORF72) - 90% sporadic - Risk factors include genetic predisposition or family history, and age >40 years. - **Associations** - Frontotemporal lobar dementia - familial cases → zinc copper superoxide dismutase deficiency → increased free radical damage - Free radical damage and glutamate excitotoxicity have been implicated

Answer 21

Amyotrophic Lateral Sclerosis (ALS) (50%) (a.k.a. Spinal ALS) Progressive Bulbar Palsy Variant Progressive Muscular Atrophy Variant Primary Lateral Sclerosis Variant

Answer 22

- **Amyotrophic Lateral Sclerosis (ALS) (50%) (a.k.a. Spinal ALS)** - AKA Lou Gehrig's disease - Combined generation of upper AND lower motor neurones resulting a mix of LMN and UMN signs - Combination of UMN and LMN signs - **LMN Features (anterior motor horn)** - Muscle wasting - Fasciculations - e.g. tongue fasiculations - Flaccid weakness, paralysis - Hyporeflexia, hypotonia - negative Babinski - **UMN Features (lateral corticospinal tract)** - Spastic weakness, paralysis - Extensor plantar response (babinski sign ) - Hyperreflexia, hypertonia - Speech disturbance (slurring or reduction in volume) - Swallowing disturbance (e.g. choking on food, especially solid foods), dysphagia - cranial nerves can be involved - Behavioural changes (e.g. disinhibition, emotional lability) - wasting & weakness of the small hand muscles/tibialis anterior is common - early sign - **Sensory examination** - should be NORMAL - • doesn't affect ocular muscle, no cerebellar signs, no sphincter disturbances Late symptoms: immpbile, cannot communicate, unable to swallow, drooling, breathing difficulties

Answer 23

- **Primary Lateral Sclerosis Variant** - UMN pattern of weakness - Brisk reflexes - Extensor plantar responses - NO LMN signs

Answer 24

- **Progressive Muscular Atrophy Variant** - Only LMN signs - affects distal muscles before proximal - carries best prognosis

Answer 25

- **Progressive Bulbar Palsy Variant** - palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of **brainstem** motor nuclei - Dysarthria - Dysphagia - Wasted fasciculating tongue - Brisk jaw jerk reflex - **carries worst prognosis**

Answer 26

clinical diagnosis - **Bloods** - Mild elevation in CK, ESR - negative Anti-GM1 ganglioside antibodies (multifocal mononeuropathy) & negative acetylcholine receptor antibodies (myasthenia gravis) - **Electromyography (EMG)-** reduced number of action potentials with increased amplitude - **Nerve conduction studies** - often normal, can help exclude a neuropathy. - **MRI** brain & spine- exclude cord compression, myelopathy and brainstem lesions - **Spirometry** - assess respiratory muscle weakness

Answer 27

no cure. The focus of medical care is to provide supportive and palliative intervention, aiming to improve the quality of life for patients Riluzole (50mg twice daily) - only drug for modifying the disease course and should be offered to patients at the time of diagnosis. - decreases glutamate release by neurones - used mainly in amyotrophic lateral sclerosis - increases ventilation free survival by just a few months - monitor LFTs and FBCs monthly for first three months, and then every 3 months thereafter Supportive care muscle cramps- tonic water, quinine spasticity- baclofenac drooling - atropine, hyoscine patch, botox pain- amitriptyline, gabapentine. pregabalin Difficulty expelling mucus secretion - carbocystine - ongoing counselling regarding prognosis and end-of-life issues, with special emphasis on advance directives. Respiratory care - non-invasive ventilation positive pressure ventilation (usually BIPAP) can also prolong survival in patients with type 2 respiratory failure. - If severe dyspnea - chronic invasive ventilation - If do not choose ventilation - palliative care is used at Dysphagia & weight loss - diety modification - when dysphagia present → Percutaneous endoscopic gasterstomy (PEG) speech and language therapy PEG/RIG

Answer 28

usually fatal in 3-5 years common cause of death is aspiration pneumonia

Answer 29

Demyelination of the posterior columns (Vibration/ proprioception loss), and a loss of lateral motor tracts (upper motor lesion symptoms) due to vitamin B12 ( cobalamin) deficiency investigation serum B12, folate (exclude folate def.), MRI (identify demyelinated lesion) management Vitamin B12

Answer 30

- bilateral neurological deficit of the legs - bowel bladder dysfunction - Perianal or perineal sensory loss (saddle anaesthesia or paraesthesia). - Unexpected laxity of the anal sphincter

Answer 31

- Sudden onset of severe central spinal pain which is relieved by lying down. - history of major trauma (such as a road traffic collision or fall from a height), minor trauma, or even just strenuous lifting in people with osteoporosis or those who use corticosteroids. - Structural deformity of the spine (such as a step from one vertebra to an adjacent vertebra) may be present. - may be point tenderness over a vertebral body

Answer 32

- >50 - gradual onset of symptoms - Severe unremitting pain that remains when the person is supine, aching night pain that prevents or disturbs sleep, pain aggravated by straining (for example, at stool, or when coughing or sneezing), and thoracic pain. - Localised spinal tenderness. - No symptomatic improvement after four to six weeks of conservative low back pain therapy. - Unexplained weight loss - Past history of cancer — breast, lung, gastrointestinal, prostate, renal, and thyroid cancers are more likely to metastasize to the spine.

Answer 33

- Fever - Tuberculosis, or recent urinary tract infection. - Diabetes. - History of intravenous drug use. - HIV infection, use of immunosuppressants, or the person is otherwise immunocompromised.

