Neuro Flashcards

Question

Define Parkinson's disease

Answer 1

A neurodegenerative disorder with the cardinal features of bradykinesia, bradycardia, resting tremor, rigidity and postural instability.

Answer 2

``` Strong = Inc age, head trauma, MPTP exposure Weak = heavy metal/toxin exposure, occupational (builders/teachers/carpenters/farmers, geography (rural) ```

Answer 3

Bradykinesia, Bradycardia, resting tremor, rigidity, postural instability, micrographia, dec vocal loudness, hypokinetic dysarithria, stooped posture, dec facial movements, depression, dementia, constipation, autonomic dysf, fatigue, Slow gazing

Answer 4

Dopaminergic agent trial - some symptom resolution MRI - may be normal in idiopathic, age related changes, "swallow tail sign" - hyperintensity of Substania Nigra Functional neuro imagery - decreased uptake of Post synaptic dopamine in basal ganglia

Answer 5

Pathology that results from compression of the arterial, venous and cerebrospinal fluid spaces, AS WELL AS the SC itself.

Answer 6

``` 16-30 - TRAUMA Male Tumour/osteoporosis in older populations Intervertebral disc disease in middle aged Occupational labour Weak = immunosuppression/IVDU ```

Answer 7

Acute presentation if herniation/trauma. Insidious if malignant/disc disease General = back pain Sensory changes, muscular wasting, hyperreflexia below level, hypotonia below level, weakness, paralysis Bowel/bladder dysfunction, hypotension/bradycardia Central cord syndrome/anterior cord syndrome/Cauda equina syndrome/brown sequards

Answer 8

Spinal MRI - loss of disc space height, presence of herniation/malignancy Plain X-ray/CT - loss of disc space height, misalignment, deformities Galodinium MRI - areas of increased infection/malignancy PETCT - areas of hypermetabolism indicative of malignancy FBC/ESR/CRP - WCC Neutrophilia inc ESRCRP Bloods/CSF if suspect infection Tumour biopsy

Answer 9

Bleeding into the subarachnoid space, brain perenchyma and ventricles that causes a severe thunderclap headache with associated N+V, photophobia, focal neuro signs and meningismus. +/- intraocular haemorrhages

Answer 10

80% from ruptured cerebral saccular aneurysms | 20% from arterial dissections, a/c's and AV malformations

Answer 11

Old age HTN AV malformations FHx Smoking | Cocaine alcohol and marfans = weak

Answer 12

50% PRESENT AS UNCONSCIOUS Severe onset thunderclap headaches, <40% have preceding headache 3 months prior, Focal neuro signs, IO haemorrhages, N+V, photophobia, mengismus 6th nerve palsy

Answer 13

CT CT CT CT CT - visualisation of hyperdense sulci, fissures or basal cisterns FBC + E - may have leucocytosis and hyponatraemia INR - check whether prolonged / A/c's Troponin 1 - elevated but not as markedly as in ACSs ECG - some present with arrhythmias/prolonged QT Cerebral angiogram - visualisation of aneurysms LP may show bloody CSF if ventricular leakage

Answer 14

Collection of blood between the dura and arachnoid membrane resulting in an increase in ICP. 50-60% of cranial haematomas

Answer 15

Elderly, a/cs, coagulopathy, TRAUMA

Answer 16

Signs of trauma Raised ICP - headache, N+V, papilloedema, dec eye responses, weakness, sensory changes, eventual seizures. IF BASILAR - otorrhoea and rhinorrhoea

Answer 17

Non contrast ct/mri - blood collection beneath dura mater | INR check

Answer 18

<10mm = observe and follow up ICP correcting therapy - raise head, hypertonic saline Anti epileptics - eg phenobarbital or phenytoin All pts = adequate pain relief and correction of coagulopathy with FFP/cryoprecipitate/platelets/vit K >10mm = SURGERY - hemicraniotomy/craniotomy/durotomy

Answer 19

Generalised headache attacks that may be acute or chronic with a predication for occipital and frontal regions. NORMAL neuro exam

Answer 20

Stress, missed meals, female, 20-39, prolonged analgesic use, fatigue

Answer 21

Non throbbing, generalised, frontal/occipital, analgesic use, non pulsatile, photophobia/phono, potential muscle pain, "tight" pain NO N+V

Answer 22

Aspirin 300-900mg, paracetamol 500-1000mg Step up to naproxen CHRONIC = treat using amitryptaline 10mg orally OD or mertazapine 15mg orally OD

Answer 23

Rare but serious inflammation of the meninges caused predominantly by bacteria. (Can have viral/fungal)

Answer 24

H.influenza = 45% S.pneumoniae = 18% N.meningitidis 14%

Answer 25

Extremities of age, crowding, contact with many people, immunosuppression, male, anatomical defects, cochlear implant, dec socio econ

Answer 26

Classic symptoms - headache, fever, neck stiffness, rash, confusion, ICP related - N+V, papilloedema, seizures, focal neuro Babies - high pitched crying, restlessness, hypothermia, bulging fontanelle KERNIGS sign = inability to straighten leg whilst supine against pressure BRUDINSKIS sign = flexing neck causes knee/hip flexion OR flexing knee causes contralateral knee flexion

Answer 27

CSF analysis Raised protein (damage), decreased glucose (used up), raised WCC (polymorphonuclear) CSF and Blood cultures - sensitivity CSF N.Meningitis antigen detection - detection of capsular polysaccharide antigen If viral - reverse PCR If fungal - test for cryptococcal antigen

Answer 28

Dexamethasone before 1st abx dose = 10mg iv 6 hrly Vancomycin 500-700mg + ceftriaxone 2g iv When sensitisation is discovered the steps are usually Ampicillin -> ceftriaxone -> meropenem

Answer 29

A transient focal neurological disorder that results from focal brain, SC or retinal ischaemia without infarction, that rapidly resolves within 24hrs. Often <1hr

Answer 30

Cardioembolic RFs AF, carotid stenosis, HTN, smoking, alcohol, age DM, hyperlipidaemia, patent foramen ovale, obesity and inactivity

Answer 31

Transient - often resolves before presentation in hospital Weakness, sensory loss, focal neuro ABSENCE of +ve signs = scotomas, spasms, shaking Absence of headache Inc BP at presentation Sometimes described as a curtain pulling over eyes COLLATERAL HX COLLATERAL HX

Answer 32

CT head = normal MRI diffusion scan = 50% have abnormal FBC - N ECG - underlying heart abnormality eg AF PT time - usually N Fasting lipids - pt will need statins Assessment for stenosis - MRI angiography

Answer 33

A type of ACUTE inflammatory neuropathy characterised by motor difficulty, loss of deep tendon reflexes and parasthesias without objective sensory loss. It is an immune mediated attack on myelin sheaths triggered by antecedent infection

Answer 34

Antecedent gastoenteritis / influenza Hep E Weak = HIV, immunisation, male, >40 Cancer/hodgkins - oat cell carcinoma, adenocarcinoma of the lung, small cell lung cancer, and chronic lymphocytic leukaemia.

Answer 35

Loss of deep tendon reflexes Sensory alterations Dysarithria, diplopia, dysphagia, ptosis Aniscoria, back / leg pain, hand + feet paresthesia precedes episodes of weakness, dysautonomia

Answer 36

Nerve conduction studies - SLOWED LP - presence of protein, N/H lymphocytes LFTs - transaminases raised >500units Spirometry - dec VC dec insp+exp pressures ANTIGANGLIOSIDE ANTIBODY TESTING - range which signify GBS

Answer 37

A slowly progressive neurodegenerative disorder characterised by chorea, psychiatric symptoms and personality changes culminating in immobility, mutism and inanimation. AD inheritence Rigid form: children or young adults present with rigidity and may not have chorea. Also called early-onset or rigid Huntington's disease (Westphal variant).[2] Choreic form: patients have chorea as a significant initial presenting feature. This is the most common phenotype.

Answer 38

AD inheritence of repeat CAG mutations on the N terminus of the gene coding for Huntingtin

Answer 39

Personality changes, impulsivity, chorea, twitching, restlessness, dec coordination, dec fine motor, cog dec, anxiety, apathy, depressions obsessions and compulsions, MOTOR impersistence

Answer 40

CAG repeat testing - >40 = disease 36-39= possible disease ANTICIPATION MRI/CT evidence of caudate/striatal atrophy

Answer 41

A condition characterised by the clinical features of hydrocephalus BUT without an increase in ICP, as measured by LP. Clinical features = Levedopa unresponsive gait apraxia w/wo urinary incontinence / cognitive impairment Still responds to a reduction in CSF pressure

Answer 42

``` Age >65 Levodopa unresponsive gait Mental slowing and impairment Urinary frequency, urgency and incontinence Faecal incontinence ```

Answer 43

CT/MRI head = normal or mild-mod ventricular enlargement Levodopa challenge - unresponsive LP - opening pressure 7-25cm water LP with large volume tap = improvement of gait

Answer 44

Facial pain syndrome occurring in >1 divisions of the trigeminal nerve, characterised by sharp stabbing intense pain that lasts >2mins w/o associated neuro dysfunction

Answer 45

80-90% = caused by abhorrent vascular loop | Other causes = demyelinating plaques of MS pressing on nerve root /// amyloid deposition

Answer 46

Pain distributed only in trigeminal regions Prior trauma = other cause Neuro dysfunction = other cause

Answer 47

A neurological emergency resulting from thiamine deficiency with varied neuro-cognitive symptoms. Typical triad is opthalmoplegia, gait dysfunction and mental state changes.

Answer 48

Alcohol dependence, chemotherapy, Male, malnutrition, HIV Bm transplants, genetic, formula milk

Answer 49

Mental slowing, inability to concentrate, confusion, delirium, psychosis, irritability, 1/3 have occulomotor findings Triad in 20% mental state change, gait dysfunction, opthalmoplegia others = coma, papilloedema, tachycardia, anisocoria, miosis, hearing loss, seizures, spastic paralysis

Answer 50

Serum thiamine and metabolites - L Therapeutic trial of thiamine - clinical response LFTs- often all raised Serum Mg2+ - can be low - cofactor of thiamine Brain MRI - involvement of mammillary bodies, dorsomedial hypothalamic nucleus, peraqueductal grey matter and cerebellar vermis

Answer 51

A rapidly developing neurological disorder with focal neurological insult of presumed cerebrovascular origin. It lasts longer than 24hrs and can be divided into haemorrhagic or ischaemic. ``` Ischaemic = insult arises due to cerebral underperfusion resulting from the occlusion or stenosis of a cerebral artery Haemorrhagic = results when either an artery ruptures and blood accumulates in the cerebral parenchyma, subarachnoid space or cerebral ventricles. ```

Answer 52

HTN, alcoholism, a/cs, asian, coagulopathy, haemophilia, FHx, AGE, vascular malformations, cocaine

Answer 53

Focal signs eg dysarithria, dysphagia, photophobia, weakness, sensory loss, confusion, ataxia, vertigo, confusion, altered consciousness KEY = N+V IS NEVER SEEN IN ISCHAEMIC, BUT OCCURS IN HAEMORRHAGIC THUNDERCLAP = subarachnoid haemorrhage

Answer 54

Non-contrast head CT - hyperdense wedge/lesion MRI - appears hypointense INR - N/H Urine drug screen - cocaine mebes CT angiography - visualisation of blood leakage / aneurysm

Answer 55

IMMEDIATE NEUROSURGICAL + NEUROCRITICAL EVALUATION Potential surgery. Pt needs: BP control - nifedipine 5mg IV If clotting abnormality then requires CCP/FFP/Plts DVT prophylaxis after stabilisation - heparin ANIMAL MODELS = hypothermia better outcome therefore cooling blanket plus paracetamol IV PLUS supportive ie aspiration risk / correction of hyperglycaemia If lobar may need phenytoin

Answer 56

