Back Pain

Back pain is a huge problem for many patients world-wide.  The incidence in the general population is 5% and the lifetime prevalence is 60-90%.  Most of these patients have chronic or relapsing symptoms and most will not have a specific diagnosis made.

The causes of back pain are many: the main issue when seeing a patient with back pain is to differentiate between ‘organic’ and ‘non-organic’ causes and to identify any ‘red-flag’ symptoms/signs:

• Age <20 or >70
• PMHx of neoplasia or osteoporosis
• Unexplained weight loss
• Fever/rigors
• Constant pain that’s worse at night
• Acute onset of incontinence (fecal or urinary) or urinary retention
• Loss of perineal sensation
• Global and progressive weakness of the lower limbs

Differential Diagnosis

Young Adults

Mechanical back pain; prolapsed disc; spondylolisthesis; Fracture; Ankylosing Spondylitis; Infection

Middle-aged

Mechanical back pain; prolapsed disc; Spondylolisthesis; Rheumatoid Arthritis; Spinal Stenosis; Tumours; Infection

Elderly

Degenerative Spinal Disease; Spinal Stenosis; Spondylolisthesis; Osteoporosis; Neoplasia/tumours; Infection

Management (non-emergency/urgent cases)

1st line treatment remains advice and reassurance: minimal bed rest; low stress aerobic exercise and minimal disruption to activities of daily living (ADL).  Most cases of mechanical back pain will resolve within 1 month.  If pain is impeding ADLs, persists for longer than one month (assuming it is not worse and more serious pathology has been excluded) or the patient is relapsing frequently, simple regular analgesia (paracetamol +/- NSAIDs) is usually effective.  Altering the strength of analgesia should be done following the analgesic ladder.

Surgery should only be considered 3rd line in patients who are fit and able.  Much of the surgery done for back pain is not highly successful.

History

• Wash hands, introduce self, check patient name and DOB, establish rapport and gain consent
• HPC
  • Timing
    • e.g. acute onset associated with heavy lifting (mechanical)
    • acute onset after coughing/sneezing or twisting or after earlier strain (prolapsed disc/spondylolisthesis)
    • gradual onset (stenosis/tumour)
  • Radiation/associated sensory features
    • Buttocks/back of leg- sciatica (prolapsed disc/spondylolisthesis)
    • Saddle anaesthesia- Red flag for cauda equina (anterior prolapse)
    • Foot/inner thigh numbness
  • Exacerbating/alleviating factors/positions
    • e.g. walking uphill (stenosis)
  • Other symptoms
    • Fever (infection)
    • Incontinence- another red flag for cauda equina
    • Leg weakness
      • NB paralysis is another red flag
• You also want details in the rest of the history e.g. FHx back problems, Employment involving heavy lifting; previous back injury/trauma/conditions/surgery etc;

Back Examination

Neurological examination of the lower limbs- motor / sensory

Falls and Immobility- Clinical Skills

History

• Wash hands, Introduce, Check name of date of birth, opening question
• If a single fall (or regarding the worst fall):
  • Has there been loss of consciousness, injury etc and if so, how long were they fallen?  Who found/finds them?
  • Is there anything that precipitates a fall (any dizziness, any movement, any breathlessness, any funny feelings)?  Do they occur when taking your medication? Do they occur in dark places? Is it on the stairs? (Think of DAME)
    • Drugs (& toxins e.g. alcohol/medications); Age-related factors (sarcopenia, immobility); Medical conditions; Environment
  • After a fall do you feel ok?  How long?
• Re: more than one
  • How many? How often?
  • Have they been getting worse with time (more/less frequent)?
• Medical and drug history are very important?
  • Heart conditions- BP drugs? Anti-arrhythmics? Anti-coagulants?
  • Epilepsy and drugs?
  • Anti-depressants?
  • Visual disturbance?
  • Diabetes?
• Social History is also very important
  • Living situation: on their own- self-care / home-help / family; living with someone else; house environment- stairs/rails; clutter etc
  • Do they get out of the house?  Can they do daily tasks e.g. shopping; cooking; cleaning?  How do they get about? (stick/zimmer/chair/rail etc)
• NB Family Hx not so important

Conditions for admission

• cannot walk; acute illness or if they are falling so frequently that home would be unmanageable/too great a risk.