Answer 34

primary = initial damage to anatomical components. Cannot be reversed, stopped or fixed secondary = inflammatory cascade after primary. Can be prevented, managed, reversed

Answer 35

- MOST CASES: trauma and tumours - Trauma can lead to compression by: (usually road traffic accidents and falls in elderly) - Direct cord contusion - Compression by bone fragments - Haematoma - Acute disk prolapse - Tumours are more frequently **METASTASES** -degeneration (bony outgrowths) -Infection: spinal abscess, TB (**Pott's disease**) - **Risk Factors** - Trauma - Osteoporosis - Metabolic bone disease - Vertebral disc disease

Answer 36

Fluid-filled space develops in the spinal canal which damages spinothalmic tract nerve fibres crossing centre - So bilateral loss of pain/temperature - Usually C8-T1 (arms/ hands) can expand to anterior horn (LMN weakness) and lateral horn (Horner's syndrome) RFs: - tauma - can occur years after spinal cord injury - type 1 Arnold chiari malformation of cerebellum - chiari malformation Dx - MRI w contrast of brain to exclude a Chiari malformation and spine to exclude a tumour or tethered cord Tx - treat underlying cause. Recurrent = place shunt into syrinx

Answer 37

clinical emergency in individuals with spinal cord injury Autonomic Dysreflexia is an uninhibited sympathetic nervous system response to a variety of noxious stimuli occurring in people with spinal cord injury at the thoracic six (T6) level and above. presentation: unbalanced physiological response: extreme hypertension, flushing and sweating above the level of the cord lesion Management: underlying cause and symptomatic management

Answer 38

inflammation of the leptomeningeal (pia and arachnoid mater)

Answer 39

Men C (meningococcus), pneumococcal and Hib vaccines (H. influenzae)

Answer 40

Group B strep Listeria monocytogenes E coli

Answer 41

strep pneumonia (most common) Haemophilus influenza (most common in unvaccinated children) Neisseria meningitidis

Answer 42

Listeria monocytogenes Neisseria meningitidis Strep pneumonia

Answer 43

- Listeria monocytogene, mycobacteria (including *M. tuberculosis*) and CMV - Cryptococcal meningitis in HIV/AIDS

Answer 44

Gram positive cocci in chains catalase -, CAMP TEST +, Beta haemolytic

Answer 45

motile, gram negative bacillus (rod) some have virulence factor: K-1 capsular antigen

Answer 46

gram positive rod facultative intracellular tumbling motility

Answer 47

gram negative cocci in pairs - diplococci - resp droplet transmission - virulence factors: polysccharide preventing phagocytosis, and lipooligosaccharide outer membrane - endotoxin meningitis, meningococcemia sepsis purpuric rash DIC waterhouse-friderichsen syndrome outbreaks in crowded places - dorms infected -> droplet precaution close contacts -> rifampacin (or ceftriaxone/ ciprofloxacin)

Answer 48

Lancet shape gram + cocci in pairs increased risk in: asplenic patients, sickle cell, alcoholic meningitis, ottitis media (kids), pneumonia, sinusitis

Answer 49

small gram negative rod un immunised children - may immigrate from another country

Answer 50

- Coxsackievirus (children; fecal-oral transmission) - Enteroviruses- echovirus - HSV 2- can cause meningitis or encephalitis - polivirus - Paramyxovirus: can be a complication of mumps infection - Measles and rubella viruses - VZV- can be a complication of chicken pox - HIV - Arboviruses - arthropod-borne viruses, cause meningoencephalitis - Rabies virus - can cause meningo-encephalitis

Answer 51

HSV-1 -> oral herpes, eye infections, encephalitis (esp temporal lobe) HSV-2 -> genital herpes. meningitis

Answer 52

- *Cryptococcus* (common cause of meningitis in HIV patients) - mycobacterial tb

Answer 53

- Amoeba: Acanthamoeba - associated with keratitis and meningitis associated with contact lens fluid contamination. - Toxoplasma gondii

Answer 54

- Malignancy (leukaemia, lymphoma and other tumours) - Sarcoidosis - Inflammatory conditions: Systemic Lupus Erythematosus, Behcet's disease - Chemical meningitis - Drugs (NSAIDs, trimethoprim) - Aseptic meningitis (not due to microbes) - Mollaret's meningitis (recurrent benign lymphocytic meningitis) - rarely: cancer, sarcoid, inflammatory diseases

Answer 55

- Severe headache - Photophobia - neck stiffness (nuchal ridgidity) , Neck or backache - Signs of INFECTION**-** Fever, Tachycardia, Hypotension - Irritability - focal neurology - seizures - Drowsiness & altered mental status - Vomiting - High-pitched crying or fits (common in children) - Reduced consciousness - baby - bulging fontanelle - Skin rash - Meningococcal disease is an acute contagious life-threatening illness, characterised by **fever, petechial or purpuric rash,** and s**igns of sepsis and/or meningitis.** It is a notifiable disease in the UK. - **IMPORTANT: take a good travel history and exposure history and take not of exposure to any of the following** - Rodents (lymphocytic choriomeningitis virus) - Ticks (Lyme borrelia, Rocky Mountain spotted fever) - Mosquitoes (West Nile virus) - Sexual activity (HSV-2, HIV, syphilis) - Travel - Neonatal meningitis is difficult to diagnose: irritable, poor feeding, fever - **Kernig's Sign** - with the hips flexed, there is pain/resistance on passive knee extension. Because you tug on meninges when you do this. - Aseptic meningitis (not due to microbes) - Mollaret's meningitis (recurrent benign lymphocytic meningitis) - rarely: cancer, sarcoid, inflammatory diseases - Brudzinski's Sign - involuntary flexion of the hips when the neck is flexed Neonatal: • Non-specific symptoms – irritability, difficulty feeding, pyrexia, respiratory difficulty

Answer 56

(treatment should be started as soon as possible. Must not be delayed by investigations, but get blood culture before starting antibiotics as can identify organism - don't need to wait for culture to come back before starting treatment ) Lumbar Puncture - DIAGNOSIS (needle between L4/L5) (GOLD STANDAED) Don't do LP if - signs of space occupying lesion: impairment of consciousness, focal neurological signs, papilloedema, after seizures, relative bradycardia with hypertension, immunocompromised or patients with systemic shock. Bloods - FBC, U&E (low sodium may indicate tb meningitis), cotting (DIC), glucose, VBG, ABG, two sets of blood cultures, serum pneumococcal and meningiococcal PCR , HIV Imaging CT prior to LP is not always necessary, but good practice, and only essential in those whose presenting features may suggest a space-occupying lesion – such as a brain abscess. CT if If there is a suggestion of encephalitis eg altered cognition, altered behaviour or new seizures

Answer 57

lumbar puncture, whilst lying on side, can also check for opening pressure - Bacterial meningitis: - Cloudy CSF - **High neutrophils** - High protein - **Low glucose** - Viral meningitis: - **High lymphocytes** - High or normal protein - **Normal glucose** - TB meningitis: - Fibrinous CSF - **High lymphocytes** - High protein - **Low glucose** - negative gram stain (need Auramine staining) Classically the opening pressure is very high in Crytococcal meningitis (poor prognostic sign)- differential in any HIV or immunocompromised patient.