``` ECranial atherosclerosis 10% IC atherosclerosis 10% Cardioembolic - 25% Lacunar infarction - 15% Indeterminate - 30% Other (eg dvts+vasculitis) - 10% ```

Answer 57

Head CT - hypoattenuation (DARK) brain parenchyma, loss of grey/white differentiation, sulci effacement, HYPERattenuation in vessel =thrombus. MRI - appears bright CT/MRI angiogram or TCdoppler- blood vessel occlusion ECG - underlying cardiac disease Heart enzymes - rule out MI U+Es - rule out RF / electrolyte imbalance PT/APTT - coagulopathy FBC - exclude anaemia / thrombocytopenia Serum toxicology - Drug taking

Answer 58

IF AVAILABLE - THROMBECTOMY <4.5hrs presentation + no contraindication to thrombolysis - Alteplase 0.9mg/kg with 10% bolus over 1hr + 300mg aspirin 24hrs later + DVT prophylaxis, swallowing assessment, supportive care >4.5 hrs / contraindication - Aspirin 300mg + supportive, DVT and swallow If cerebral sinus thrombosis = don't use aspirin use heparin-warfarin 8-12% mortality

Answer 59

STRONG Intranasal flu Pregnancy ``` WEAK URTI Black/hispanic Cold climate HTN FHx bells DM ```

Answer 60

``` Keratoconjunctivitis sicca Ulcerative keratitis Ectropion Contracture and synkinesis Gustatory hyperlacrimation ```

Answer 61

The extent of facial palsy following complete evolution of Bell's palsy (i.e., within 72 hours of onset) is the parameter most predictive of ultimate recovery outcome. Of those who present with incomplete paralysis on clinical examination, 94% will fully recover, as compared to 61% of those who present with complete paralysis

Answer 62

Bell's palsy Post-auricular pain commonly occurs concurrently or as a prodrome to facial palsy in Bell's palsy.[1] At least one study demonstrated that up to 8% of those diagnosed with Bell's palsy present with additional cranial neuropathies, most commonly of the trigeminal nerve.[5] Facial weakness in Bell's palsy may range from a mild palsy to complete flaccid paralysis with absence of volitional and evoked electromyography (EMG) activity. One author has defined four patterns of facial palsy onset in Bell's palsy in order of worsening prognosis: 1) sudden incomplete, without progression; 2) sudden incomplete, with progression, yet remaining incomplete; 3) sudden complete; and 4) sudden incomplete, with rapid progression to complete.[6] Degrees of otalgia, post-auricular pain, facial dysaesthesias, hypogeusia, and hyperacusis vary.

Answer 63

Cluster headache Migrainous features can be seen in cluster headache, including aura symptoms (14% of patients[1]), ipsilateral photophobia and/or phonophobia (61.2%), and nausea and vomiting (27.8%).[3] During remission periods, patients may report mild pre-headache sensations or shadows in the same location as the cluster headaches. Three percent of patients fail to report autonomic features and agitation during attacks. Continuous background pain has been reported in nearly one third of patients with chronic cluster headache.

Answer 64

``` Male FHx Head injury Cigarette smoking Heavy Drinking ```

Answer 65

ACUTE 1. Oxygen 1. Subcut sumatriptan 2. Intranasal zolmitriptan 3. Intranasal sumatriptan 3. Intranasal lidocaine TRANSITIONAL THERAPY NEEDED Prednisolone 60mg OD 5 days, decrease by 10mg/day every 3 days OR Dihydroergotamine - 1 mg intravenously as a single dose, may repeat after 60 minutes if required, maximum 2 mg/day or 6 mg/week Adjunct: Greater occipital nerve (GON) block has been found to render nearly two-thirds of patients pain-free at 4 weeks DO NOT GIVE SUMATRIPTAN TO CVD / UNCONTROLLED HTN ``` ONGOING Rx Verapamil Lithium Topiramate Gabapentin Melatonin Valproate Semi-sodium Surgery - occipital nerve stimulation ```

Answer 66

Depression = only complication Long-term prognosis is unclear. However, epidemiological studies have suggested that symptoms tend to improve with increasing age. Although the disease is excruciating and incapacitating, there are no real long-term complications.

Answer 67

Encephalitis is defined as inflammation of the brain parenchyma associated with neurological dysfunction such as altered state of consciousness, seizures, personality changes, cranial nerve palsies, speech problems, and motor and sensory deficits. It is the result of direct inflammation of the brain tissue, as opposed to the inflammation of the meninges (meningitis), and can be the result of infectious or non-infectious causes. Unfortunately, an aetiological agent is only identified in around 50% of cases. Pathological state of brain parenchymal dysfunction leading to an altered state of consciousness or focal neurological signs. Serious, complex, and potentially fatal disorder with non-infectious and infectious causes. Presents with acute onset of a febrile illness and altered mental status; typical features include headache, seizures, and focal neurological signs. Investigations should include blood cultures, neuroimaging (preferably MRI), and CSF analysis. Aciclovir should be administered as soon as possible in all cases of suspected viral encephalitis. Complications include seizures, hydrocephalus, and neurological sequelae (e.g., behavioural disturbances, motor problems).

Answer 68

Around 2500 cases of encephalitis occur in the UK and Ireland each year. There is no specific gender predominance, but frequently a bimodal distribution is seen with respect to age groups: under one year and over 65 years. Seasonal (summer and early autumn for enteroviruses and most arboviruses) and geographical (certain arboviruses) variations occur in some cases of viral encephalitis in the US and other parts of the world.

Answer 69

STRONG ``` <1, >65 Immunodeficiency Viral infections Blood/body fluid exposure Organ transplantation Animal/insect bites Location Season Freshwater swimming ``` WEAK ``` Vaccination Infected contact exposure Trekking Spelunking (CAVE EXPLORING) Death in animals Cancer ``` CAUSED BY: Viral - herpesvirus - HSV1/2, VZV, CMV, EBV, HHV6, FLAVIVIRUS Bacteria - N men, TB, syphilis, listeria, bartonella, B burgdorferi Fungal - cryptococcus, coccidioides, histplasma, candida etc Parasitic - Toxoplasma gndi, amoebic, entamoeba histolytica Para-infectious - Acute disseminated encephalomyelitis (ADEM), Acute haemorrhagic leukoencephalitis, Rasmussen's encephalitis, Bickerstaff's encephalitis Prion diseases -CJD Paraneoplastic (autoimmune) syndromes - Encephalomyelitis, Anti-GABA receptor encephalitis, Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis

Answer 70

COMMON ``` Fever Rash Altered mental state Focal neuro Meningismus Cough GI infection Seizures - generalised tonic-clonic seizures, partial complex seizures, and focal seizures (with secondary generalisation) ``` UNCOMMON ``` Parotitis - mumps Lymphadenopathy - bartonella Optic neuritis - ADEM Acute flaccid paralysis - rabies/west nile Movement disorder Biphasic illness Autonomic/hypothalamic disturbance Myocarditis/pericarditis Jaundice Arthritis Retinitis Parkinsonism ```

Answer 71

FBC - WCC raised Blood smear - falciparum Electrolytes - hyponatraemia LFTs - elevated in (Coxiella burnetii, Rickettsia, tick-borne disease, CMV, EBV) Blood cultures Throat swab Nasopharyngeal aspirate PCR Sputum culture if pulmonary Sx Chest radiography - Routinely performed as part of a febrile work-up CT - ordered in all patients with altered mental state. Often normal in encephalitis MRI - highly recommended (preferably initially) in suspected encephalitis and is invaluable in diagnosis EEG - often shows background slowing LP - findings depend on aetiology; may have elevated white cell count (WCC), normal/elevated protein, normal/low glucose, normal/elevated RBC CSF culture CSF serology CSF PCR Blood IgM/G serology

Answer 72

Immunocompetent: Aciclovir: 10 mg/kg intravenously every 8 hours for 10-21 days Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, DVT prophylaxis, and gastrointestinal (ulcer) prophylaxis. If ICP consider mannitol / corticosteroids Immunocompromised: - Must suspect CMV encephalitis - Foscarnet + ganciclovir added Bacterial: Ben/pen and tailor A/I - steroid Rx / plasma exchange / rituximab / cyclophosphamide

Answer 73

12% mortality in England Due to the varied aetiology, survivors of the critical phases of the illness are a heterogeneous group. Prognosis is often bleak. Mortality and morbidity vary depending on the underlying aetiology, the immune status of the host, the extent and location of anatomic lesions, the development of complications, and the time to initiate treatment. Age >65 years old, immunocompromised (HIV or immunosuppressive medication-induced), mechanical ventilation, coma, acute thrombocytopenia, elevated CSF polymorphonuclear count, cerebral oedema, and status epilepticus are associated with poor outcomes. Post-encephalitic epilepsy occurs in 10% by 5 years and 20% by 20 years.