Examination

• Stance
  • Examine standing still, checking balance.  Do Romberg’s test: ask the patient to close their eyes and see if sway worsens (in cerebellar disease, patients can sway to the ipsilateral side).  Also do Trendelenburg’s test– ask the patient (with eyes open and with your support) to stand on one leg for 30s (In weakness/structural/pain problems of the hip adductors, the contralateral hemipelvis will drop)
• Gait
  • Watch the patient walk a few metres, turn and come back again.  Look from all angles.
  • Trendelenburg gait (trunchal lurch to the affected side); spastic gait (hyperextented leg); Parkinsonian gait (shuffling; slow to start; no arm swing); cerebellar ataxia (broad-based gait)
  • Do they need support?
• Examination of limbs
  • On the bed, inspect the limbs for lengthening/external rotation (hip fracture).  Also look for any other signs.
  • Feel the hip and knee joints for deformity/tenderness.
  • (On the bed, carry out Thomas’s Test: Flex both hips and knees and place your hand under the lumbar spine.  Ask the patient to extend the testing hip (A fixed flexion deformity will make this difficult).)
    • NB In practice, many (particularly elderly) patients, will have difficulty doing this.  Furthermore, as it rarely will change the management of a patient, it is no longer routinely performed, although it may still be taught and required in an OSCE
  • Test range of movement (passive and active) of the hip and knee and foot

Differential Diagnosis

Underlying causes and complications of falls are numerous, but the following associations warrant more immediate evaluation:

• Any history of or current change in alertness or level of consciousness: possible causes include cerebrovascular (transient ischemic attack, stroke, seizure), cardiovascular (hypotension, bradycardia or tachycardia), or infectious causes. [31]
• Head trauma: anticoagulation or antiplatelet therapy raise concern for a subdural hematoma. [32]
• Pain suggesting a potential fracture: persistent pain, inability to bear weight, or any obvious anatomical abnormality should prompt a quick evaluation for fracture, along with appropriate orthopedic consultation. Consideration should be given to treating osteoporosis in patients with fractures relating to low-impact falls. [33]

Neuropsychiatric

• Visual impairment: may manifest as blurred vision or diplopia.
• Peripheral neuropathy: may be accompanied by a history of diabetes or neurodegenerative disease.
• Vestibular dysfunction, particularly benign paroxysmal positional vertigo: may manifest itself as dizziness, vertigo, or imbalance.
• Gait and balance disturbance: possible history of disc disease, peripheral neuropathy, arthritis or prior injury; specific abnormality may suggest underlying disorder such as Parkinson disease.
• Fear of falling itself can be a factor in increasing the risk of falls.
• Cognitive or mood impairment: includes dementia, depression, or delirium; behavioral disturbances, functional impairments, and the use of neuroleptics may all contribute to falls.
• Seizure disorder: may have vascular, infectious or malignant causes.
• Subdural hematoma: suggested by head trauma in the presence of anticoagulation.
• Cerebrovascular accident or transient ischemic attack: focal neurologic symptoms of nontransient or transient duration, respectively.

Cardiovascular

• Syncope: for example, cardiac syncope caused by tachyarrhythmias or bradycardia, or vasovagal syncope caused by an abnormal or exaggerated autonomic response to stimuli such as standing or emotion.
• Orthostatic hypotension: suggested by positional symptoms.
• Carotid sinus syndrome: may be elicited by activities such as facial shaving.
• Postprandial hypotension: event documented based on history of observed fall coincident with meal times.

Musculoskeletal

• Joint buckling/instability: may be due to prior injury.
• Mechanical mobility/gait abnormality: patient may have prior fracture or arthritis.
• Deconditioning: insufficient exercise is common with aging.

Toxic/environmental

• Medications: especially benzodiazepines, antidepressants, and antipsychotics; others associated with an increased risk of orthostatic hypotension include alpha-blockers, antipsychotics, antihypertensives, diuretics, beta-blockers, bromocryptine, levodopa, nonsteroidal anti-inflammatory drugs, marijuana, narcotics and sedatives, hypnotics, sildenafil, tricyclic antidepressants, and vasodilators; there is some suggestion that high-dose cholecalciferol supplementation (500,000 IU once yearly) is associated with an increased risk of fall and fracture.
• Polypharmacy; use of 5 or more medications increases the risk of falls by 30% in community-dwelling people, and by at least a factor of 4 in nursing-home patients.
• Substance abuse: including alcohol (chronic misuse or acute intoxication) or OTC medications.
• Environmental hazards such as loose rugs or tiles, poor lighting, recent use of a cane or walker, or living alone: these factors are of increased importance with age.