Answer 58

viral meningitis (despite being the most common cause of meningism) is clinically indistinguishable from bacterial meningitis. all cases of suspected meningitis should be treated empirically as bacterial meningitis until proven otherwise. Initial - stabilise patients (A-E) - **IMMEDIATE Empiric IV Antibiotics** (before specific organism identified) - First choice: 3rd generation **cephalosporin** (e.g. **cefotaxime or ceftriaxone) (secondary care) within 1st hour of arrival** - 2g of IV ceftriaxone twice daily (twice the standard dose to ensure CNS penetration - In penicillin allergic patients second line antibiotics such a chloramphenicol - vancomycin or rifampicin IV - if patients are at risk of penicillin-resistant pneumococci or have travelled within the last 6 months, - bezylpenecillin IV/IM used in primary care at first presentation before hospital transport (Or cephalosporin if available. ) - still give hospital antibiotics after - Use IV cefotaxime in neonates and infants,as ceftriaxone may worsen jaundice orcause acidosis, particularly in newborns or premature babies - then add IV amoxocilin if infants or >50 (antibiotic cover for Listeria monocytogenes) - OR trimethoprim/sulfamethoxazole - if viral encephalitis is suspected, add IV aciclovir Antibiotic options: ceftrioxone, vancomycin, ampicillin, gentamicin - **Dexamethasone IV** - Given shortly before or with the first dose of antibiotics to ALL patients - **should be given within 4 hours of antibiotic therapy and no later than 12 hours after antibiotics have been administered** - (iStop intravenous dexamethasone if a cause other than *S pneumoniae* is identified (or *Haemophilus influenzae?))* - **NICE advises against the use of high-dose steroids in meningococcal septicaemia,** - • **should not be used in infants who are less than 3 months old** - Dexamethasone reduces morbidity and mortality in bacterial meningitis. This has been shown to improve outcomes if given within 4 hours of IV antibiotics. It has a modest reduction in mortality however significantly reduces hearing loss and neurological sequelae. If the patient is showing signs of meningism, like this patient is, it is important to administer this quickly. - **Resuscitation**

Answer 59

HiB (10 days), Enterobacteria (21d), Step pneumonia (10d), meningococcal → ceftriaxone or cefotaxime Listeria monocytogenes (21d) → amoxicillin Staph aureus (14d) → flucocoxacillin (+ rifmampicin sometimes) MRSA → vancomycin (if resistant, linezolid or rifampicin) For TB meningitis treat with isoniazid, rifampicin, pyrazinamine, ethambutol Viral → self limiting. support care usually. If HSV or VZV confirmed: aciclovir/ vanciclovir. If CMV confirmed: ganciclovir/ valganciclovir Fungal → Induction: if cryptococcal: amphotericin B & fluconazole & antiretroviral (ART), if histoplasmal or other→ amphotericin B Maintainance is with azole, usually fluconazole

Answer 60

- **Respiratory isolate** all patients with suspected meningitis or meningococcal sepsis until meningococcal meningitis or meningococcal sepsis is excluded or ceftriaxone (or other recommended antibiotic) has been given for 24 hours - supportive care - intravenous cefotaxime or ceftriaxone immediately - vancomycin & rifampicin If the patient has recently travelled to a country where antimicrobial resistance is prevalent - Intravenous corticosteroids should **not** be given in patients with signs of sepsis or rapidly evolving rash (with or without symptoms and signs of meningitis) - After confirmation of N. meningitidies - Continue intravenous ceftriaxone or cefotaxime - • Intravenous benzylpenicillin may be given as an alternative; follow your local protocol. - Stop intravenous dexamethasone if a cause other than *S pneumonia* is identified Ciprofloxacin or Rifampacin for contacts in meningococcus meningitis

Answer 61

Complications are **usually seen with bacterial meningitis**, due to all the cerebral oedema and pus. - Septicaemia, Shock - Disseminated Intravascular Coagulation - Cerebral oedema - Coma - Subdural effusions - Syndrome of inappropriate antidiuretic hormone secretion - Hydrocephalus, hearing loss, and seizures-sequelae related to fibrosis - Delayed: Hearing loss, cranial nerve dysfunction, hydrocephalus, intellectual deficits, ataxia, blindness - Death-herniation secondary to cerebral edema - Waterhouse-Friderichsen Syndrome (bilateral adrenal haemorrhage caused by severe meningococcal infection)

Answer 62

can be infectious or non-infectious - Most commonly due to **VIRAL INFECTION** - **Viral Causes** - **Herpes Simplex Virus** - MOST COMMON in the UK - HSV-1 responsible for 95% of cases in adults, HSV-2 possible - typically affects **temporal** and inferior frontal lobes - also possible: cytomegalovirus, epstein barr virus, varicella zoster virus, HIV and the mosquito virus a.k.a. arboviruses (including west nile virus) - Bacterial pathogens that cause meningitis may sometimes progress to cause a meningoencephalitis - Autoimmune encephalitis, including the NMDA-receptor-antibody associated encephalitis should also enter the differential diagnosis. - **Non-Viral (RARE)** - Syphilis - Staphylococcus aureus - **In immunocompromised patients** - CMV - Toxoplasmosis - Listeria - **Autoimmune or Paraneoplastic** - Associated with certain antibodies (e.g. anti-NMDA, anti-VGKC) hypoglycaemia, hepatic encephalopathy, DKA, drug-induced encephalopathy, uremic encephalopathy, SLE, hypoxic encephalopathy, and Beri-Beri. Each subtype may have suggestive clinical features and associations which may aid diagnosis.

Answer 63

age under 1 or over 65 years, immunodeficiency, viral infections, body fluid exposure, organ transplantation, animal or insect bites, viral cause depends on location of recent travel and season.