Answer 74

``` Death Hypothalamic /autonomic dysfunction Ischaemic stroke Encephalitis lethargica Neurological sequelae Seizures Cerebral haemorrhage Cerebral vein thrombosis Cerebral vasculitis Hydrocephalus Post-viral chronic fatigue syndrome ```

Answer 75

Focal (or partial) seizures refer to the electrical and clinical manifestations of seizures that arise from one portion of the brain. Focal seizures most commonly arise from the temporal lobe. Simple focal seizures are those in which consciousness is preserved. The clinical definition of epilepsy was revised in 2014 to include any of the following conditions: 1) at least two unprovoked seizures occurring >24 hours apart; 2) one unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years; 3) diagnosis of an epilepsy syndrome. Electrical and clinical manifestations of seizures that arise from one portion of the brain. May evolve to bilateral tonic-clonic seizures. Underlying structural brain abnormalities are known to cause a localised epileptiform focus, but idiopathic cases may occur. History taking is the most important aspect of diagnosis. Supportive tests, although helpful, need not be abnormal for a diagnosis of focal seizures. Monotherapy with antiepileptic medication is the preferred treatment. When polytherapy is required, this should be approached rationally, by considering the combination of different mechanisms of action and different adverse-event profiles. Patients with 2 failed monotherapy trials followed by a failed trial of polytherapy are considered to have refractory focal seizures and should be assessed for the possibility of resective epilepsy surgery.

Answer 76

4 to 8 per 1000 people. In industrialised countries, age-specific incidence is highest in the under 20 years of age group (especially the first few months of life) and in adults aged >60 years, with the lowest incidence in between. The incidence of epilepsy is somewhat higher for men.

Answer 77

Traumatic brain injury is an increasingly common aetiology for focal seizures. Penetrating head injuries have the highest risk for the development of epilepsy. For a closed head injury to carry a significant seizure risk there should be loss of consciousness or amnesia lasting longer than 30 minutes. Central nervous system (CNS) infection may result in focal seizures. Brain tumours are another source of focal seizures; low-grade tumours seem more epileptogenic than high-grade tumours. Focal seizures may also develop after a stroke. Both Alzheimer's disease (AD) and non-AD dementia have been associated with seizure development. Neurocutaneous syndromes such as neurofibromatosis, Sturge-Weber syndrome, and tuberous sclerosis may result in focal or generalised seizures. Although some intracranial arteriovenous malformations are largely asymptomatic, others, including cavernous haemangiomas, carry a risk of seizures.

Answer 78

STRONG ``` Febrile seizures Head trauma CNS infection Stroke Brain tumour Mental retardation / cerebral palsy Dementia FHx Vascular malformations ``` WEAK Male

Answer 79

COMMON Movement of one side of body/specific part Premonitory sensation - (fear, epigastric sensation, déjà vu, jamais vu) Automatisms (picking at clothes, smacking of the lips) Aphasia Staring and loss of contact with the environment A postictal paralysis or weakness on one side of the body is suggestive of a focal seizure and generally indicates that the seizure focus is contralateral to the side of the deficit. Persistent focal neurological deficit UNCOMMON Poor memory Stigmata of neurocutaneous syndromes

Answer 80

Blood glucose - hypoglycaemia or hyperglycaemia FBC - establishing an underlying systemic or CNS infection Electrolyte panel - Electrolyte disturbances including uraemia and decreased/elevated sodium; magnesium or calcium abnormalities should be excluded Toxicology screen LP + CSF analysis CT head - intracranial haemorrhage, skull fracture, presence of structural lesion MRI brain - anatomical temporal lobe abnormalities (mesial temporal sclerosis, neoplastic lesions, vascular malformations, and developmental lesions) EEG - spikes or sharp waves in one temporal lobe Consider Prolactin - elevated in focal seizures with impairment of consciousness (complex focal seizures), Needs to be measured 10 to 20 minutes after an event, which greatly limits utility in actual practice. Video EEG functional MRI

Answer 81

MULTIPLE SEIZURES IN SHORT INTERVAL 1) Lorazepam/diazepam + airway maintenance 2) Phenytoin/fosphenytoin 3) Propofol/phenobarbital Ongoing: Frequently chosen first-line AEDs include lamotrigine, levetiracetam, oxcarbazepine, or carbamazepine. Cochrane review supports the use of carbamazepine and lamotrigine as first-line treatments for adults with focal-onset seizures If the patient does not respond to 2 separate monotherapy trials, then a polytherapy trial may be initiated using 2 first-line AEDs combined or a choice may be made from the list of second-line therapies, such as lacosamide, pregabalin, perampanel, eslicarbazepine, or clobazam. 4th line: Resective epilepsy surgery / vagal nerve stimulation / responsive neuro stimulation system / ketogenic diet Pregnant = lamo / carbam

Answer 82

Nearly two-thirds of patients with focal seizures are controlled with antiepileptic drugs (AEDs), either mono- or polytherapy. Most patients are treated with AEDs for at least 2 years. Tapering of AEDs may be considered when a patient has remained seizure free for 2 years. About one third of patients continue to have seizures despite 2 monotherapy and 1 polytherapy trials. Others have good seizure control but unacceptable adverse effects of AEDs. These patients should be evaluated for resective epilepsy surgery.

Answer 83

Head trauma Bone fracture Memory loss Sudden unexpected death in epilepsy SUDEP The major risk factor for SUDEP is the occurrence of generalised tonic-clonic (GTCS) seizures, and the risk of SUDEP increases in association with increasing frequency of GTCS occurrence.

Answer 84

Common seizure type characterised by loss of consciousness, widespread motor manifestations of tonic contractions followed by clonic jerking movements, and a suppressed level of arousal following the event. May either reflect an underlying generalised-onset epilepsy or focal epilepsy that has secondarily generalised. Magnetic resonance imaging and an electroencephalogram (EEG) are essential tests for properly diagnosing an epilepsy syndrome after a generalised tonic-clonic seizure (GTCS). During a GTCS, the EEG will demonstrate bilateral synchrony in the epileptiform activity. After a GTCS, treatment depends on the type of epilepsy syndrome identified. Generalised-onset epilepsy is treated differently to focal epilepsy. With proper diagnosis, most seizure disorders respond to treatment. The primary treatment complications include lack of efficacy and medication side effects or idiosyncratic reactions.

Answer 85

Generalised tonic-clonic seizures (GTCSs) represent 25% of all seizure types This incidence does not seem to differ based on age, sex, or ethnicity.

Answer 86

Generalised tonic-clonic seizures (GTCSs) Aetiology depends directly on the epilepsy syndrome or provoking element that initiated the epileptiform activity. GTCSs associated with any epilepsy syndrome are termed unprovoked, while GTCSs caused by a reversible stimulus are termed provoked. Provoked seizures can be caused by toxins, illicit substances, medications that lower seizure thresholds, or metabolic derangements. Generalised-onset epilepsies - Aetiology is presumed to be genetic in most cases.

Answer 87

``` STRONG FHx CNS infection Head trauma Hx ``` WEAK Hx substance abuse Premature birth Multiple or complicated febrile seizures

Answer 88

COMMON Focal neuro deficits/symptoms Premonitory sensation or experience (fear, epigastric sensation, déjà vu, jamais vu) Temporary hemiparesis (focal-onset epilepsy) Temporary aphasia Fever, nuchal rigidity, altered mental status - men/enc Some physical signs (neurocutaneous findings) may suggest an underlying neurological disease that is associated with seizures. For instance, the presence of ash-leaf spots, shagreen patches, facial angiofibromas, and/or periungual fibromas are often indicative of tuberous sclerosis. Café au lait spots, axillary freckling, and/or fibromas are suggestive of neurofibromatosis.

Answer 89

EEG - generalised epileptiform activity or focal, localising abnormality BG - extreme hypoglycaemia or hyperglycaemia FBC - infection Electrolytes - electrolyte imbalance, particularly hyponatraemia, hypernatraemia, or uraemia, can lead to a provoked generalised tonic-clonic seizure Toxicology - Cocaine, amfetamines, heroin, phencyclidine, and gamma-hydoxybutyric acid CT - structural Prolactin - greater than twice baseline indicates GTCS (<20 mins since seizure) Serum CK - When drawn several hours after the event or followed sequentially until the next day, an elevated serum CK can provide evidence that a generalised tonic-clonic seizure has occurred. However, this test has questionable reliability and is not widely used Consider LP MRI brain

Answer 90

Basic life-support measures should be initiated, including securing the airway, delivering 100% oxygen by mask or nasal cannula, and correcting hypotension, hyperthermia, glucose and electrolyte abnormalities, and potential thiamine deficiency. ECG should be monitored. 1) IV / rectal lorazepam/diaze/midaz 2) Phenytoin/fosphenytoin ONGOING: >/=2seizures: An appropriately chosen single medication is likely to be effective for most patients. Monotherapy ensures the lowest risk of side effects and drug-drug interactions. One 2017 Cochrane review supports the use of sodium valproate as first-line treatment for individuals with GTCSs (with or without other generalised seizure types). 2) change to another When a second agent has been chosen, the current and new medications should be cross-titrated slowly; agents should not be started or stopped abruptly. A written schedule is often required to help patient compliance. 3) Add a second drug At this stage of epilepsy treatment, it is also appropriate to investigate the possibility of localisation-related epilepsy in any patient who did not have a formal epilepsy syndrome diagnosed. A patient with a well-localised epileptic focus should be considered a candidate for resective epilepsy surgery and referred to a tertiary epilepsy centre.

Answer 91

In general, all patients with GTCSs respond well to treatment. When an accurate epilepsy syndrome diagnosis is made, and the appropriate medication is chosen, a majority of patients do well, with approximately 60% achieving freedom from seizures.