 

Tonsillitis/Pharyngitis

Background

Inflammation of the tonsils secondary to infection.  NB Pharyngitis is inflammation of the oropharynx without tonsillar involvement.

Aetiology

• Most cases of ‘sore throat’ will be viral and will be self-limiting, non-serious disease.  However, and in children especially, 30% of cases are bacterial
  • Almost entirely Group A β-haemolytic Strep (Strep pyogenes) (other causal organisms include S aureus, S pneumoniae, M pneumoniae, Moraxella catarrhalis, H influenzae etc)

Presentation

• Sore throat, may refer to the ears
  • Red, swollen throat and tonsils
  • The patient may have noticed foul smelling breath
  • There may be a yellow-white exudate covering the tonsils
• There may be changes in voice and dysphagia may be a problem
• There may be associated lymphadenopathy of the neck.
• Abdominal pain, headache and fever may also be present.
• Centor criteria for diagnosis (3 or 4 present merits antibiotic treatment)
  • tonsillar exudate
  • tender anterior cervical lymph nodes
  • history of fever
  • absence of cough

Investigations

• Not usually necessary (unless it will alter treatment e.g. if the condition doesn’t respond to treatment)
  • I.e. Do NOT take throat swabs- these can often be misleading.  Many people are carriers for Group A Strep and others will not grow anything on culture but have clear clinical evidence of infection.
• Paul Bunnell test is usually indicated (mononucleosis/EBV- glandular fever)

Management

• According to the antibiotic man (Tayside area formulary- primary care), penicillin V (oral 1g BD or 500mg QDS for 5 days in adults; 10 days in children (dose calculated by weight in kids too) can be given to patients who fulfill 3 or more of the Centor criteria
  • But they add that this will only shorten the duration of illness by 1/7 days (on average)
  • Centor criteria (3/4 suggest GABHS tonsillitis- may benefit from antibiotics)
    • Presence of tonsillar exudate
    • Presence of tendor anterior cervical lymphadenopathy
    • History of fever
    • Absence of cough
  • Or in those with marked systemic upset; unilateral peritonsillitis; a history of rheumatic fever; an increased risk from acute infection (such as a child with diabetes mellitus or immunodeficiency)
• Alternatively, clarithromycin can be considered (500mg BD for 5 days)
• However, antibiotics should be used sparingly (not really for symptomatic relief)
  • DO NOT prescribe penicillin (particularly amoxicillin) in suspected cases of (or without ruling out) EBV as this could precipitate a maculopapular rash
• Ibuprofen (400mg TDS) and/or paracetamol can be used for symptom relief

Surgery

• SIGN recommends that surgery (tonsillectomy) be considered for patients with recurrent acute sore throats lasting at least a year
  • due to tonsillitis
  • disabling
  • seven or more significant and adequately treated sore throats in the preceding year OR 5 or more in the each of the last 2 years (10) OR 3 or more in the each of the last 3 years (9)
    • NICE suggests 5 in the last year
  • NB Surgery should be avoided, where possible, in children with sore throat. Watchful waiting is more appropriate
• NSAIDs, antiemetics (postoperatively) and a single dose of dexamethasone (at induction of anaesthetic) can be used for peri/postoperative managements of pain and nausea/vomiting (respectively)

Complications

• Quinsy- peritonsillar abscess
• Septicaemia
• Glomerulonephrities

Bacterial Meningitis

Aetiology

Commonly a complication of ENT/facial infections, cranial trauma or blood-borne infection.

Common organisms that cause meningitis are:

• Meningococcus (B + C) – Niesseria meningitidis (37% of all CABM)
• Pneumococcus – Streptococcus pneumoniae (51%)
• Haemophilus Influenza B (Hib) (rarer now vaccinated)
• Tuberculosis
• Others e.g. listeria (more common in the elderly population); Lyme disease (think travel/tic borne); cryptococcus (more common in HIV/immunocomprimised)

Pathogenesis

1. Attach/invade the pharyngeal mucosa (usually)
2. Get into the blood supply and spread
3. Cross the BBB (mechanism unclear)
4. Toxic products/ immune response causes damage

Signs/symptoms

• Fever; Stiff neck; Altered conscious level (GCS); Headache; Nausea & vomiting; Photophobia; lethargy; confusion
• Meningococcaldisease usually produces a non-blanching purpuric rash
• Papilloedema
• Kernig’s sign- painful knee extension while lying supine

NB signs and symptoms may be atypical in extremes of age or immunocomprimised individuals

Management

Admission to hospital is required if there are signs of meningeal irritation; loss of consciousness; petechial rash; febrile/unwell and fitting; known contact with meningococcal infection.