Answer 64

cardinal feature: **altered mental state** (not as prominent in meningitis) & behavioural change - Deteriorating GCS - Mini–Mental State Examination (MMSE) may reveal cognitive/psychiatric disturbance - History of seizures - Generalised tonic-clonic seizures and focal seizures (with or without secondary generalisation) are very frequently seen at some point in the clinical course. - Focal neurological symptoms (e.g. dysphagia, hemiplegia) - Subacute onset (hours to days) - Signs of **raised ICP**: - Cushing's Response: widening pulse pressure + bradycardia + irregular breathing - Papilloedema - maybe signs of meningism: Headache, Fever, Vomiting, Neck stiffness, Photophobia, Behavioural changes, Drowsiness, Confusion - rash - Obtain a detailed **TRAVEL HISTORY** - peripheral lesions (e.g. cold sores) have no relation to presence of HSV encephalitis

Answer 65

Vesicular eruption - enterovirus, herpes simplex virus (HSV), varicella zoster virus. Maculopapular eruption - Epstein-Barr virus (after treatment with ampicillin), measles, human herpesvirus-6, Colorado tick fever, West Nile virus. Malar rash - systemic lupus erythematosus. Petechial rash - rickettsial fever. Erythema migrans - Lyme disease. Erythema nodosum - tuberculosis and histoplasmosis, sarcoidosis. Erythema multiforme - HSV, *Mycoplasma*. Mucous membrane lesions - herpes virus, Behcet's. Pharyngitis - enterovirus, adenovirus. Conjunctivitis - St. Louis encephalitis virus, adenovirus, leptospirosis (conjunctival suffusion). Gumma - syphilis. Kaposi's sarcoma - HIV/AIDS. Non-healing skin lesions - *Balamuthia mandrillaris*, *Acanthamoeba*. Genital lesions - HSV-2, Behcet's.

Answer 66

- **Bloods** - culture - FBC - high lymphocytes (indicates viral cause) - U&Es - SIADH may occur as a result of encephalitis - LFT - Glucose - Viral serology - ABG - **MRI (/CT)- Highly recommended (preferably initially) in suspected encephalitis and is invaluable in diagnosis.** - Exclude mass lesion - HSV causes oedema of the **temporal lobe** on MRI - medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - but normal in one-third of patients - MRI is better than CT - A ring-enhancing lesion on CT/MRI: consideration of several possible diagnoses, including cerebral abscesses, tumours, metastasis and demyelination. Most cases require neurosurgical referral. If a **cerebral abscess is suspected, IV ceftriaxone** should also be administered. - **Lumbar Puncture (barring any contraindications)** - High lymphocytes - High monocytes - High protein - Glucose is usually normal - Viral PCR - **EEG** - may show epileptiform activity - lateralised periodic discharges at 2 Hz - **Brain biopsy** (rarely needed) - PCR - for HSC

Answer 67

empirical - broad spectrum antimicrobial cover with 10 mg/kg aciclovir TDS and 2g IV ceftriaxone BD for two weeks. - Aciclovir is given until HSV infection can be excluded (HSV PCR). - stop ceftriaxone once LP excludes bacterial infection - if immunosuppressed, also add Ganciclovir and foscarnet for potential cytomegalovirus (CMV) encephalitis Supportive management of complications including prompt termination of seizure activity with anticonvulsants (such as phenytoin) also important. **Corticosteroids are not encouraged but** if streptococcus pneumoniae is suspected give dexamethasone for 4 days or just before first antibiotic dose.

Answer 68

- Generalised fatigue/malaise (common) - Gastrointestinal disturbance (common) - Photosensitivity and urticarial rash (common) - Acute renal failure - Haematological abnormalities - Hepatitis - Neurological reactions

Answer 69

seizures, hydrocephalus, neurological sequelae (behavioural, motor disturbances)

Answer 70

Aetiological agents may be bacterial, fungal, and parasitic. - number of causes: extension of sepsis from middle ear or sinuses, trauma or surgery to the scalp, penetrating head injuries and embolic events from endocarditis - Can also be from extension of pathogens located in contiguous site - mastoiditis (pneumococcal), sinusitis, surgical site wound infection (staphylococci). More common in adult men <30yrs. In neonates, Proteus mirabilis and Citrobacter species are the most common isolates, while in children Streptococcus species in combination with cyanotic heart disease is most often responsible for brain abscess. In adults, brain abscess is most often due to sinusitis or otitis, and is most often caused by Streptococcus species.

Answer 71

The most frequent microbial pathogens isolated from brain abscesses are Staphylococcus and Streptococcus . Among this class of bacteria, Staphylococcus aureus and Viridian streptococci are the commonest..

Answer 72

Aspergillus fumigates, Candida albicans, Cryptococcus neoformans. parasitic: Toxoplasma gondii, Trypanosoma species, Echinococcus granulosus, Entamoeba histolytica, Naegleria fowleri, Balamuthia mandrillaris.

Answer 73

difficult to spot due to thin collections suspect in very unwell patient, with thin enhancing ubdural collection and brain oedema disproportionate to collection

Answer 74

Sinusitis, otitis media, recent dental procedure or infection, recent neurosurgery, meningitis, congenital heart disease, endocarditis, diverticular disease, hereditary haemorrhage telangiectasia, arteriovenous malformation, granulomatous disease, diabetes mellitus, HIV or immunocompromise, intravenous drug abuse, haemodialysis premature birth . M>F

Answer 75

- depend upon the site of the abscess (those in critical areas e.g. motor cortex) will present earlier. - mass effect in the brain and raised intracranial pressure is common: nausea, vomiting,papilloedema, seizures - headache - often dull, persistent - fever - may be absent and usually not the swinging pyrexia seen with abscesses at other sites - focal neurology - e.g. oculomotor nerve palsy or abducens nerve palsy secondary to raised intracranial pressure - may have meningitis like signs - Recent infection: sinusitiis, otitis media, dental procedure or meningitis