Answer 92

SE drugs Worsening seizures Status epilepticus - Status epilepticus is defined as more than 5 minutes of continuous seizure activity or two or more discrete generalised tonic-clonic seizures without complete recovery between events. Efficacy failure

Answer 93

generalised seizures

Answer 94

generalised seizures

Answer 95

Ambulatory within 2w onset OR non-ambulatory within 4w onset WITHOUT IgA Deficiency or renal failure 1) Plasma Exchange 1) IVIG Both plasma exchange and intravenous immunoglobulin (IVIG) have been shown to be equally efficacious. The choice between them is often institution-dependent. IVIG - can precipitate anaphylaxis in an IgA-deficient person ``` SUPPORTIVE Rx BP Ventilatory support DVT Pain - gabapentin or carbamazepine Rehabilitation ``` If IgA Def - only plasma exchange can be offered

Answer 96

``` Resp failure Bladder areflexia Adynamic Ileus Paralysis Fatigue Immobilisation hypercalcaemia DVT ```

Answer 97

The overall prognosis of GBS is good, with approximately 85% of survivors making a good functional recovery. Mortality of 20% has been shown in ventilated patients. Prognosis worsens with older age. Factors associated with poorer outcome include more severe weakness, rapid onset, older age, muscle wasting, electrically inexcitable nerves, and preceding diarrhoeal illness.

Answer 98

This condition is characterised by an immune-mediated attack on myelin sheath or Schwann cells of sensory and motor nerves. This is due to cellular and humoral immune mechanisms, frequently triggered by an antecedent infection. Although genetic predisposition has not been fully established, the acute motor axonal neuropathy (AMAN) type of the disease occurs more commonly in Japan and China than in North America or Europe. Polymorphisms in macrophage mediators (MMP-9 and TNF-alpha) have been associated with severe weakness and poorer outcome in GBS patients

Answer 99

1) Couselling Counselling involves recognition of impulsivity, and safety advice regarding driving, use of firearms, and discarding accumulated prescription medicines. OT / Physio / SALT / Nutrition 2) Chorea control: Tetrabenazine and antipsychotics are the most efficacious medicines available. Amantadine/diazepam also used 3) If depressed - antidepressants 4) Mood stabilisers possible 5) If bradykinesic/rigid WO behavioural problems: Carbidopa/levodopa Refractory depression: ECT

Answer 100

Depression should improve with therapy in people with Huntington's disease. Dose reduction or tapering may be appropriate after sustained improvement is seen, although a low threshold should exist for reintroducing therapy in view of the ongoing organic cause (Huntington's disease) of the depression. Many people with Huntington's disease stay on antidepressants for the rest of their lives once treatment has been commenced. Chorea may lessen in severity in some patients with disease progression and, as such, occasional dose reductions in antichoreic therapy are warranted to see whether lower doses are effective or still needed. Behavioural problems may lessen or fluctuate as cognitive impairment worsens. Other symptoms of Huntington's disease (e.g., dysphagia, dysarthria, impaired gait, cognitive dysfunction, and incoordination) are progressive. The duration of disease is approximately 20 years from time of diagnosis to time of death. The sequential evolution of events and ultimately the recurrent nature of Huntington's disease, from the perspective of a child born to an affected parent, can be depicted pictorially. Events within an affected family may occur in different sequences for different individuals.

Answer 101

``` WL Dysphagia Suicide Falls Incontinence ```

Answer 102

Huntington's disease n children or young adults, Huntington's disease may present as a decline in performance at school or work, associated with rigidity and incoordination but little or no chorea. Generalised slowing and awkwardness to movement may be noted, and ocular motility examination often reveals slowed saccadic eye movements. Children with Huntington's disease are more likely to inherit the disorder from an affected father because the pathogenetic trinucleotide repeat is less stable in spermatogenesis than oogenesis, making it more susceptible to expansion. Sometimes, because of divorce or denial, the family history of Huntington's disease is not known, making the disorder particularly difficult to diagnose.

Answer 103

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of increased intracranial pressure that occurs mainly in overweight women of childbearing years, often in the setting of weight gain. Its cause is not known (hence the preferred name IIH). It is a syndrome characterised by increased intracranial pressure and its associated signs and symptoms in an alert and orientated patient but without localising neurological ﬁndings. There is no evidence of deformity or obstruction of the ventricular system, and neurodiagnostic studies are normal except for increased cerebrospinal fluid (CSF) pressure and the related neuroimaging signs. Furthermore, no secondary cause of intracranial hypertension is apparent. IIH can either be self-limited or have a lifelong chronic course. The most popular hypothesis is that idiopathic intracranial hypertension (IIH) is a syndrome of reduced cerebrospinal fluid absorption. Clinical features include headaches, pulse-synchronous tinnitus, transient visual obscurations, visual loss, neck and back pain, and diplopia. Signs include papilloedema, sixth nerve paresis, and disturbances in sensory visual function. Visual field loss is ubiquitous, and the prototype pattern for early loss is enlargement of the blind spot and inferonasal loss. Diagnostic criteria are the modified Dandy criteria. In all patients with IIH, treatment consists of first eliminating causal factors, such as drugs and other conditions known to cause increased intracranial pressure, and instituting a low-sodium weight-reduction diet plus acetazolamide when indicated. Therapy can be given to reverse and prevent loss of vision.

Answer 104

More than 90% of patients are obese, and >90% are women of childbearing age. The annual incidence of idiopathic intracranial hypertension (IIH) in the US is 0.9/100,000 persons, and 3.5/100,000 in females aged 15 to 44 years. Among obese women aged 20 to 44 years who are ≥20% over their ideal weight, the incidence is 19/100,000 The mean age at diagnosis is about 30 years. Incidence is the same in pregnant and non-pregnant women and can occur at any trimester of pregnancy.

Answer 105

Many aetiologies have been suggested but none proven. Any aetiology must explain the preponderance of women and presence of obesity. It is thought that there is increased resistance to CSF outflow along one or both of these pathways, but abnormalities of these absorption pathways in idiopathic intracranial hypertension (IIH) patients remain unproven.

Answer 106

KEY RFs Visual field loss FHx ``` COMMON Headache Transient visual obscurations Pulse-synchronous tinnitus Photophobia Retrobulbar pain Optic disc swelling Decreased visual acuity Ocular motility defects ``` UNCOMMON Diplopia Relative afferent pupillary defect

Answer 107

Visual field testing - defects Optic disc photographs - Frisen staging of papilloedema MRI brain - negative intracranial and intraorbital pathology; empty sella; flattening of the globe LP - elevated: opening pressure >250 mm H2O MR venogram

Answer 108

1) Elimination of causal factors EG medications - (e.g., discontinue tetracyclines, retinoids, or excessive vitamin A, restart corticosteroid therapy and taper more slowly, start a low-sodium diet, and advise mild fluid restriction). 2) Acetazolamide / furosemide 3) Headache control - analgesia eg naproxen / amitriptyline +/- CSF Shunting 4) Visual loss: optic nerve sheath fenestration 5) Optical prisms 6) Transverse sinus stenting

Answer 109

STRONG Female Weight gain Sleep apnoea Medications: There is reasonable evidence that the following medications and conditions can cause increased intracranial pressure: nalidixic acid,[18] nitrofurantoin,[19] ketoprofen or indometacin (in Bartter's syndrome),[20][21] vitamin A intoxication,[22] isotretinoin,[23] all-trans retinoic acid,[24] thyroid replacement therapy in hypothyroid children,[25] lithium,[26] and anabolic steroids (which may cause venous sinus thrombosis).[27] There are many associated causes of idiopathic intracranial hypertension (IIH), with varying strengths of association. One likely cause is decreased flow through arachnoid granulations, due to scarring from previous inflammation (e.g., meningitis, sequel to subarachnoid haemorrhage). Another is obstruction to venous drainage, which may result from venous sinus thromboses due to hypercoagulable states, contiguous infection (e.g., middle ear or mastoid-otitic hydrocephalus), bilateral radical neck dissections, superior vena cava syndrome, or increased right heart pressure. IIH may also be associated with endocrine conditions, such as Addison's disease, hypoparathyroidism, obesity, and corticosteroid withdrawal. Further likely causes include nutritional disorders, such as hypervitaminosis A (vitamin, liver, or isotretinoin intake) and hyperalimentation in deprivation dwarfism, as well as arteriovenous malformations and dural shunts.

Answer 110

The disease can either be self-limited or have a lifelong chronic course. Generally, the worse the papilloedema, the worse the visual loss, so this factor has to be considered in treatment decisions, especially if the grade of papilloedema is increasing. With treatment, about 50% of patients have marked improvement in visual field function. Other groups at risk of severe loss of vision are black males, patients with glaucoma or marked arterial hypertension, and patients with rapid corticosteroid tapering. Men are at greater risk than women of severe visual outcome.

Answer 111

``` Severe, irreversible visual loss can occur in advanced presentations of the disease. Symptomatic patients with extensive field loss and severe papilloedema should should be considered for a surgical procedure emergently. Cerebrospinal fluid (CSF) diversionary procedures carry significant risks. The most common complications are shunt occlusion and intracranial hypotension. Shunt malfunction can be accompanied by severe loss of vision. ```

Answer 112

idiopathic intracranial hypertension Other common presentations include severe headaches and visual loss in young women. Usually, the course is chronic.

Answer 113

idiopathic intracranial hypertension Other common presentations include severe headaches and visual loss in young women. Usually, the course is chronic.

Answer 114

Horner's syndrome, also known as oculosympathetic paresis, is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side (ipsilateral) as it is a lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhydrosis (decreased sweating), with apparent enophthalmos (inset eyeball). The nerves of the sympathetic trunk arise from the spinal cord in the chest, and from there ascend to the neck and face. The nerves are part of the sympathetic nervous system, a division of the autonomic (or involuntary) nervous system. Once the syndrome has been recognized, medical imaging and response to particular eye drops may be required to identify the location of the problem and the underlying cause.

Answer 115

ptosis (drooping of the upper eyelid)[3] anhidrosis (decreased sweating)[4] miosis (constriction of the pupil)[4] sinking of the eyeball into the face[4] inability to completely close or open the eyelid[4] facial flushing[4] headaches[4] loss of ciliospinal reflex bloodshot conjunctiva, depending on the site of lesion. unilateral straight hair (in congenital Horner's syndrome); the hair on the affected side may be straight in some cases. heterochromia iridum (in congenital Horner's syndrome)[4] Interruption of sympathetic pathways leads to several implications. It inactivates the dilator muscle and thereby produces miosis. It inactivates the superior tarsal muscle which produces ptosis. It reduces sweat secretion in the face. Patients may have apparent enophthalmos (affected eye looks to be slightly sunken in) but this is not the case. The ptosis from inactivation of the superior tarsal muscle causes the eye to appear sunken in, but when actually measured, enophthalmos is not present.

Answer 116

Causes can be divided according to the presence and location of anhidrosis: Central (anhidrosis of face, arm and trunk) Syringomyelia Multiple sclerosis Encephalitis Brain tumors Lateral medullary syndrome Preganglionic (anhidrosis of face) Cervical rib traction on stellate ganglion Thyroid carcinoma Thyroidectomy Goiter Bronchogenic carcinoma of the superior fissure (Pancoast tumor) on apex of lung Klumpke paralysis Trauma - base of neck, usually blunt trauma, sometimes surgery. As a complication of tube thoracostomy Thoracic aortic aneurysm Postganglionic (no anhidrosis) Cluster headache - combination termed Horton's headache An episode of Horner's syndrome may occur during a migraine attack and be relieved afterwards[6] Carotid artery dissection/carotid artery aneurysm Cavernous sinus thrombosis Middle ear infection Sympathectomy Nerve blocks, such as cervical plexus block, stellate ganglion or interscalene block

Answer 117

Cocaine drop test: Cocaine eyedrops block the reuptake of post-ganglionic norepinephrine resulting in the dilation of a normal pupil from retention of norepinephrine in the synapse. However, in Horner's syndrome the lack of norepinephrine in the synaptic cleft causes mydriatic failure. A more recently introduced approach that is more dependable and obviates the difficulties in obtaining cocaine is to apply the alpha-agonist apraclonidine to both eyes and observe the increased mydriatic effect (due to hypersensitivity) on the affected side of Horner syndrome (the opposite effect to what the cocaine test would produce in the presence of Horner's).[citation needed] Paredrine test: This test helps to localize the cause of the miosis. If the third order neuron (the last of three neurons in the pathway which ultimately discharges norepinephrine into the synaptic cleft) is intact, then the amphetamine causes neurotransmitter vesicle release, thus releasing norepinephrine into the synaptic cleft and resulting in robust mydriasis of the affected pupil. If the lesion itself is of the third order neuron, then the amphetamine will have no effect and the pupil remains constricted. There is no pharmacological test to differentiate between a first and second order neuron lesion.[7] Dilation lag test[clarification needed]

Answer 118

Suitable for surgery: Ventriculoperitoneal shunting or endoscopic third ventriculostomy A catheter is placed into the lateral ventricle under general anaesthesia, usually via the non-dominant cerebral hemisphere, and the distal end placed in the peritoneal cavity via a line tunnelled under the skin. The valve is usually positioned behind the ear. A programmable shunt is used. Endoscopic third ventriculostomy (ETV) is an alternative option to shunting. Unlike shunting, ETV does not require prosthesis implantation. However, preliminary results from an open label randomised trial found that shunting is superior to ETV, as it is associated with better functional neurological outcomes 12 months after surgery. Control of CV RFs NOT SUITABLE FOR SURGERY Some patients who refuse surgery, or those who are unsuitable for surgery, may respond to repeated large-volume CSF taps, with apparent improvement sustained for weeks or months. This involves the removal of 30 to 60 mL of CSF via lumbar puncture. The procedure improves cerebral blood flow temporarily, possibly by reducing the turgor of the subarachnoid space and relieving obstruction to CSF flow at the aqueduct.

Answer 119

Response to surgery is variable, but a positive response is less likely in those with extensive radiological change on neuroimaging. Response should be evaluated by video-recording a timed walk, and by repeat testing of cognitive function. Improvement of symptoms provides a reasonable reflection of the success of surgery. Gait is usually the symptom most improved postoperatively, cognitive function somewhat less, and urinary symptoms show the least improvement

Answer 120