NB In the community setting- antibiotics should be given empirically, whether or not the patient will require an LP.  Meningitis can be life-threatening.

Once in hospital

• ABCDE if necessary
• Blood cultures and coagulation screen
• Give treatment as appropriate (see below)
• Disrupt and swab/aspirate any petechial or purpuric skin lesions for microscopy and culture.
• CTb and MRIb should be done in all patients with papilloedema or focal neurological signs (or other indication of raised ICP)
• Lumbar puncture

Warning signs

• Marked depressed conscious level (GCS<12) or fluctuating conscious level (ΔGCS >2)
• Focal neurological signs
• Seizure before presentation
• Shock (bradycardia/hypotension)
• Papilloedema

Treatment

• General measures
  • Elevate head >30° off bed (increases venous return and decreases ICP)
• Monitor neurological status closely; assess for pain/restlessness regularly
• Administer analgesia as required (AVOID narcotics)
• Manage in a dark, quiet room
• Assess response to treatment regularly

• Antibiotics
  • Empiric (adult)
    • IV Ceftriaxone2g BD
      • + IV Ampicillin/Amoxicillin 2g QDS if listeria is suspected (and in >55yo)
      • IV amphotericin B/ flucytosine / fluconazole if cryptococcus suspected (HIV)
      • Rifampicin, Isoniazid, Pyridostigmine and Ethambutol for TB
    • If Penicillin Allergic
      • Chloramphenicol IV 25mg/kg and Vancomycin IV 500mg QDS
      • + Co-trimoxazole if suspect listeria
  • Empiric treatment (child)
    • Ceftriaxone + amoxicillin (basically as long as there is a 3rd gen cephalosporin: ceftriaxone/cefotaxime (2nd gen: cefuroxime can be used too- more useful for resistant infections) to cover Niesseria/E coli and a penicillin to cover strep, I think you’re covered.  Gentamicin will help the action of penicillins and will provide some coverage against gram negatives BUT it has ototoxic/nephrotoxic risk so a risk/benefit decision should be made there and then.  In general, I don’t think it is first line, but as soon as cultures come back, should be added if appropriate)
      • If group B strep: benzylpenicillin and gentamicin
      • If listeria: amoxicillin and gentamicin
    • If paracetamol hypersensitive: as adult
• Steroids
  • Give to all patients suspected of bacterial meningitis:
    • 10mg IV 15-20mins BEFORE or with the first dose of antibiotic, then every 6 hours
  • Improves survival
  • Contraindicated in post-surgical meningitis; severely immunocomprimised; meningococcal/septic shock or hypersensitivity

Prognosis

• Worse if showing organ failure/distress or increasing sepsis

Acute Spinal Cord Compression

Background

• Neurological/oncological emergency
• Often underrecognised due to non-specific symptoms
• Can result in paraplegia

Aetiology

• Can be caused either by a space occupying lesion compressing the spinal cord or injury causing transection of the cord
  • Trauma is the most common cause- high impact C-spine damage
  • Tumours- extradural are usually mets; intradural are normally primaries
  • Infection- abscess (TB/staph most common)
  • Haemorrhage (trauma/bleeding disorders/anticoagulant treatment/ AVMs)
  • Oedema from venous obstruction or ischaemia from arterial obstruction (spinal stroke)
• Aetiology can be classed by site:
  • Vertebral (80%)
    • e.g. trauma; metastatic (bony) disease (most commonly from breast, prostate or bronchial)
  • Meninges (15%)
    • e.g. tumours (meningioma; neurofibroma; ependymoma; lymphoma; metastatic disease; leukaemia); epidural abscess
  • Spinal cord (intramedullary; 5%)
    • Spinal cord tumours e.g. gliomas; ependymoma or metastases