Answer 76

- Bloods: FBC (leukocytosis), Serum ESR (elevated), Serum CRP (elevated), serum PT & PTT (clotting profile ordered for anyone who may need urgent surgical treatment; normal) - Blood culture: may be positive - Serum toxoplasma titre (esp if immunocompromised) - MRI with contrast is the initial radiographic test ordered in a patient with suspected brain abscess, unless a head CT has already been obtained. - MRI generally offers more detail than CT, it is more time-consuming and expensive. - CT often performed as the first radiographic study in patients with new neurological findings. - Ultrasound head in infants

Answer 77

1. stabilise 2. blood culture before antibiotic 3. contact duty neurosurgical team early for advice 4. empirical treatment - IV antibiotics: Treatment tailored to cover most likely pathogens - - vancomycin AND metronidazole/clindamycin AND ceftrioxone if otogenic may use amoxicilin instead of ceftriaxon. - intracranial pressure management: e.g. dexamethasone. - prophylactically with anticonvulsants e.g. phenytoin or carbamazepine 5. after culture results, tailored regimen 6. surgery - Urgent surgical decompression of the abscess is required, the pus should be collected in a sterile container NOT ON SWABS and sent to microbiology - often do aspiration, rarely surgical excision. - a craniotomy is performed and the abscess cavity debrided - the abscess may reform because the head is closed following abscess drainage. - intracranial pressure management: e.g. dexamethasone duration If abscess has been drained then use therapy for 4-6 weeks, may need 6-8 weeks or longer if no drainage. Some patients with abscess may need a long term IV cannula and should have CT brain scans (3, 6, 12, 20 weeks after starting antibiotics).

Answer 78

Cryptococcus Neoformans - in CD4<200 - Invasive fungus present in soil and gigot droppings - meneingitis, lungs too - fever & headache over weeks, altered mental state, raised ICP, sometimes herniation - IX: CrAg -> if positive opening pressure (raised) (MRI (soap bubble lesions), sabaurad's agar, later agglutination test, India ink shows yeast w halos ) - Tx → IV amphoterocin B or Fluconazole Cytomegalovirus (CMV) - CMV retinitis - retinal edema and necrosis - floaters & decreased vision Toxoplasmosis gondii (cerebral toxoplasmosis) - protozoa - Primary infection: oocyte ingestion - Found in cat feces -causes encephalitis - causes space-occupying cysts in the brain - Usually asymptomatic, Organisms disseminate throughout body, Encyst in nucleated cells and enter dormant state , Most disease presentations: reactivation - CD4 <100cells/mm3 - Multiple “ring-enhancing” lesions on imaging - Sulfadiazine/pyrimethamine or sulphadiazine plus clindamycin are used. JC virus - slow onset encephalopathy - Progressive multifocal leukoencephalopathy (PML) - reactivation of JC -> demyelination - Dx: JC virus DNA in CSF or brain biopsy (similar symptoms to MS) - BK virus encephalopathy - HIV associated dementia above are opportunistic infections when CD4 <200 in patients w high CD4 counts, types are more limited and confined to TB meningitis, herpes simplex encephalitis, neurosyphilis

Answer 79

S aureas salmonella (in children) E coli Pseudomonas

Answer 80

inflammation that develops between the intervertebral discs of your spine. Spondylodiscitis is when the primary infection of the intervertebral disc (discitis) causes secondary infections of the vertebrae (spondylitis). It can lead to osteomyelitis of the spinal column. usually assocaited with a disseminated endocarditis infection. Staphylococci and streptococci species are involved. unremitting back pain fever weakness/ sensory Dx -> blood culture -> CT guided aspirate -> open surgical biopsy Tx -> Fluoroquinalones, clindamycin and rifampicin achieve excellent bone levels

Answer 81

Most postoperative infections occur between three days and three months after surgery. Vertebral osteomyelitis is the most common form of vertebral infection. It can develop from direct open spinal trauma, infections in surrounding areas and from bacteria that spreads to a vertebra from the blood.

Answer 82

Poliomyelitis → Damage to the anterior motor horn due to polio virus infection - caused by single stranded RNA virus - Prevention = vaccination - results in destruction of anterior horn → LMN lesions → flacid paralysis - gastrointestinal route → may manifest as the minor illness, which is usually gastrointestinal, or as the major illness, characterised by acute flaccid paralysis (AFP). Werdnig-Hoffman disease (spinal muscular atrophy) → Inherited degeneration of the anterior motor horn; autosomal recessive it is type 1 spinal muscular atrophy

Answer 83

Polio - unvaccinated child - residence or travel in endemic area - asymmetrical paralysis of the affected limb (usually the lower extremities) → decreased tone and motor function of affected limb - weakness (legs>arms) - flaccid muscle tone - decreased tendon reflexes of affected limb - febrile illness, sore-throat, diarrhoea, nausea & vomiting - neuro symptoms 4-5 days later. WH disease - same ^, - 'floppy baby" - diffuse muscle atrophy - Hypotonia/ weakness in newborns - classic finding: tongue fasciculations - die in a few months or years after birth

Answer 84

Prevention oral vaccines containing attenuated poliovirus in disease-endemic, outbreak-affected areas inactivated poliovirus vaccine in the rest of the world Investigations Virus culture from stool, CSF or pharynx Management No cure Early physiotherapy Notify local health authority Initial Gastrointestinal illness → oral rehydration or IV fluids. Monitor for acute flaccid paralysis (AFP). Paralytic poliomyelitis → supportive: physiotherapy, mobilisation, monitor for progression to respiratory paralysis. If respiratory involved (bulbar) → transfer to specialised care & intubation Post-poliomyelitis → occurs years later. Supportive care & physiotherapy.