``` Stroke Subdural haematoma Bleeding Shunt infection Vascular disease and cognitive impairment ```

Answer 121

The underlying aetiology is still unclear. However, many theories have been proposed, including: Abnormal absorption of cerebrospinal fluid (CSF) Build-up of toxic metabolites in the CSF Abnormal arterial pulsatility compressing the venous vasculature. While often quoted in the literature, these theories are considered implausible as they do not take into account the physiological function of CSF.

Answer 122

normal pressure hydrocephalus Other presentations Faecal incontinence is a feature of advanced disease and is rare.

Answer 123

Extravasation of blood into the space existing between the dura mater and skull. Usually arterial (10% from veins) Often there is loss of consciousness following a head injury, a brief regaining of consciousness, and then loss of consciousness again. Other symptoms may include headache, confusion, vomiting, and an inability to move parts of the body. The cause is typically head injury that results in a break of the temporal bone and bleeding from the middle meningeal artery

Answer 124

M>F 4% of head injuries Young adults mainly

Answer 125

The cause is typically head injury that results in a break of the temporal bone and bleeding from the middle meningeal artery. Occasionally it can occur as a result of a bleeding disorder or blood vessel malformation

Answer 126

Epidural hematoma commonly results from a blow to the side of the head. The pterion region, which overlies the middle meningeal artery, is relatively weak and prone to injury. Thus, only 20 to 30% of epidural hematomas occur outside the region of the temporal bone. The brain may be injured by prominences on the inside of the skull as it scrapes past them. Epidural hematoma is usually found on the same side of the brain that was impacted by the blow, but on very rare occasions it can be due to a contrecoup injury.

Answer 127

Lucid period Severe headache Impingement on 3rd CN = down and out + fixed dilated pupil Weakness of extremities due to compression of crossed pyramidal pathways Contralateral visual loss due to compression of posterior cerebral artery May progress to: tonsillar herniation cushings triad (HTN, brad, irreg breathing) Respiratory arrest

Answer 128

CT or MRI (usually CT unless negative MRI) Epidural hematomas usually appear convex in shape because their expansion stops at the skull's sutures, where the dura mater is tightly attached to the skull. Thus, they expand inward toward the brain rather than along the inside of the skull, as occurs in subdural hematomas. Most people also have a skull fracture. Epidural hematomas may occur in combination with subdural hematomas, or either may occur alone

Answer 129

SURGICAL EMERGENCY - Removal of blood to relieve compression Burr hole or craniotomy If transfer to a facility with neurosurgery is unavailable, prolonged trephination (drilling a hole into the skull) may be performed in the emergency department. Medications may be given after surgery. They may include antiseizure medications and hyperosmotic agents to reduce brain swelling and intracranial pressure It is extremely rare to not require surgery. If the volume of the epidural hematoma is less than 30 mL, the clot diameter less than 15 mm, a Glasgow Coma Score above 8, and no visible neurological symptoms, then it may be possible to treat it conservatively.

Answer 130

The prognosis is better if there was a lucid interval than if the person was comatose from the time of injury. Arterial epidural hematomas usually progress rapidly. However, venous epidural hematomas, caused by a dural sinus tear, are slower. Outcomes are worse if there is more than 50 mL of blood in the hematoma before surgery. Age, pupil abnormalities, and Glasgow Coma Scale score on arrival to the emergency department also influence the prognosis. In contrast to most forms of traumatic brain injury, people with epidural hematoma and a Glasgow Coma Score of 15 (the highest score, indicating the best prognosis) usually have a good outcome if they receive surgery quickly

Answer 131

Seizures Focal neurology Death

Answer 132

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder, characterised by progressive muscle weakness that can start in limb, axial, bulbar, or respiratory muscles and then generalises relentlessly, causing progressive disability and ultimately death, usually from respiratory failure. Progressive disease characterised by degeneration of the motor neurons with cortical, brainstem, and ventral cord locations. Usually presents as a combination of upper motor neuron and lower motor neuron symptoms and signs, involving initially 1 segment of the neuroaxis (i.e., cranial, cervical, thoracic, or lumbosacral), and then progressively spreading typically to contiguous areas. Typical presentations include limb-onset and bulbar-onset ALS, and, less frequently, respiratory-onset ALS. Generalisation of the symptoms follows in time, without intervals of remission, exacerbation, or stabilisation, resulting in progressive disability and death. There is no cure for ALS at the present time. The focus of medical care is to provide supportive and palliative intervention, aiming to improve the quality of life for patients. Riluzole is used for modifying the disease course and should be offered to patients at the time of diagnosis. Continued discussions regarding advance directives, as well as methods of respiratory and nutritional support are critical; palliative care options should also be presented and discussed prior to the need for their initiation.

Answer 133

The incidence of ALS in Europe and the US is about 1.5 to 2.5 per 100,000 per year, with a prevalence of 2.7 to 7.4 per 100,000. The mean age of onset is about 57 years. In age groups <70 years of age, the male-to-female ratio is 1.3:1.5. For age groups >70 years of age, the ratio is close to 1 There are no definite data regarding the distribution of disease among different races, although a higher incidence of ALS in white people as compared with Asian, African, and Hispanic groups has been suggested.

Answer 134

ALS is a neurodegenerative disorder, characterised by progressive loss of cortical (frontotemporal), bulbar (pons, medulla), and ventral cord motor neurons. After motor cell death, retrograde axonal degeneration follows, with subsequent denervation and reinnervation in corresponding muscles. ``` Mechanism unknown, several theories: Glutamate toxicity. Protein misfolding. Oxidative stress. Microglial activation. Mitochondrial dysfunction. Disrupted axonal transport. RNA metabolism. ``` OTHER MND types: Amyotrophic lateral sclerosis (ALS) Primary lateral sclerosis (PLS) (UMN>LMN) Progressive muscular atrophy (LMN>UMN) Amyotrophic lateral sclerosis with frontotemporal dementia

Answer 135

STRONG FHx Age >40 ``` WEAK Military service Athletes Cig smoking Chemical exposure Lead exposure ```

Answer 136

``` Weakness Stiffness Hyperreflexia Poor coordination Spastic unsteady gait painful muscle spasms Difficulty getting out of chairs Foot drop Head drop Progressive difficulty maintaining posture Muscle atrophy Increased lumbar lordosis Dyspnoea Strained slow speech Slurred / nasal speech Falls Drooling Inappropriate bursts of crying/laughing - Pseudobulbar affect, otherwise called emotional incontinence. Cognitive impairment +/- frontotemporal dementia ```

Answer 137

Clinical diagnosis - presence of upper and lower motor neuron signs, disease progression, and absence of any other explanation for the presentation Consider EMG - evidence of diffuse, ongoing, chronic denervation CK often high May wish to rule out MG - anti ACH May wish to rules out Lambert eaton syndrome - anti-voltage gated-CCB antibodies

Answer 138

COUNSELLING and education on disease progression and support offered 1st line - Riluzole - Riluzole prolongs survival in patients with ALS, with potential advantage in patients with bulbar onset. LFTs monitored monthly for the first 3 months, then every 3 months thereafter; neutropenia can also occur but is rare. Resp - BiPAP or chronic ventilation Drooling - carbocysteine WL - may need PEG feed Communication methods once voice is lost. Physio for weakness Spasticity - baclofen: 5 mg orally three times daily initially, increase by 5 mg/dose increments every 3 days according to response, maximum 80 mg/day Antidepressant therapy may be needed with pseudo bulbar affect

Answer 139

ALS follows a progressive course without intervals of remissions, relapses, or stabilisation, causing progressive disability and ultimately death. The disease is highly variable between affected individuals in terms of clinical presentation and time course. Median survival is 3 to 5 years, but survival up to 10 years and even beyond (although rare at approximately 10% to 20%) has been reported. Prognostic factors associated with positive impact on survival include: Treatment with non-invasive positive pressure ventilation (NIPPV)[104] Enteral nutrition[76] Younger age at diagnosis Limb onset[105][106][107] Baseline FVC of >75% (median survival 5 years). Prognostic factors associated with worse outcome include: Older age at diagnosis Bulbar onset Comorbidity with frontotemporal dementia Baseline FVC of <75%.

Answer 140

``` Resp failure Nutritional deficit Aspiration pneumonia Riluzole hepatoxicity Riluzole neutropenia ```

