Neuro

Question 1

Cluster headache acute attack (without CVD or uncontrolled htn)

Answer 1

1. FL: sub. Cut. Sumatriptan 6mg single dose. Repeat after at least 1 hour if needed. Max 12mg/d. (CI in CAD, PVD or cerebrovascular Dx). Also give high-flow oxygen >12L/min for >15mins or until attack terminated (not CI in vascular Dx)
   
   2. 2L: zolmitriptan nasal 5mg as single dose can repeat >2hrs after initial dose if nec. Max 10mg/day. . (CI in CAD, PVD or cerebrovascular Dx)
   3. 3L: intranasal sumatriptan (5-20mg, repeat at 2hr if nec. Max 40mg/day) or PO zolmitriptan 2.5-5mg single dose, repeat at 2hrs if nec. Max 10mg/day)
      3L: lidocaine 1mL of 10% solution placed with cotton swab intranasally for 5mins

Question 2

translational therapy in acute cluster headache in ALL patients

Answer 2

For all acute attacks in this group as translational therapy: pred 60mg OD PO 5d then reduce by 10mg every 3 days.

Question 3

adjunct to acute cluster attack in ALL patients

Answer 3

greater occipital nerve block: effective in 2/3. Should be considered before any surgical procedure when medical treatment has failed. Mixture of corticosteroid and LA or LA alone is injected into GON (situated 2/3 between mastoid and external occipital protuberance, leave 3 months between blocks)

Question 4

Acute attack cluster headache with CVD/uncontrolled HTN

Answer 4

1. FL: Oxygen >12L/min for at least 15mins or until attack terminted
   
   2. 2L: intranasal lidocaine: 1mL of 10% lidocaine placed with cotton swab

Question 5

Episodic/ongoing cluster headache

Answer 5

1. FL: verapamil 80mg PO immediate release TDS initally. Titrate up to max of 480mg/day.
   a. Start therapy as soon as possible at the start of an episode. Perform ECG to rule out conduction delay and don’t use if patient has heart block or arrythmia.
   
   2. 2L: Lithium consult spsecialist for guidance (max conc. = 1.2mEq/L)
   3. 2L: topiramate: 25mg OD 7d increase dose to 100-200mg/day in 2 doses over period of weeks
   4. Gabapentin: consult specialist on dose
   5. Melatonin: consult specialist on dose
      Surgery is a 4th line treatment only in medically refractory headaches. (occiptal nerve stimulation, or deep brain stimulation of posterior hypothalamic region)

Question 6

ALL ongoing/episodic cluster headache tapering

Answer 6

FOR ALL: If episodic taper off therapy after 2 wks no symptoms. If chronic continue indefinitely (trialling perioidic dose reduction if Sx free)

Question 7

Complications of cluster headache:

Answer 7

A/w psychiatric conditions (depression, anxiety, aggressive behaviour)
Autonomic dysregulation (brady/tachycardia, hypertension, arrythmias (AVN block and SA block) Blood pressure regulation abnormality increases end organ disease and CV disease.

Question 8

Prognosis of cluster headache

Answer 8

Hard to predict - progression from episodic to chronic is possible ane vice versa.
Tends to remit with age, with longer periods of remission.
There is no underlying sinister disease so these patients are not at higher mortality resulting from the condition alone
May have large impact on life if severe and therefore mental health and quality of life.

Question 9

encephalitis management initial (immunocompetent)

Answer 9

All cases of community acq. Viral encephalitis is treated with 10mg/kg IV every 8hrs for 10-21 days. Assumed to be HSV until proven otherwise.

Question 10

encephalitis Mx inital (immunosupressed)

Answer 10

Ganciclovir 5mg/kg IV every 12hrs for 14-21 days AND foscarnet 60mg/kg IV every 8hrs for 14-21 days. AND aciclovir 10 mg/kg every 8hrs for 21d. In immunocompromised CMV is treated alongside the HSV cover.