Answer 85

- Demylination of the posterior (dorsal) coloumns & roots - TD is a late consequence of Neurosyphilis. Presentation -> patient with STD, has loss of proprioception & vibration. Will have difficulty walking, wide based gate -> may have Fleeting, recurrent shooting pains , loss of ankle/knee reflexes ,Argyll Robertson pupils (small, non-reactive to light, reactive to accommodation. specific to syphilis) , Strength = 5 / 5 on arms and legs Investigations -dark-field microscopy of swab from lesion -serum treponemal enzyme immunoassay (EIA) -serum Treponema pallidum particle agglutination or haemagglutination (TPPA or TPHA) Management IV benzylpenicillin & prednisolone

Answer 86

- primary → painless chancre (painless genital ulcer) - Secondary phase → rash (few weeks later) - tertiary phase → tabes dorsalis (years later)

Answer 87

cell-mediated autoimmune destruction of CNS myelin and oligodendrocytes causing demyelination in the central nervous system. clinically defined by two episodes of neurological dysfunction (brain, spinal cord, or optic nerves) that are separated in space and time. - Multi-focal demyelination (“plaque”) - Glial hyperplasia (“sclorsé”) - Axonal and neuronal degeneration in white and grey matter oligoclonal bands are Intrathecally produced -IgG

Answer 88

2.3 x more common in FEMALES Age of presentation: 20-40 yrs | mean=30 - white women - More commonly seen at higher/ Northern latitudes (5 times more common than in tropics) Associated with HLA-DR2 -environment: vit D/ Prenatal vitamin D levels, tobacco/ smoking, obesity, intestinal microbiome factors, Epstien Barr virus, autoimmune disease

Answer 89

- Multi-focal demyelination (“plaque”) - Glial hyperplasia (“sclorsé”) - Axonal and neuronal degeneration in white and grey matter oligoclonal bands are Intrathecally produced -IgG mostly affects cervicle white matter, with random, asymmetric lesions seen in the spinal cord periventricular plaques: oligodendrocyte loss, reactive glosis

Answer 90

- Relapsing-Remitting MS - COMMONEST form (80% of disease presentation) - Clinical acute attacks (e.g. last 1-2 months) of demyelination with complete recovery in between attacks - Secondary progressive disease - describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses - around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis - gait and bladder disorders are generally seen - Primary Progressive MS - Steady accumulation of disability with NO relapsing-remitting pattern - progressive deterioration from onset - more common in older people - Progressive Relapsing MS steadily worsening neurologic function from the beginning with occasional relapses. - Clinically Isolated Syndrome - Single clinical attack of demyelination - The attack in itself does NOT count as MS - 10-50% progress to develop MS - Marburg Variant - Severe fulminant variant of MS leading to advanced disability or death within weeks

Answer 91

Varies depending on the site of inflammation 75% of patients have significant lethargy. - esp after hot shower UMN signs Odd sensations. Lhermitte's syndrome: paraesthesiae in limbs on neck flexion/ electric shock-like sensations extending down the cervical spine radiating to the limbs Uhthoff's Sign - worsening of neurological symptoms as the body gets overheated from hot weather, exercise, saunas, hot tubs etc. Graying or blurring of vision in one eye ->optic atrophy Internuclear Ophthalmoplegia -> Impaired adduction in the eye ipsilateral to the affected medial longitudinal fasciculus, and nystagmus in the contralateral abducting eye Optic Neuritis - demyelination of optic nerve , Unilateral deterioration of visual acuity and colour perception cerebellar Limb ataxia (intention tremor, past-pointing, dysmetria), Dysdiadochokinesia, Ataxic wide-based gait, Scanning speech, tremor Vertigo and scanning speech mimicking alcohol intoxication (brainstem/ Subacute cerebellar ataxia) Autonomic Urinary urgency, spastic bladder, overflow incontinence , mpotence- sexual dysfunction emotional lability → pathological weeping , - Depression,Psychosis, cognitive impairment

Answer 92

Diagnosis is made by Clinical history/examination, MRI (gold standard) and lumbar puncture. Diagnosis made on the basis of two or more relapses: - either objective clinical finding of two or more CNS lesions with corresponding symptoms, separated in time and space - or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse. MRI Brain & spinal cord (with gadolinium)MRI with contrast should be used to view demyelinating lesions Gadolinium enhancement shows active lesions Lumbar Puncture increased lymphocytes, immunoglobulins with unmatched oligoclonal IgG bands on high resolution electrophoresis, and myelin basic protein. High/ mildly raised protein, Evoked Potentials in patients for whom MRI is contraindicated. Visual, auditory and somatosensory evoked potentials may show delayed conduction velocity FBC/ metabolic panel/ TSH/ vit b12 - to exclude other diseases. should be normal in MS

Answer 93

MS diagnosis made on the basis of two or more relapses: - either objective clinical finding of two or more CNS lesions with corresponding symptoms, separated in time and space - or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse.

Answer 94

Expanded disability status score gold standard in measuring MS disability 0- no disability 10 - death

Answer 95

- high-dose steroids every day, for 3 days, every day - methylprednisolone- usually IV - steroids reduce the duration & severity of the attacks - plasma exchange if refractory to steroids In practical terms, it is usually necessary to involve the local neurology team for guidance on when to start steroids for patients with an acute flare of multiple sclerosis. Always ensure to check routine bloods and urine dip to rule out any intercurrent infection.

Answer 96

by reducing triggers and use of disease-modifying therapies; disease modifying therapies (DMTs) - beta-interferon, glatiramer, dimethyl fumarate, diroximel fumarate, and teriflunomide are generally considered to be first-line agents. - New oral agents such as dimethyl fumarate, teriflunomide and fingolimod - Biologics such as natalizumab and alemtuzumab. - **Rate of relapse in multiple sclerosis in reduced most significantly by biological DMTs: natalizumab, and alemtuzumab.** - biologics reserved for more aggressive disease Natalizumab has the strongest evidence base!! -> antagonises alpha-4 beta-1-integrin complication of DMARD → increase risk of JC infection and subsequent PML

Answer 97

- Physiotherapy - Pain- SNRI / carbamezapine / valproate / gabapentin / lamotrogine / pregabalin (manage pain - normal painkillers don't work) - Fatigue → exclude other problems (anaemia, thyroid or depression), amantadine, mindfulness training and CBT - Baclofen, gabapentin, BoTox for spasticity - Other options include diazepam, dantrolene and tizanidine - Modafinil and exercise therapy for fatigue - Anticholinergics for bladder dysfunction - importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients - if significant residual volume → intermittent self-catheterisation - if no significant residual volume → anticholinergics may improve urinary frequency - SSRIs for depression - Oscillopsia (visual fields apper to oscillate)- gabapentin is first-line - Sildenafil (viagra) for erectile dysfunction - Clonazepam for tremor