Answer 141

Amyotrophic lateral sclerosis (most common MND) ALS may present as upper motor neuron (UMN)-dominant ALS, lower motor neuron (LMN)-dominant ALS, or bulbar-onset ALS. Patients may present with associated cognitive and behavioural impairment that may precede the onset of motor neuron disease symptoms or may become evident late in the disease course. Some of these patients have frontotemporal dementia meeting the Neary criteria (consensus guidelines for the clinical diagnosis of frontotemporal dementia).[3][4] Patients may occasionally have associated extrapyramidal-type (parkinsonian) symptoms, such as rigidity, resting tremor, postural instability, bradykinesia, or bradyphrenia. They may also present with a combination of UMN and/or LMN symptoms and signs, extrapyramidal signs, and frontotemporal-type cognition deficit. Patients with typical ALS may develop associated autonomic symptoms, such as urinary urgency, constipation, and perspiration, later in the course of the disease.[5][6]

Answer 142

Amyotrophic lateral sclerosis (most common MND) ALS may present as upper motor neuron (UMN)-dominant ALS, lower motor neuron (LMN)-dominant ALS, or bulbar-onset ALS. Patients may present with associated cognitive and behavioural impairment that may precede the onset of motor neuron disease symptoms or may become evident late in the disease course. Some of these patients have frontotemporal dementia meeting the Neary criteria (consensus guidelines for the clinical diagnosis of frontotemporal dementia).[3][4] Patients may occasionally have associated extrapyramidal-type (parkinsonian) symptoms, such as rigidity, resting tremor, postural instability, bradykinesia, or bradyphrenia. They may also present with a combination of UMN and/or LMN symptoms and signs, extrapyramidal signs, and frontotemporal-type cognition deficit. Patients with typical ALS may develop associated autonomic symptoms, such as urinary urgency, constipation, and perspiration, later in the course of the disease.[5][6]

Answer 143

Multiple sclerosis (MS) is defined as an inflammatory demyelinating disease characterised by the presence of episodic neurological dysfunction in at least two areas of the central nervous system (brain, spinal cord, and optic nerves) separated in time and space. Demyelinating central nervous system condition clinically defined by two episodes of neurological dysfunction (brain, spinal cord, or optic nerves) that are separated in space and time. Classically presents in white women, aged between 20-40 years, with temporary visual or sensory loss. However, may affect either sex and any age or ethnic group, and may have variable neurological symptom location and/or duration. May have subtle changes in vision, ambulation, and reflexes on examination that provide evidence of previous attacks (which may not have been noticed by the patient). Magnetic resonance imaging (MRI) of the brain is sensitive, but very susceptible to over-interpretation in the absence of clinical correlation. Spinal MRI is abnormal less often, but lends greater specificity when present with brain lesions. Treatment of the condition can be divided into three parts: treatment of the acute attack; prevention of future attacks by reducing triggers and use of disease-modifying therapies; and symptomatic treatments of neurological difficulties such as spasticity, pain, fatigue, and bladder dysfunction.

Answer 144

F:M 3:1 White female A geographic gradient, with higher incidence at latitudes closer to the poles, has been linked with MS.

Answer 145

Acute relapse: Methylprenisolone Severe worsening - plasma exchange Ongoing: Relapsing-remitting Although there are no strict guidelines for choice of disease-modifying therapies in RRMS, interferon beta preparations, glatiramer, dimethyl fumarate, and teriflunomide are generally considered 'first-line' agents. Rx of urinary frequency with oxybutynin: 5 mg orally (immediate-release) two to three times daily Pain + dysaesthesia - gabapentin: 100 mg orally once daily at bedtime initially, increase by 100 mg/day increments to 300 mg once daily at bedtime; or 100-300 mg three times daily Physiotherapy Secondary progressive: Siponimod: consult specialist for guidance on dose; dose depends on CYP2C9 genotype Primary progressive: ocrelizumab: 300 mg intravenously as a single dose initially, followed by 300 mg as a single dose 2 weeks later, then 600 mg every 6 months Same other Rx for these..

Answer 146

Various factors favouring better prognosis have been supported by older demographic studies done in the pre-treatment era and include female sex, sensory symptoms, or optic neuritis at onset. Poorer prognostic factors include frequent relapses and motor or cerebellar onset. Newer studies have looked at lesion burden on magnetic resonance imaging at onset, indicating that a higher lesion burden at onset portends a poorer prognosis, particularly for cognitive outcomes

Answer 147

``` UTIs Osteopenia/porosis Depression Visual impairment ED Cognitive impairment Impaired mobility ```

Answer 148

MS MS can present in a myriad of ways in individuals from disparate demographic backgrounds. The most common presentations for MS are optic neuritis and transverse myelitis, but other presentations include brainstem syndromes, cerebellar syndromes (ataxia), and sensory syndromes. Patients may also present with a progressive course, often with foot drop or spastic paraplegias in the later years. Incidental findings of changes on brain magnetic resonance imaging consistent with MS are also reported in asymptomatic patients. MS may present with depression, cognitive decline, or even psychosis and should be considered in the differential of these disorders, particularly if there are associated physical symptoms or signs.

Answer 149

MS MS can present in a myriad of ways in individuals from disparate demographic backgrounds. The most common presentations for MS are optic neuritis and transverse myelitis, but other presentations include brainstem syndromes, cerebellar syndromes (ataxia), and sensory syndromes. Patients may also present with a progressive course, often with foot drop or spastic paraplegias in the later years. Incidental findings of changes on brain magnetic resonance imaging consistent with MS are also reported in asymptomatic patients. MS may present with depression, cognitive decline, or even psychosis and should be considered in the differential of these disorders, particularly if there are associated physical symptoms or signs.

Answer 150

Myasthenia gravis (MG) is an uncommon disease with an estimated worldwide prevalence of 100 to 200 per million population. The disease is manifest from infancy to old age and can occur in both genders although more women than men are affected. Women usually present during childbearing age. Men typically develop symptoms at a later age with a median age of onset in the seventh decade. It appears that the prevalence of MG is on the increase, particularly in developed countries

Answer 151

Several lines of evidence suggest that myasthenia gravis (MG) is an organ-specific, antibody-mediated autoimmune disease. Antibodies are present at the neuromuscular junction (NMJ), the site of pathology. About 80% to 90% of patients have detectable antibodies against the nicotinic acetylcholine receptor (AChR) on the post-synaptic muscle membrane at the NMJ. Another 3% to 7% of patients have antibodies directed against muscle-specific tyrosine kinase (MuSK), another NMJ protein. The reduced number of available binding sites for acetylcholine leads to inconsistent generation of muscle fibre action potentials, which manifests as skeletal muscle weakness.

Answer 152

ONGOING 1. Pyridostogmine +/- immunosuppressant pyridostigmine: 30-60 mg orally (regular-release) twice daily initially, increase by 30-60 mg/day increments to a usual dose of 60-120 mg every 3-4 hours while awake, maximum 540 mg/day and/or prednisolone: 15-20 mg orally once daily initially, increase by 5 mg/day increments every 3 days to a dose of 60 mg once daily, continue until clinical improvement or for 2-3 months, whichever is earlier, then taper gradually (Other immunosuppressants can be used) 2. Eculizumab or Rituximab MAY need thymectomy +/- plasma exchange or IVIG 3. SEVERE disease Intermittent IVIG Chronic, intermittent intravenous immunoglobulin (IVIG) therapy, every 4 to 6 weeks, may be indicated in patients who cannot tolerate immunosuppressive therapy SEVERE CRISES Intubation and mechanical ventilation Plasma exchange / IVIG Supportive care includes deep venous thrombosis prophylaxis; ulcer prophylaxis; adequate nutrition and hydration; and avoidance of infections and drugs that may worsen myasthenia symptoms.

Answer 153

Symptomatic improvement and clinical remission are the goals of therapy. However, the onset of improvement varies greatly from days to months. Typically, older men with ocular complaints respond promptly to corticosteroid monotherapy whereas generalised symptoms are slower to respond and require more aggressive therapy. Chronic maintenance drug therapy is often required. Disease exacerbations can occur due to infections, surgery, medicine exposures, malignancy, pregnancy, or other stressors. Myasthenic crisis can sometimes be averted with aggressive early intervention during an exacerbation.

Answer 154

``` Muscarinic side effects - NB can often be decreased by giving in combination with glycopyrronium. Alternatively, loperamide may prove useful for persistent diarrhoea when the dose of pyridostigmine cannot be reduced. Rest failure Impaired swallowing Acute aspiration Secondary pneumonia ``` Plasma exchange reactions - May occur following therapy and include coagulopathy, thrombocytopenia, electrolyte disturbance, arrhythmias, and hypotension. IVIG reactions - May occur during therapy and include headache, aseptic meningitis, worsening of migraine headache, back pain, fever, chills, hives, and rarely anaphylactoid reactions. Occasionally patients may develop reduction of white blood cells, red blood cells, and/or platelets, renal failure, stroke, or myocardial infarction.

Answer 155

myasthenia gravis The disease usually presents with 1 of 3 different forms: ocular, oropharyngeal, or generalised. Approximately 50% of patients present with purely ocular symptoms (ptosis, diplopia), so-called ocular myasthenia.[19] Between 50% and 60% of those who present with purely ocular symptoms will progress to develop generalised disease, and the vast majority will do so within the first 1 to 2 years. Overall, 15% to 20% of patients will experience a myasthenic crisis (exacerbation necessitating mechanical ventilation) and it usually occurs within the first 2 years after diagnosis of MG.[20] The subset of patients with antibodies directed against muscle-specific tyrosine kinase (MuSK) present with 3 predominant phenotypes: severe faciopharyngeal weakness, with atrophy of involved muscles in long-standing disease; predominant neck and respiratory weakness with frequent progression to crisis; and clinical features undistinguishable from non-MuSK MG.[3] Using a cell-based assay, some patients with ocular MG without detectable acetylcholine receptor (AChR) antibodies have been reported to have MuSK autoantibodies.[21] The clinical phenotype of LRP4- and agrin-antibody-positive myasthenic people is not as yet clear, although in one report LRP4 patients had mild to moderate generalised MG, similar in pattern to most AChR-antibody-positive patients.[8][9][10][22][23][24][25][26] The clinical importance of antibodies to collagen Q and cortactin is not yet clear.