Question 11

Supportive care in encephalitis

Answer 11

IF cx (electrolyte abn., stroke, raised ICP, cerebral oedema, coma, seizure) manage in ICU
May require tubing, circulatory and electrolyte support.
Prevention of secondary Cx eg DVT, bacterial infection, gastric ulcer
Antiretroviral therapy if HIV
Raised ICP: corticosteroid and mannitol, bed at 30-45 deg. Hyperventilation at PaCO2 of 30
Shunting or decompression indicated if medical Mx fails to reduce ICP

Question 12

Confirmed viral cause of encephalitis HSV

Answer 12

aciclovir 10mg/kg IV every 8 hours for 14-21d (immunosuppressed full 21d)

Question 13

confirmed VZV encephalitis

Answer 13

aciclovir 10mg/kg IV every 8 hours 14d OR ganciclovir 5mg/kg every 12h 14-21d
Adjunctive methylprednisolone 1000mg IV OD 3-5d for possible cerebral vasculitis

Question 14

Confirmed CMV encephalitis

Answer 14

ganciclovir 5mg/kg IV every 12h 14-21d FU w/5mg/kg/day for 7d AND foscarnet 60mg/kg IV every 8hrs 14-21d

Question 15

Confirmed EBV encephalitis

Answer 15

aciclovir 10mg/kg IV every 8hrs 14d. PLUS 1000mg methylprednisolone IV 3-5d

Question 16

confirmed HBV encephalitis

Answer 16

ganciclovir 5mg/kg every 12h 14-21d OR aciclovir 10mg/kg every 8hrs 14-21d

Question 17

confirmed HH6 encephalitis

Answer 17

ganciclovir 5mg/kg every 12h 14-21d. OR foscarnet 60mg/kg IV every 8hrs

Question 18

NON viral encephalitis Mx

Answer 18

Treat underlying cause: examine and culture CSF e.g. appropriate Abx, Afx, Avx
Auto-Immune: methylprednisolone 1000mg IV 3-5d
ADEM: methylprednisolone 1000mg IV 3-5d
Paraneoplastic: normal human IG 2g/kg IV given over 4-5 days
Syphilis: Benzylpenicillin 1.2-2.4g IV every 4hrs 10d
Listeria: ampicillin 1-2g IV every 4hrs 21d AND gentamicin 2mg/kg loading dose then 1.7mg/kg every 8hrs
Mycoplasma pneumonia: doxycycline 100mg IV every 12hrs 5-10d

Question 19

ALL aetiologies of encephalitis aftercare

Answer 19

• Depends on functional deficits can include cognitive and behavioural rehab and motor rehab.

Question 20

Complications of encephalitis

Answer 20

electrolyte abn - correct
Stroke - neuroimaging with appropriate Mx
Raised ICP - corticosteroid and mannitol, bed at 30-45 deg. Hyperventilation at PaCO2 of 30. Shunting or decompression indicated if medical Mx fails to reduce ICP
Cerebral oedema,
Coma
Seizure: manage in ICU
May require tubing, circulatory and electrolyte support.
Prevention of secondary Cx eg DVT, bacterial infection, gastric ulcer

Question 21

Prognosis of encephalitis

Answer 21

Aetiological agent found in 50%
30% mortaility in HSV with treatment 70-80% without
If mild most can expect full recovery
Viral usually recover without sequele
May have residual difficulties in concentration, behaviour, speech, memory loss
Rarely can become in vegetative state
Depends on patient underlying health eg age, HIV status

Question 22

Extradural haemorrhage Management

Answer 22

> 30cm3 should be managed surgically regardless of other factors
<30cm3 with low thickness, minimal midline shift and GCS >8 without any FNS are candidates for non surgical management.

Question 23

non-surgical Mx of extradural haemorrhage

Answer 23

Non surgical mx: serial CT scans and close neuro obs

Question 24

Surgical Management of extradural haemorrhage

Answer 24

NO specific procedure but craniotomy and burr holes are able to relieve raised intracranial pressure. Any bleeding sources can be identified and ligated/cauterised.

Question 25

post-op extradural haemorrhage

Answer 25

neuro-critical care or HDU with close neuro-obs and routine post op CTs. Ongoing neurorehab often required.