Answer 98

Life expectancy is fairly close to normal, may be 5 years below. - Older - Male - Motor signs at onset - Early relapses - Many MRI lesions - Axonal loss

Answer 99

fatigue, dysesthesia, pain, depression, cognitive impairment, spasticity and muscle spasms, emotional lability (pathological weeping - involuntary crying not associated with sadness or emotion), bladder symptoms - can be embarrassing and may not discuss (increased frequency, urge incontinence, hesitancy and enuresis), sexual dysfunction (impotence), UTIs and decubitus ulcers. It has a large impact on life - cost, employment, relationships

Answer 100

What is it? acute inflammatory (immune-mediated) demyelination of the peripheral nervous system Pathophysiology inflammatory process where antibodies after a recent infection cross-react with gangliosides and self-antigen on myelin or neurons in the peripheral nervous system anti-ganglioside antibody (e.g. anti-GM1) triggering infection is classically Campylobacter jejuni (bloody diarrhoea) /mycoplasma/ EBV/ CMV (asymptomatic infection, detected by CMV antibodies, immunosuppressed patient, febrile illness)

Answer 101

Two-thirds of patients have a history of gastroenteritis or influenza-like illness weeks before onset of neurological symptoms. muscle weakness, respiratory distress, speech problems, ptosis - PROGRESSIVE symptoms, resolve in weeks to months - **General MOTOR Examination** - **progressive, symmetrical weakness of all the limbs.** - Lower motor neurone signs in the lower limbs (hypotonia, flaccid paralysis, areflexia). - proximal muscles (e.g. hips/shoulders) are usually affected before than the distal ones (e.g. feet/hands) - **General SENSORY Examination** - Impairment of sensation in multiple modalities (ascending from feet to head) - Paraesthesia may precede the onset of motor symptoms. - sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs - **Cranial Nerve Palsies** - Facial nerve weakness - diplopia- Abnormality of external ocular movements - oropharyngeal weakness is common - If pupil constriction is affected, consider botulism - **Type II Respiratory Failure** - Due to paralysis of respiratory muscles - CO2 flap, bounding pulse - **Autonomic Function** - Assess postural blood pressure change. Hypo or hypertension - arrhythmias - urinary retention - diarrhoea

Answer 102

- Investigations - **Bloods** - Anti-ganglioside antibodies GM1 - anti-GM1 (IgM) with multifocal motor neuropathy, GQ1b (IgG) with the Miller-Fisher syndrome and GM1 (IgG) with the Guillain-Barre syndrome. - ABG (to check for type 2 respiratory failure) - LFTs → hepatic aminotransferases may be elevated (elevated AST and ALT) - **Lumbar Puncture** - HIGH protein - NORMAL WBC cell count and glucose -(albuminocytologic dissociation) - found in 66% - **Nerve Conduction Study** - Reduced conduction velocity - NOTE: it may be normal in the early stages of the disease - **Spirometry** - Reduced forced vital capacity - suggests ventilatory weakness - at 6 hour intervals initially at the bedside - **ECG** - Arrhythmias may develop

Answer 103

Conservative measures include monitoring of ventilation (with serial spirometry and ABG) ± ventilation, measures to reduce the risk of VTE (TEDs, LMWH), and protection of pressure areas. Respiratory support Medical management is with intravenous immunoglobulin and, if ineffective, plasmapheresis. Hypotension: fluid boluses Hypertension: short acting agents (e.g., labetalol, esmolol, or nitroprusside)

Answer 104

- variant of Guillain-Barre syndrome - associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first - condition is typically positive for anti-GQ1b antibodies. - usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome

Answer 105

a differential for GBS This syndrome typically presents with asymmetric lower motor neuron weakness that often being in the upper limbs between the ages of 20 and 75 years. Cranial nerve signs are uncommon, as are areflexia and sensory changes. The presents of conduction block and anti-GM1 ganglioside antibodies make the diagnosis even more likely.

Answer 106

JC virus infection ofoligodendrocytes (white matter) 1. Immunosuppression (e.g., AIDS or leukemia) leads to reactivation of the latent virus. demyelination multiple white matter lesion Presents with rapidly progressive neurologic signs (visual loss, weakness, dementia) leading to death

Answer 107

Focal demyelination of the pons (anterior brain stem) 1. Due to rapid intravenous correction of hyponatremia 1. Occurs in severely malnourished patients (e.g., alcoholics and patients with liver disease) Classically presents as acute bilateral paralysis ("'locked in" syndrome)

Answer 108

degeneration of the cerebral cortex, with cortical atrophy and reduction in acetylcholine production Sporadic most cases are sporadic. It has a genetic risk factor: apoprotein E allele E4 - encodes a cholesterol transport protein Inherited 5% of cases mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes Risk factors: Down's syndrome- APP in chromosome 21, familial form: presenilin 1 & 2 gene mutations , increased age Early-onset AD is seen in Familial cases and Down syndrome - Pathalogical findings of Alzheimers - Brain lesions are marked by neurofibrillary tangles (Hyperphosphorylation of tau protein linked to AD), senile plaques (/cortical Beta amyloid plaques/ or neuritic plaques due to extracellular beta amyloid protein deposition) , neuronal loss and cerebral brain atrophy, with defects in acetylcholine synthesis at the cellular level.

Answer 109

brain damage due to several incidents of cerebrovascular disease (e.g. strokes/TIAs) Multifocal infarction and injury due to hypertension, atherosclerosis, or vasculitis - Types - Stroke-related VD – multi-infarct or single-infarct dementia - Subcortical VD – caused by small vessel disease - Mixed dementia – the presence of both VD and Alzheimer’s disease - Rarely, VD can be inherited as in the case of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy - RFs: stroke/ TIA RFs, increasing age,

Answer 110

deposition of abnormal proteins (Lewy bodies) within the brain stem and neocortex. - alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas. - Associated with Parkinson's disease - patients are also highly sensitive to neuroleptics, which causes a deterioration in parkinsonism - A neurodegenerative disorder with parkinsonism, progressive cognitive decline, prominent executive dysfunction, and visuospatial impairment.