Answer 156

myasthenia gravis The disease usually presents with 1 of 3 different forms: ocular, oropharyngeal, or generalised. Approximately 50% of patients present with purely ocular symptoms (ptosis, diplopia), so-called ocular myasthenia.[19] Between 50% and 60% of those who present with purely ocular symptoms will progress to develop generalised disease, and the vast majority will do so within the first 1 to 2 years. Overall, 15% to 20% of patients will experience a myasthenic crisis (exacerbation necessitating mechanical ventilation) and it usually occurs within the first 2 years after diagnosis of MG.[20] The subset of patients with antibodies directed against muscle-specific tyrosine kinase (MuSK) present with 3 predominant phenotypes: severe faciopharyngeal weakness, with atrophy of involved muscles in long-standing disease; predominant neck and respiratory weakness with frequent progression to crisis; and clinical features undistinguishable from non-MuSK MG.[3] Using a cell-based assay, some patients with ocular MG without detectable acetylcholine receptor (AChR) antibodies have been reported to have MuSK autoantibodies.[21] The clinical phenotype of LRP4- and agrin-antibody-positive myasthenic people is not as yet clear, although in one report LRP4 patients had mild to moderate generalised MG, similar in pattern to most AChR-antibody-positive patients.[8][9][10][22][23][24][25][26] The clinical importance of antibodies to collagen Q and cortactin is not yet clear.

Answer 157

Myasthenia gravis (MG) is a chronic autoimmune disorder of the post-synaptic membrane at the neuromuscular junction (NMJ) in skeletal muscle. Circulating antibodies against the nicotinic acetylcholine receptor (AChR) and associated proteins impair neuromuscular transmission. Patients present with muscle weakness, which typically worsens with continued activity (fatigue) and improves on rest. Severity varies from isolated eye muscle weakness to generalised muscle weakness and respiratory failure requiring mechanical ventilation. A chronic autoimmune disorder of the post-synaptic membrane at the neuromuscular junction in skeletal muscle. Characterised by muscle weakness that increases with exercise (fatigue) and improves on rest. Commonly presents with drooping eyelids, double vision, oropharyngeal and/or appendicular weakness, and shortness of breath. Elevated serum acetylcholine receptor antibody titres or muscle-specific tyrosine kinase antibodies are present. Antibodies to 4 new autoantigens, low-density lipoprotein receptor-related protein (LRP4), agrin, collagen Q, and cortactin, located at the neuromuscular junction, have been identified. Clinical electrophysiology shows decremental response on repetitive nerve stimulation or increased jitter on single-fibre study. Treatments include anticholinesterases and immunotherapy. Thymectomy may be required and has been shown to be effective in people with generalised myasthenia gravis who are acetylcholine receptor (AChR) antibody positive with no thymoma. It is not yet clear at which stages of myasthenia gravis, in which patients, and in what sequence in relation to other therapies that thymectomy in non-thymoma patients should be done. Eculizumab, a novel monoclonal antibody that inhibits complement activation, has shown clinical benefit in patients with resistant generalised myasthenia gravis and AChR antibodies. Approximately 15% to 20% of patients may experience a myasthenic crisis (exacerbation necessitating mechanical ventilation). Most patients, but not all, enjoy good quality of life and normal lifespan due to advances in diagnosis and immunosuppressive and immunodulatory treatment.

Answer 158

Phaeo - remove surgically Malignant nerve sheath tumours - remove surgically +/- chemoradio ONGOING Surveillance of neurofibromas + remove surgically or debulk NB - Surgical removal may cause a peripheral neuropathy distal to the surgical disruption of the involved nerve. ``` MDT Specialist clinic Counselling Psych Genetic counselling ```

Answer 159

Patients with NF1 have reduced life expectancy due to malignancy and cardiovascular disease, particularly due to excess deaths <50 years of age. The sooner serious problems arise - such as major learning disabilities, optic pathway glioma, tibial pseudarthrosis, dystrophic scoliosis, plexiform neurofibromas - the greater the expectation for ultimate major long-term compromise.

Answer 160

Serious compromise from skeletal manifestations: Examples include spinal cord compression from dystrophic scoliosis, lower limb amputation from tibial pseudarthrosis, and ocular proptosis from sphenoid wing dysplasia.

Answer 161

neurofibromatosis

Answer 162

neurofibromatosis

Answer 163

Idiopathic Parkinson's disease (PD) is a neurodegenerative disorder. The cardinal features include resting tremor, rigidity, bradykinesia, and postural instability. Patients may demonstrate a combination of these motor symptoms, as well as other non-motor symptoms. Chronic progressive neurological disorder characterised by motor symptoms of resting tremor, rigidity, bradykinesia, and postural instability. Insidious, often asymmetrical, onset. Associated with numerous, often disabling, non-motor symptoms. Diagnosis is made clinically. Treatment is symptomatic and involves multidisciplinary care.

Answer 164

1% The mean age of onset is about 65 years.

Answer 165

Selective loss of nigrostriatal dopaminergic neurons in the substantia nigra pars compacta (SNc) occurs with findings of intracytoplasmic eosinophilic inclusions (Lewy bodies) and neurites, both of which are composed of the protein synuclein. The aetiology of PD is unknown, although several factors have been implicated. There is probably a genetic predisposition, with subsequent environmental factors/exposures contributing to the evolution of clinical disease. Within this multifactorial model, age is the only undisputed risk factor.

Answer 166

STRONG Increasing age Fix Mutation in gener encoding glucocerebrosidase ``` WEAK Chronic exposure to metals Male Head trauma Geographic influence Toxin exposure Occupation as a teacher, healthcare provider, construction worker, carpenter, or cleaner ```

Answer 167

Bradykinesia Resting Tremor Rigidity Postural instability ``` COMMON Masked facies Hypohonia Hypokinetic dysarthria Micrographia Stooped posture Shuffling gait Conjugate gaze disorders Fatigue Constipation Depression Anxiety Dementia ``` UNCOMMON Exposure to neuroleptics or anti-emetics Features of atypical Parkinson's These include acute onset, rapidly progressive disease, cognitive impairment, prominent postural instability, severe autonomic dysfunction, and significant neuropsychiatric features (i.e., hallucinations, fluctuating levels of arousal).

Answer 168

Dopamine trail: Improvement in symptoms The diagnosis of PD is made clinically, and in cases without atypical features no additional diagnostic testing is indicated. MRI.- swallow tail sign normal image in most patients with idiopathic PD; age-related changes such as mild small vessel disease acceptable; similarly acceptable if patient has appropriate history to explain other abnormalities (i.e., stroke, trauma); in advanced disease with dementia, may see cortical atrophy; dorsolateral nigral hyperintensity (the 'swallow-tail-sign') may be absent

Answer 169

When symptoms begin to interfere with the patient's quality of life or activities of daily living, treatment is initiated with a dopaminergic agent. carbidopa/levodopa: 50 mg orally (immediate-release) three times daily initially, increase by 50-100 mg/day increments every 5-7 days according to response For very mild, early symptoms, a monoamine oxidase-B (MAO-B) inhibitor (e.g., rasagiline, selegiline) may be used. Exercise should always be encouraged; it has been shown to improve functional performance on motor tasks at any stage of disease, and may have beneficial effects on cognition. Physiotherapy, occupational therapy, and speech therapy are important to treat specific symptoms, such as hypophonia and dysphagia. For patients being treated with a dopamine agonist, nausea and vomiting can be treated with domperidone.

Answer 170

Therefore, the course is progressive. Unilateral symptoms ultimately become bilateral. Rates of progression vary between patients, but the typical course is as follows: A period of 2 to 3 years when treatment with dopaminergic agents results in resolution of symptoms. After 5 years of levodopa treatment, motor complications develop. Eventually, after a number of years, the emergence of symptoms such as freezing, falling, and dementia, which do not respond to levodopa, cause significant disability. Factors to predict more rapid rate of progression are: Older age at symptom onset Rigidity/hypokinesia as presenting symptoms (versus rest tremor) Associated comorbidities Decreased response to dopaminergic medications.

Answer 171

Levodopa induced dyskinesias: The excess dopamine results in excessive movements. Management involves lowering dopaminergic medication dosages and/or lengthening dosing intervals. If this results in decreased efficacy, amantadine can be used in lieu of reducing dopaminergic medications. ``` Motor fluctuations Dementia Constipation Psychosis Depression Anxiety Impulse control disorder - If it is present, dopamine agonist should be reduced or discontinued. Appropriate psychological and social support should be provided. ```

Answer 172

Parkinson's disease PD can present in myriad ways. The cardinal features of resting tremor, bradykinesia, rigidity, and postural instability can occur in various combinations and sequences during the course of the disease. The signs and symptoms are typically asymmetrical. Bradykinesia and rigidity often present in subtle fashion early in the disease course. For example, reduced arm swing, shuffled gait, softened voice, decreased blink rate, decreased facial expressivity, and reduced spontaneous movement are all signs of parkinsonism. The non-motor symptoms of PD, such as depression, anxiety, fatigue, autonomic dysfunction (constipation, incontinence, dysphagia), and sleep disturbance, may precede the evolution of motor symptoms. Given their non-specificity, however, their relationship to PD is only made after motor symptoms/signs have been identified.

Answer 173

Due to disc compression and stenosis of the spinal canal. The syndrome consists of saddle (perineal) anaesthesia, bladder retention, and leg weakness. The reported incidence of cauda equina syndrome resulting from herniated lumbar disc varies from 1% to 15% and commonly affects males aged 40 to 60 years. Bladder dysfunction may present as incontinence but often presents earlier as difficulty starting or stopping a stream of urine. Urinary incontinence is on the basis of overflow.

Answer 174

An acute form of cord compression injury, often associated with hyper-extension injury in an individual with cervical spondylosis. It occurs more frequently among older people. Central cord syndrome is associated with greater loss of upper limb function compared to the lower limbs, including the vestibulospinal tract.

Answer 175

Results in a group of symptoms known as spinal shock, which, when seen at the high cervical level, include quadriplegia, respiratory insufficiency, loss of bladder and bowel function, anaesthesia below the affected level, and neurogenic shock (hypotension and hypothermia). Lower cervical transection spares the respiratory muscles. Horner's syndrome may be seen with higher transections due to loss of descending sympathetic pathways from the hypothalamus and consists of miosis, anhidrosis, and ipsilateral ptosis. High thoracic lesions result in paraparesis with autonomic function loss. Lower thoracic, lumbar, and sacral cord transection result in loss of bowel and bladder function.

Answer 176

Results from a hemisection lesion of the spinal cord (due to tumour or disc herniation). In the acute presentation, signs include unilateral spastic paralysis on the same side of the body, as well as ipsilateral loss of vibration and proprioception (position sense), with pain and temperature sensation being lost from the contralateral side beginning 1 or 2 segments below the lesion.

Answer 177

Occurs when the blood supply to the anterior portion of the spinal cord is interrupted. It is characterised by loss of motor function below the level of injury, loss of sensations carried by the anterior columns of the spinal cord (pain and temperature), and preservation of sensations carried by the posterior columns (fine touch and proprioception).