Presentation

• Onset is usually gradual (weeks) but can be acute e.g. in traumatic, metastatic or vascular causes
  • Pain (early)
    • Localised over the spine (this is a common early symptom, particularly in patients with metastatic disease- i.e. DO NOT ignore this in these patients) and/or in a radicular distribution (may be aggravated by coughing, sneezing, straining)
  • Sensory (early)
    • Paraesthesia and numbness which often begins in the lower limbs and spreads up, often to a specific level on the trunk
      • Can be loss of proprioception, light touch or pin-prick sensation
    • Can also cause a reduction in proprioception depending on extent of compression (often later than light sensation; particularly in anterior compression syndromes where the dorsal columns are the last to be affected)
  • Motor (late but common)
    • Weakness or stiffness of the limbs (lower > upper)
      • Weakness may be hard to determine if the patient is not walking prior to symptom onset
      • Can be bilateral or unilateral
  • Sphincters (late)
    • Urgency or hesitancy of micturition, progressing to urinary retention
    • Bowel constipation
• Typically, there are a mixture of upper and lower motor neuron signs
  • Below the level of compression there are usually upper motor neuron signs i.e. brisk reflexes and spasticity
  • At the level of compression there are usually lower motor neuron signs (due to compression of the nerve roots as well as spinal cord) i.e. hyporeflexia and weakness
  • Above the level of compression, signs can be normal

Signs of injury at different spinal levels

• Cervical (above C5)
  • Upper motor neuron signs in all limbs and diaphragmatic weakness (phrenic nerve affected)
• Cervical (at or below C5)
  • Lower motor neuron signs and segmental sensory loss in the arms; upper motor neuron signs in the legs
  • Respiratory (intercostal) weakness
• Thoracic
  • Spastic paraplegia with a sensory level at the trunk
  • Weakness of the legs, sacral loss of sensation and extensor (upward) plantar reflexes

Specific syndromes associated with cord compression (often traumatic/hyperacute causes)

• Cord transection i.e. complete lesion (all motor and sensory modalities affected)
  • Complete loss of motor control and sensation from the anywhere below the level affected
  • Initially a flaccid arreflexic paralysis (spinal shock) with hypotension, bradycardia and hypothermia (classic triad)
    • UMN signs later on
  • Unlikely to recover

• Brown-Sequard Syndrome (Cord hemisection- very rare but produces some classical signs to note; NB can be due to compressive lesions so may present slowly)
  • Disruption of the ipsilateral motor pathways; ipsilateral dorsal column tracts (fine touch/proprioception/reflexes) and contralateral spinothalamic tract

 

*- side of lesion; 1- hypotonic paralysis; 2- spastic paralysis and loss of vibration, proprioception and fine touch; 3- loss of pain/temperature sensation

• Central cord syndrome
  • usually caused by a hyperflexion/hyperextension injury to an already stenotic neck
  • Predominantly distal upper limb weakness; cape-like spinothalamic sensory loss (lower limb power and dorsal column sensation preserved)

NB that weakness is the distal upper limb

Investigations

• Arrange an URGENT MRI (as soon as possible)

Management

• Depends on cause
• Trauma-
  • ABCDE; Immobilise; investigate (X-ray/MRI)
  • Methylprednisolone (Must be given within 8 hours)- Bolus and 24hr infusion
  • Decompress and stabilise
• Tumours
  • Depends on tumour/patient
  • Dexamethasone as soon as possible
    • 16mg IV stat then 4mg PO QDS + PPI cover
    • Reduces vasogenic oedema
  • Radiotherapy or Surgery if clinically suitable
    • Surgery if single level involvement without widespread disease; or radio-resistant disease/previous radiotherapy to the site OR if unknown primary
    • Chemotherapy can only really be considered in rarer highly sensitive tumours
• Infection
  • Surgical drainage
  • Antimicrobial treatment- (High dose IV Ceftriaxone and Metronidazole +/- Flucloxacillin if staph aureus involvement)
• Bleeding
  • Reverse any anticoagulant and surgically decompress

Myaesthenia

Myaesthenia Gravis

Overview

• Antibody-mediated autoimmune disease against Acetyl-Choline receptors, decreasing their number at the post-synaptic neuromuscular synapse
• Can be associated with other autoimmune disease (e.g. T1DM/thyroid disease etc) and thymoma

Epidemiology

• Affects 1/10000 in UK: commoner in females <50 and males >50
• Associated with autoimmune disease (thymitis)
  • DM, hyperthyroid, Addison’s, Pernicious anaemia, RA, SLE
  • Thymic atrophy/thymoma