Question 26

Complications of extradural haemorrhage

Answer 26

Parenchymal compression/cerebral herniation: reducing ICP and controlling bleed with neurosurgery Residual deficit - paralysis or loss of sensation Coma Normal pressure hydrocephalus leading to weakness, headache, incontinence, ataxia

Question 27

Prognosis of extradural haemorrhage

Answer 27

Carries 30% mortality Poor prognostic factors: age, herniation, raised ICP Potential of residual symptoms

Question 28

Guillain-Barre Management (without IgA def. or renal failure)

Answer 28

1L: plasma exchange and IVIG are equal in effectiveness. Ambulatory patients: plasma exchange within 2 weeks of onset of neurology Non-ambulatory: plasma exchange within 4 weeks of onset Plasma exchange dose (through central line): 50mL/kg every other day for 7-14d. Monitor closely for coagulopathy and electrolyte abnormality IVIG: 400mg/kg/day IV for 5 days

Question 29

Guillain-Barre Mx (with IgA def. or renal failure)

Answer 29

Must use plasma exchange due to risk of anaphylaxis Plasma exchange dose (through central line): 50mL/kg every other day for 7-14d. Monitor closely for coagulopathy and electrolyte abnormality IVIG: 400mg/kg/day IV for 5 days

Question 30

Severe Guillain-Barre supportive Mx

Answer 30

``` HR and BP until they are off ventilator support. With fluid bolus if needed. Consider intraarterial monitoring if labile. Hypertn. Use short acting agents (labetalol, nitroprusside) DVT proph (sc LMWH+TEDS). Pain: gabapentin or carbamazepine acute. TCAs, tramadol, gabapentin may be helpful long term ```

Question 31

rehab in Guillain-barre

Answer 31

acute: gentle strengthening, focus on limb posture | Nutrition

Question 32

Complications in guillain-barre

Answer 32

Respiratory failure: ventilation +/- intubation Residual symptoms: weakness, parasthesia, pain (neuropathic pain agents) DVT: LMWH and TEDS HR and BP instability (autonomic dysfunction) close obs and use of short acting agents and boluses Of plasma exchange: Complications include severe infection, blood pressure instability, cardiac arrhythmias, and pulmonary embolus.Other adverse effects include hypocalcaemia. Of IVIG: Risk of pathogen (HIV, hep B+C, CJD) but is low IVIG can promote anaphylaxis in IgA deficiency

Question 33

Prognosis of Guillain barre

Answer 33

Worst case: tetraplegia after 24 with inability to walk at 18m. Usual: peak weakness 10-14d with recovery in weeks-months 80% independent walking at 6months and 60% full motor recovery at 1yr 5-10% have prolonged recovery (several months on ventilation and possible incomplete recoery) Up to 12% mortality despite ICU 15-20% with residual deficits and up to 10% severely diabled.

Question 34

Huntingtons Disease Mx

Answer 34

All patients to receive counselling (behavioural and genetic) Occupational therapy, physiotherapy and SALT provide support With specific symptomatic support - citalopram 20mg 2wks then 40mg (mood) - olanzapine 5mg 1wk then 10mg (behavioural, chorea, bradykinesia or ACUTE mx) - carbidopa 25mg TDS then up to 200 (bradykinesia w/o prominent behaviour)

Question 35

Complications of huntingtons

Answer 35

All those listed above Aspiration pneumonia from unsafe swallow Heart disease/hypertension from stress (physical and emotional) Suicide

Question 36

Prognosis of huntingtons

Answer 36

No disease modifying treatment 10-30yrs from disease emergence to death Shorter life expectancy and more rapid progression with subsequent generations Most commonly die from pneumonia or fall related injury

Question 37

Hydrocephalus Mx acute

Answer 37

Insertion of VP shunt to act as a temporary measure to reduce ICP. Meds (furosemide and acetazolomide inhibit CSF secretion in choroid plexus so can have role in Mx)

Question 38

hydrocephalus Mx definitive

Answer 38

Depends on underlying cause: debulking potentially resectable tumour, endoscopic third ventriculostomy (bypass from 3rd to 4th V) or choroid plexus resection Shunt insertion is most common option: VP or VA shunts can reduce ICP long term

Question 39

Complications of hydrocephalus

Answer 39

Raised ICP causing herniation or brain stem compression -> respiratory depression. Opthalmoplegia (down and out) and visual changes due to chiasm compression Incontinence Gait change Infected VP shunt - surgical removal Children will need a new shunt every 2yrs roughly. Comes with risks All managed definintely with VP shunt/ventriculostomy to reduce ICP. Emergency NS procedures (craniotomy) an option if life threatening.

Question 40

Prognosis of hydrocephalus

Answer 40

Depends on cause: acqueductal stenosis can be treated with surgery and most of the time things can be Mx with VP shunt Some symptoms in NPH (dementia) may not resolve In newborns 90% will survive with treatment. Usually have a normal life expectancy if treated early

Question 41

idiopathic intracranial htn define

Answer 41

Raised ICP w/o detectable cause.

Question 42

idiopathic intracranial htn aetiology/RF

Answer 42

RF: overweight, woman, tetracycline use Thought to be result of increased production/decreased resorption of CSF Unknown cause hence idiopathic

Question 43

IIH epi

Answer 43

Typically women 20-44 (90% post pubertal are women) | Incidence 20:100,000 in women 20% above IBW

Question 44

IIH symptoms

Answer 44