Answer 178

Caused by a lesion of the posterior portion of the spinal cord. It can be caused by an interruption to the posterior spinal artery. Unlike anterior cord syndrome, it is a very rare condition. It is possible for it to present as Brown-Sequard's syndrome with unilateral spastic paralysis on the same side of the body as well as ipsilateral loss of vibration and proprioception (position sense), with pain and temperature sensation being lost from the contralateral side beginning 1 or 2 segments below the lesion.

Answer 179

``` Acute spinal cord injury: Immobilisation Decompressive surgery IV corticosteroids VTE proph Prevention of gastric stress ulcers May need ventilation / feeding ``` Disc = depressive laminectomy Malignant spinal cord = surgery +/- radio Epidural abscess = Rx infection

Answer 180

The likelihood of recurrence after a traumatic spinal cord injury is estimated to be 2% to 5% Recurrence rates of malignant spinal cord compression range from 7% to 9%. Recurrence rates are reported as being from 5% to 15% but no single factor has been associated with same-level recurrence.

Answer 181

``` Pressure ulcers Discetomy complications - These complications occur in 15% to 30% of cases and include haemorrhage, soft-tissue infection, nerve root injury, dural tear, recurrent or residual disc herniation, epidural scar formation, discitis, arachnoiditis, pseudomeningocoele, facet joint fracture (iatrogenic or stress related), spinal stenosis, and epidural haematoma Post-operative autonomic dysfunction CV dysfunction Heterotopic ossification 0 The incidence of heterotopic ossification in patients with spinal cord injury (SCI) is between 16% and 53%. The incidence of clinically significant cases is between 18% and 27%. The pathophysiology involves an inflammatory process with increased blood flow in soft tissue. Bisphosphonates and/or surgery are reserved for cases with moderate pain or joint dysfunction DVT UTI PE MRSA ```

Answer 182

SCC The clinical findings of pain, sensory changes, and motor loss (including sphincter dysfunction) can be seen in most forms of spinal cord compression. However, the onset of severe back or neck pain, in the historical setting of intravenous drug use, or the historical setting of chronic, low-grade back pain, should raise a suspicion of epidural compression of the cord elements by infection. Rarely, an acute presentation will exhibit unilateral spastic paralysis on the same side of the body, as well as ipsilateral loss of vibration and proprioception (position sense), with pain and temperature sensation being lost from the contralateral side beginning 1 or 2 segments below the lesion. This is known as Brown-Sequard's syndrome and can result from a lateral cord compression (from tumour or disc herniation). An additional group of patients present after a fall occurring while the neck is extended. The result is intact motor and sensory function in the legs, and absent motor and/or sensory function in the arms. This is known as central cord syndrome.

Answer 183

SCC The clinical findings of pain, sensory changes, and motor loss (including sphincter dysfunction) can be seen in most forms of spinal cord compression. However, the onset of severe back or neck pain, in the historical setting of intravenous drug use, or the historical setting of chronic, low-grade back pain, should raise a suspicion of epidural compression of the cord elements by infection. Rarely, an acute presentation will exhibit unilateral spastic paralysis on the same side of the body, as well as ipsilateral loss of vibration and proprioception (position sense), with pain and temperature sensation being lost from the contralateral side beginning 1 or 2 segments below the lesion. This is known as Brown-Sequard's syndrome and can result from a lateral cord compression (from tumour or disc herniation). An additional group of patients present after a fall occurring while the neck is extended. The result is intact motor and sensory function in the legs, and absent motor and/or sensory function in the arms. This is known as central cord syndrome.

Answer 184

Radiculopathy, also commonly referred to as pinched nerve, refers to a set of conditions in which one or more nerves are affected and do not work properly (a neuropathy). This can result in pain (radicular pain), weakness, numbness, or difficulty controlling specific muscles

Answer 185

83 cases per 100,000

Answer 186

Radiculopathy most often is caused by mechanical compression of a nerve root usually at the exit foramen or lateral recess. It may be secondary to degenerative disc disease, osteoarthritis, facet joint degeneration/hypertrophy, ligamentous hypertrophy, spondylolisthesis, or a combination of these factors. Other possible causes of radiculopathy include neoplastic disease, infections such as shingles, HIV, or Lyme disease, spinal epidural abscess, spinal epidural hematoma, proximal diabetic neuropathy, Tarlov cysts, or, more rarely, sarcoidosis, arachnoiditis, tethered spinal cord syndrome, or transverse myelitis.

Answer 187

Most often the radiculopathy found in the patients are located in the cervical spine, most commonly affecting C6-C8 spinal nerves. Radiculopathy most often is caused by mechanical compression of a nerve root usually at the exit foramen or lateral recess. It may be secondary to degenerative disc disease, osteoarthritis, facet joint degeneration/hypertrophy, ligamentous hypertrophy, spondylolisthesis, or a combination of these factors. Other possible causes of radiculopathy include neoplastic disease, infections such as shingles, HIV, or Lyme disease, spinal epidural abscess, spinal epidural hematoma, proximal diabetic neuropathy, Tarlov cysts, or, more rarely, sarcoidosis, arachnoiditis, tethered spinal cord syndrome, or transverse myelitis.

Answer 188

LMN Sx? Pain Weakness Numbness Reduced reflexes

Answer 189

Normally clinical Spurlings test - cervical radiculopathy The Spurling test is a medical maneuver used to assess nerve root pain (also known as radicular pain). The examiner turns the patient's head to the affected side while extending and applying downward pressure to the top of the patient's head. A positive Spurling's sign is when the pain arising in the neck radiates in the direction of the corresponding dermatome ipsilaterally. Straight leg raise Femoral stretch test Reflexes may be diminished or absent If symptoms do not improve after 4-6 weeks of conservative treatment, or the person is more than 50 years old, further tests are recommended

Answer 190

Conservative treatment may include bed rest, physical therapy, or simply continuing to do usual activities; for pain, nonsteroidal anti-inflammatory drugs, nonopioid or, in some cases, narcotic analgesics may be prescribed Physio + strength training Procedures such as foraminotomy, laminotomy, or discectomy may be considered by neurosurgeons and orthopedic surgeons.

Answer 191

Cardiopulmonary support Surgical clipping or coil embolisation CCB - nimodipine: 60 mg orally every 4 hours for 21 days Stool softeners - Stool softeners to prevent straining can reduce the risk of rebleeding. There are many available, including docusate and senna. Anti-tussives - codeine phosphate: 15-30 mg orally every 6-8 hours when required, maximum 120 mg/day ANALGESIA Correct any coagulopathies Sodium replacement Fludrocortisone potentially

Answer 192

At 6 months after SAH, more than 25% of patients are dead and up to half of the survivors are moderately to severely disabled. Mortality is slightly greater in black patients and women. Causes of mortality are initial haemorrhage (19%), rebleeding (22%), vasospasm (23%), and medical complications (23%). Medical complications account for one-quarter of deaths in SAH. Forty percent of patients will have at least one medical complication during the first 3 months after SAH.

Answer 193

``` Rebleeding Acute hydrocephalus Vasospasm Cardiac abnormalities Pulmonary oedema Hyperglycaemia Seizure Fever Neuropsychiatric problems Death Chronic hydrocephalus ```

Answer 194

subarachnoid haemorrhages An atypical history of SAH includes less severe headaches, headaches accompanied by vomiting and low-grade fever, and prominent neck pain. Around 10% to 43% of patients experience a sentinel headache during the 3 months prior to SAH. Some of these headaches are caused by minor leaks from the aneurysm, which CT is unreliable in detecting. Patients who experience sentinel headache might have an increased risk of rebleeding.

Answer 195

STRONG Increased age MS WEAK Female Hypertension

Answer 196

New diagnosis - try anticonvulsant eg carbamazepine: 200 mg/day orally initially given in 1-2 divided doses, usual maintenance dose is 400-1200 mg/day given in 2 divided doses If unresponsive - baclofen: 15 mg/day orally initially given in 3 divided doses, increase according to response, maximum 80 mg/day If unresponsive - microvascular surgery: Open neurosurgical procedure where the compressive vascular loop is manually separated from the trigeminal nerve pontine entry site or ablative surgery LAst line: neurostimulation

Answer 197

Trigeminal neuralgia (TN) is a chronic affliction. There are few published data regarding the natural history of the condition, but anecdotal data suggest variable periods of remission and relapse. Most patients find at least partial relief with medical therapies or ablative procedures, although patients typically become less responsive or relapse over time. Symptomatic TN (i.e., secondary to another condition such as multiple sclerosis) appears to be less responsive to therapeutic manoeuvres. If vascular compression can be treated intraoperatively, microvascular decompression produces equal or better symptom control with durable treatment efficacy and fewer long-term sequelae.

Answer 198

Operative treatment of trigeminal neuralgia can be complicated by any of the following: hearing loss (1% to 19% in microvascular decompression), facial hypaesthesia (highest for stereotactic radiosurgery, percutaneous balloon compression, and partial sensory rhizotomy), corneal hypaesthesia, trigeminal motor weakness, anaesthesia dolorosa, keratitis, cranial nerve palsies, cerebrospinal fluid leak, meningitis, herpes labialis reactivation, and arteriovenous fistula formation.

Answer 199

trigeminal neuralgia Maxillary/mandibular (V2/V3) distribution symptoms occur in the majority of patients. This can present as sharp pain running from the mouth to the jaw (commonly mistaken for dental pain) or less commonly as pain from the upper lip to the orbit. Symptoms in an exclusively ophthalmia (V1) distribution are the least common, and patients typically refer to the pain as headache.

Answer 200

thiamine: 250-500 mg intravenously every 8 hours and magnesium sulfate: 2-4 g/day intravenously and multivitamin The dose, route, and duration of administration after the first 3 days have not been studied, but it would be prudent to continue to administer until adequate serum levels.

Answer 201

If the condition is not recognised and treated early, patients can have permanent brain injury, manifested by impairment of recent and remote memory, apathy, and confabulation, along with persistent manifestations of Wernicke's encephalopathy, including ataxia and varying degrees of ophthalmoparesis.

Answer 202

``` Ataxia and opthalmoparesis Korsakoff's psychosis Hearing loss Seizures Spastic paraparesis ```

Answer 203

Wernicke's encephalopathy In infants and children Wernicke's encephalopathy may present with non-specific symptoms, including lethargy, irritability, and vomiting. In some patients additional signs and symptoms of thiamine deficiency may be seen, such as high-output cardiac failure, peripheral neuropathy, abdominal pain, nausea, and vomiting.

Answer 204

Wernicke's encephalopathy In infants and children Wernicke's encephalopathy may present with non-specific symptoms, including lethargy, irritability, and vomiting. In some patients additional signs and symptoms of thiamine deficiency may be seen, such as high-output cardiac failure, peripheral neuropathy, abdominal pain, nausea, and vomiting.

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