Answer 111

specific degeneration of the frontal and temporal lobes - type of Frontotemporal lobar degeneration (includes Frontotemporal dementia, chronic progressive aphasia, Semantic dementia) - Atrophy of the frontal and temporal lobes. Pick bodies - spherical aggregations of tau protein (silver-staining). Characterized by round aggregates of tau protein (Pick bodies) in neurons of the cortex. Gliosis, Neurofibrillary tangles, Senile plaques.

Answer 112

insidious onset - Amnesia (recent memories lost first), Aphasia (word-finding problems, speech muddled and disjointed), Agnosia (recognition problems), Apraxia (inability to carry out skilled tasks despite normal motor function) 1.Slow-onset memory loss (begins with short-term memory loss and progresses to long-term memory loss) and progressive disorientation 2. Loss of learned motor skills and language 3. Changes in behavior and personality (loss of social and occupational functioning) 4. Patients become mute and bedridden; infection is a common cause of death. 5. Focal neurologic deficits are not seen in early disease.

Answer 113

- step-wise decline - Multi-infarct dementia (stroke) - The executive functions of the brain such as planning are more prominently affected than memory. Motor and mood changes are often seen early. - Focal neurological abnormalities e.g. visual disturbance, sensory or motor symptoms - The difficulty with attention and concentration, Seizures - Memory, Gait, Speech, Emotional disturbance

Answer 114

- progressive cognitive impairment - cognition may be fluctuating, in contrast to other forms of dementia - in contrast to Alzheimer's, early impairments in attention and executive function rather than just memory loss - fluctuating levels of consciousness - Parkinsonian symptoms - **visual hallucinations** (other features such as delusions and non-visual hallucinations may also be seen)- Lilipution hallucination - see small people running about

Answer 115

- behavioural changes and intellectual changes - Manifests primarily as disruption in personality and social conduct, or as a primary language disorder. - insidious onset, Relatively preserved memory and visuospatial skills - **Personality change** and social conduct problems - Behavioral and language symptoms arise early; eventually progresses to dementia

Answer 116

- Diagnosis is based on the HISTORY. brain biopsy is gold standard but not usually needed. - Assessment tools: 10-point cognitive screener (10-CS), 6-item cognitive impairment test (6CIT) - mini-mental state examination (MMSE) score of 24 or less out of 30 suggests dementia - NINDS-AIREN criteria for probable vascular dementia-> presence of contrite decline affecting activities of daily living, cerebrovascular disease, decline 3 months following stroke, abrupt deterioration, fluctuating, stepwise progression of cognitive deficit Blood screen FBC, U&E, LFT, calcium, glucose, TFTs, Vitamin B12, folate levels EEG -> normal in dementia. Abnormal in delirium, so to exclude delirium. Ensure no treatable cause is missed (e.g. hypothyroidism, vitamin B12/folate deficiency, space-occupying lesion, normal pressure hydrocephalus) Neuroimaging: MRI, to exclude other reversible conditions (e.g. Subdural haematoma, normal pressure hydrocephalus), DATscan & SPECT for levy bosy dementia

Answer 117

Non-pharmacological - supportive treatment, environmental control measures (reduce falls risk) - tailored activities to promote wellbeing - group cognitive stimulation therapy for patients with mild and moderate dementia - consider include group reminiscence therapy and cognitive rehabilitation - Mild to moderate AD. First line: three acetylcholinesterase inhibitors (**donepezil**, galantamine and rivastigmine) - Second line treatment? Indications? - add or switch to memantine (an NMDA receptor antagonist) - moderate Alzheimer's who are intolerant of, or have a contraindication to, acetylcholinesterase inhibitors - as an add-on drug to acetylcholinesterase inhibitors for patients with moderate or severe Alzheimer's - monotherapy in severe Alzheimer's Management of non-cognitive symptoms - Don't use antidepressants for mild to moderate depression. If clinically significant → SSRIs. If poor appetite & insomnia → mertazapine. - antipsychotics should only be used for patients at risk of harming themselves or others, or when the agitation, hallucinations or delusions are causing them severe distress

Answer 118

- Treatment is mainly symptomatic with the aim to address individual problems and provide support to the patient and carers - Important to detect and address cardiovascular risk factors – for slowing down the progression If atherosclerotic ischemic disease 1) Antiplatelet & lifestyle modification (aspirin, clopidogeral, dipyridamole) 2) Carotid endarterectomy is the preferred mode of treatment for symptomatic carotid stenosis (grade >70%) Cardioembolic disease 1) Anticoagulation or anti platelet therapy (warfarin or DOAC or aspirin etc) & lifestyle modification. Non-pharmacological management - Tailored to the individual - Include: cognitive stimulation programmes, multisensory stimulation, music and art therapy, animal-assisted therapy - Managing challenging behaviours e.g. address pain, avoid overcrowding, clear communication Pharmacological management - There is no specific pharmacological treatment approved for cognitive symptoms - Only consider AChE inhibitors or memantine for people if they have suspected comorbid Alzheimer’s disease, Parkinson’s disease dementia or dementia with Lewy bodies. - no evidence that aspirin or statins is effective in treating patients with a diagnosis of vascular dementia.

Answer 119

- acute behaviour disturbances → benzodiazepines - both acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine can be used. - Typical Antipsychotics (neuroleptics) should be **avoided** in Lewy body dementia as patients are extremely sensitive and may develop irreversible parkinsonism. If needed atypical should be used but contact consultant specialist. - atypical antipsychotics (risperidone. olanzapine, quitapine, pimavanserin) can be used if needed for psychotic symptoms - **Questions may give a history of a patient who has deteriorated following the introduction of an antipsychotic agent** - depression & anxiety - SSRI or benzodiazepines - REM sleep behavioural disorder - clonazepam / melatonin - motor symptoms - carbidopa/ levadopa

Answer 120

- Supportive care. - Is acute irritability or aggressive: Benzodiazepine or antipsychotic - compulsions affecting life→ SSRI. - distractibility. perseverance → amantadine - euphoria/ agitation/ persistant aggression → valproate semisodium or topimate - abnormal eating behaviour → tomiporate NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia Palliative care at the end.

Neurology Flashcards

(144 cards)