``` Headache often daily (worse in morning and valsalva. a/w N+V and photophobia) Transient vision loss (almost any type) Diplopia (abducens palsy) Pulsatile tinnitus Photopsia ```

Question 45

IIH signs

Answer 45

Papilloedema | Abducens palsy esotropia/horizontal diplopia

Question 46

IIH Ix

Answer 46

Neuroimaging: MRI with venography (MRV) best to rule out other causes of raised ICP LP: opening pressure >25cmH2O. CSF analysis (cell count, glucose, protein, gram stain, culture) Opthalmology: check papilloedema, visual acquity, perimetry testing FBC: rule out anaemia or lymphoproliferative causes of papilloedema

Question 47

IIH Mx

Answer 47

Diagnostic LP can transiently relive or resolve completely Weight loss Carbonic anhydrase inhib (acetazolomide) decreases CSF production and is diuretic Diuretics: furosemide (less effective than CA inhib) Steroids: can acutely lower ICP (caution when stopping as rebound wt gain and raised ICP - usually saved for severe vision loss or refractory cases) Possibility ot using VP/LP shunt (good for headache not as good at vision loss)

Question 48

IIH Cx

Answer 48

``` Permanent vision loss (option nerve compression) Otherwise mainly drug related: CA inhibs: hypokalaemia Steroids: wt gain, rebound ICP raise Diuretics: hypokalaemia, hypomangesnia LP: infection, damage, post LP headache ```

Question 49

IIH prognosis

Answer 49

Can go on for months/years even with Rx Rapid onset requires more aggressive Mx More vision loss at presntation suggestive of higher risk of permanence Higher grade papilloedema suggests greater risk of permanent vision loss

Question 50

MND management all patients

Answer 50

Riluzole 50mg BD (prolongs survival) - LFTs and FBC monitoring Physiotherapy for muscle weakness

Question 51

MND specific symptoms

Answer 51

Respiratory: NIPPV or chronic invasive ventilation. ALSO palliative care consideration Excessive mucous secretion: carbocysteine Dysphagia and weight loss: PEG tube Drooling: hyoscyamine Spasticity: baclofen 5mg TDS max 80mg/day Depression: SSRI (sertraline 20mg 2wks then 40)

Question 52

MND complications

Answer 52

Respiratory: NIV or intubation | Dysphagia and dysphasia: SALT, PEG tube to stop aspiration pneumonia

Question 53

MND prognosis

Answer 53

Life expectancy is 3yrs from start of Sx. May live up to 10yrs Severe and progressive, terminal

Question 54

MS management acute

Answer 54

1. Methylprednisolone 1000mg IV OD for 3d (up to 5d if severe) ADJUNCT: plasma exhange if severe or rapidly progression

Question 55

MS Mx RR

Answer 55

Immunomodulator: IFN b 1a (30mcg IV once weekly) OR glatiramer 20mg SC OD OR dimethyl fumarate (120-240md PO BD) More severe disease and/or not responded: fingolimod, natalizumab)

Question 56

MS Mx 2ndry Progressive

Answer 56

First line: Siponimod: reduces disability vs placebo Secondary option: methylprednisolone (pulse dose - managed by specialist) Second line: Cladribine - specialist management

Question 57

MS Mx primary progressive

Answer 57

First line: Ocrelizumab: 300mg IV single dose, then 300mg dose 2 weeks later, then 600mg every 6months`

Question 58

MS Cx

Answer 58

Fatigue: regular exercise, sleep hygiene. ?modafinil 100-200mg OD Urinary frequency: avoid caffiene. ?oxybutynin 5mg BD/TDS Pain/dysaesthesia: gabapentin/pregabalin Increased muscle tone: gentle stretching. ?baclofen 5mg TDS up to 80mg Tremor: propranolol 5mg BD up to 20mg Gait: physiotherapy Pneumonia UTI`

Question 59

MS prognosis

Answer 59

``` Rarely by itself fatal but because of Cx/disease burden life expectancy is 5-10 years lower No cure Reduced QOL - disability (walking) Depends if RR or progressive Each relapse reduces likely future QOL ```

Question 60

Myasthenia gravis Mx acute (severe)

Answer 60

1. 1L: intubation and mechanical ventilation (indication is FVC <15mL/kg) PLUS plasma exchange or IVIG PLUS supportive care (DVT, ?NG tube, ulcers) ADJUNCT: Pred PO OD 1-1.5mg/kg 2L: eculizumab or rituximab

Question 61

MG ongoing Mild (class I+II)

Answer 61

``` 1. 1L: pyridostigmine 30-60mg BD titrate up by 30-60mg/day usual dose 60-120mg every 3-4hrs max of 540mg/day ADJUNCT: Prednisolone 15-20mg OD continue until improved or 2-3months have passed then taper down CONSIDER: thymectomy (if class II w/anti-AChR) ```

Question 62

MG ongoing Mx moderate (class III)

Answer 62

``` 1. 1L: pyridostigmine 30-60mg BD titrate up to max of 540mg/day. AND/OR Prednisolone 15-20mg OD continue until improved or 2-3months OTHER OPTIONS: - Aziothioprine - Mycophenolate - Tacrolimus ``` 2. 2L: eculizumab OR rituximab ADJUNCT: thymectomy if anti-AChR+ve

Question 63

MG ongoing Mx severe (IV+V) post crisis NO intolerance or CI to immunosuppressants

Answer 63

1. 1L: pyridostigmine 30-60mg BD up to max of 540mg/day AND prednisolone 15-20mg up to 60mg OD until improvement or 2-3months Other options (Can be added or used as solo therapy): - Azothioprine - Mycophenolate - Tacrolimus Can also use: intermittent IVIG (every 4-6wks), thymectomy, plasma exchange

Question 64

severe disease MG post crisis with CI or intolerance to IS

Answer 64

1. 1L: Intermittent IVIG (every 4-6weeks) | Adjunct: thymectomy, plasma exchange

Question 65

MG Cx

Answer 65

Respiratory failure from muscle weakness (myasthenic crisis): intubation +/- ventilation Long term steroid use complications: fat pad, striae, immunosupression, osteoporosis, hyperglycaemia, peptic ulcer Dx Risk of immunosupression: TB, systemic fungal infectionl, PCP pneumonia

Question 66

MG prognosis

Answer 66

Depends on severity some may only have mild Sx With treatment most can live normal or nearly normal lives Life expectancy normal unless rare cases Death from crisis still low (<5%)

Question 67

NFM1 Mx acute

Answer 67

- Phaeochromocytoma: Immediate Surgical removal. ALL NF1 adults with htn or episodic headache need screening using 24hr urinary catecholamines. - Malignant peripheral nerve sheath tumour: Can occur in up to 10%. Surgical management is approach, however even with good margins recurrence and mets often result in death. Consideration for chemo +/- radio should be managed by specialist.

Question 68

NFM1 ongoing Mx of neurofibromas

Answer 68

- Neurofibromas (non cutaneous): 1L: surveillance (unless painful, or causing symptoms eg nodular plexiform causing spinal cord compression or vetebral degeneration then surgical removal) - Neurofibromas (cutaenous): 1L: Surveillance unless painful, excessively itchy(eg nail bed vascular tumour). Then can be surgically removed.

Question 69

NFM1 ongoing Mx of not neurofibromas

Answer 69

- Headache: Surveillance and investigation of cause. (concern regarding hydrocephalus, intracranial glioma, or other CVA, and phaeochromoctyoma) - Renovascular hypertension: Rule out phaeo. Then anti-hypertensives - Glaucoma - Optic pathway/intracranial glioma: Cranial MRI. If found then surgery and/or chemotherapy if endocrine or hypothalamic disorders. Surgery may be last resort depending on location - Impaired cognitive function: Special education resources and learning aids ``` - Peripheral neuropathy: Pain management (morphine may be necessary) ``` - Skeletal malformations: Possibilty of referral for surgery, specialised orthotics Genetic counselling on autosomal dominant heritability.

Question 70

NFM2 Cx

Answer 70

Vestibular schwannoma Glaucoma Visual impairment Parasthesia in limbs

Question 71

complications in NFM

Answer 71

Seizure Phaeo Glaucoma Malignant nerve tumours

Question 72

prognosis of NFM

Answer 72

Type 1: Lifelong condition, without Cx life expectancy is nearly normal. Often mild mental impairment Autosomal dominant Type 2: Lifelong autosomal dominant Almost all with develop vestibular shwannoma Seveirty depends on location on tumours, progression to maligancy and development of complications.

Question 73

Parkinsons Mx mild

Answer 73

- Very mild very early: MAO-B eg. Rasagiline 1mg OD (doesn’t conder long term benefit), selegiline (only for adjunctive) - First line: Dopamine agonist eg pramipexole, ropinirole, OR carbidopa/levodopa IMMEDIATE RELEASE (50mg TDS initially titrate to response) Due to risks of dyskensia in young pts with carbi/levo and orthostasis/hallucinations in old with dopamine agonists <70 usually given DA's, >70 given carbi/levo Second line: anticholinergics (amantadine, trihexyphenidyl) are effective at early Sx treatment esp. tremor but side effects limit their use esp. in elderly. q

Question 74

Moderate parkinsons Mx

Answer 74

- Very mild very early: MAO-B eg. Rasagiline 1mg OD (doesn’t conder long term benefit), selegiline (only for adjunctive) - First line: Dopamine agonist eg pramipexole, ropinirole, OR carbidopa/levodopa IMMEDIATE RELEASE (50mg TDS initially titrate to response) Due to risks of dyskensia in young pts with carbi/levo and orthostasis/hallucinations in old with dopamine agonists <70 usually given DA's, >70 given carbi/levo Second line: anticholinergics (amantadine, trihexyphenidyl) are effective at early Sx treatment esp. tremor but side effects limit their use esp. in elderly.

Question 75

Advanced parkinsons Mx

Answer 75

- Rasagiline 1mg OD (doesn't confer long term benefit - Most commonly: EXTENDED RELEASE carbi/levo (initially 50mg titrate to response) AND/OR pramipexole, ropinirole, rotigotine Second line: anticholinergics (amantadine, trihexyphenidyl) are effective at early Sx treatment esp. tremor but side effects limit their use esp. in elderly. Use if tremor is refractory

Question 76

extra management in parkinsons

Answer 76

PLUS: physical activtity Refractory tremor: consider anti-Ach, then propranolol, then DBS Unpredictable off times/motor fluctuation: apomorphine OR as needed dose of C/L. If still refractory intrajejunal infusion brings more consistent plasma concs. Dyskinesia: reduce dopaminergic meds and add amantadine if tolerated. Consider DBS Dysphagia: dissolvable solutions of medications N+V from carbi/levo: add more carbi eg. 25mg per dose N+V from DA: add domperidone

Question 77

Complications of parkinsons

Answer 77

Those listed above (tremor, N+V, dysphagia, worsening physical condition) Hallucinations on dopamine agonist esp. if old Anticholinergic side effects Cognitive decline and dementia (cholinesterase inhibitors eg rivastigmine) as well as low mood Constipation (laxative)

Question 78

Prognosis of parkinsons

Answer 78

Not a direct cause of death but significantly increases morbidity Progressive and chronic, not curable Most now have normal or near normal LE Risk of vulnerability