Clinical Features

• Ptosis; diplopia; transverse smile; bilateral facial weakness; hypophonia (on prolonged talking); fatigable weakness; reduced vital capacity
• Normal reflexes

Tests/Investigations

• Antibody
  • ACh receptor Antibody
    • 90% in generalised MG; 50% in ocular
  • Other Antibodies e.g. Anti MUSK
• Neurophysiology
  • Repetitive nerve stimulation- decremental response with ‘jittering’ on single fibre EMG
• Tensilon test (Gold standard but not routinely performed due to risk of cardiorespiratory arrest)
  • Require resus and atropine facility on site
  • 10mg edrophonium in syringe 1
  • 0.9% saline in syringe 2
  • Give 20% and observe response after 1 minute- muscle function should gradually improve (tensilon prevents AChE from breaking ACh down)
• Imaging (CXR for thymoma- about 15% will have thymoma)

Treatment

Block Acetylcholinesterase, increasing ACh concentration at the NMJ.

• Pyridostigmine (Mestinon)
  • 30-90mg 3-6 hourly PO (side effects are diarrhoea and colic)
• AND Probantheline (to prevent against side effects)
  • 15mg/8hrs (cholinergic effects- more weakness; lacrimation; sweats; meiosis; vomiting)

Treat underlying immune disease with

• Steroids: Prednisolone
  • Single does, alternate day regime
  • Increase dose to max benefit; reduce dose with remission
• Immunosuppression (Azathioprine)
  • Regular blood checks need to be done (every week for first 2 months, then monthly) to check for thiopurine methyl transferases
  • Alternatively use methotrexate
• Others e.g. plasma exchange, IVIg (these are more commonly used in acute crises)
• thymectomy

Lambert-Eaton Myaesthenic Syndrome (LEMS)

• Rare (1/100000)
• Affects proximal limbs/trunk- characteristicallystrongerwith exertion (opposite to MG); also autonomic involvement (dry mouth); hyporeflexia
• Autoimmune? (Ca Channel antibody)
• Tests include
  • CXR- often associated with SCLC
  • Voltage Gated Ca Channel Antibody test
  • EMG shows improvement with repetitive stimulation
• Treatment is with 3,4-diaminopyridine, immunotherapy and tumour treatment as appropriate

Other causes of weakness

• Remember polymyositis, botulism, takayasu’s disease, SLE, functional weakness, MND

Multiple Sclerosis

Overview

• An inflammatory, demyelinating disorder of the CNS
• Characterised by neural plaques disseminated in time and space
• More common in females (3:1); usually presents in early adulthood (20s, early 30s)

Aetiology

• Genetic predisposition (family history)
• Geography (more common the further away from the equator you live)
• Immune mediated (mechanisms are still unclear)

Clinical Course/Type

• Relapsing remitting; secondary progressive (NB nearly 60% of relapsing/remitting will become secondary progressive)
• Relapsing progressive
• Primary progressive

Clinical Features

• Visual Loss
  • Optic Neuritis
    • Painful visual loss
    • Lasts 1-2 weeks- most improve after that
    • Classic sign is an RAPD
      • On moving the penlight to the affected eye- both pupils dilate
• Pyramidal dysfunction
  • UMN dysfunction
    • Weakness; spasticity; hyperreflexia
• Sensory symptoms
  • Pain; anaesthesia; numbness; trigeminal neuralgia
  • Proprioceptive loss
• Lower urinary tract dysfunction
  • Increased tone at bladder neck
  • Detrusor hypersensitivity
  • Detrusor sphyncteric dysenergia
    • All 3 of the above cause frequency; nocturia; urgency; urge incontinence; retention
• Cerebellar/brainstem features
  • gait/vestibular problems
  • Facial weakness (+/- forehead involvement)
• Cognitive impairment

Investigations

• MRI- brain and spine
  • hyperdense plaques
  • brain atrophy (early sign)
• CSF
  • Oligoclonal bands (protein analysis)
• Neurophysiology
  • Visual evoked response
    • Electrode measures response to strobe light
    • Delayed in MS
• Bloods
  • Negative for most tests

Diagnosis

• At least two episodes demonstrating disseminating lesions in time/space
• McDonald Criteria

Treatment

Acute Treatment

• Mild attack- symptomatic tx
  • Pyramidal dysfunction
    • Weakness/spasticity
      • Physio/OT
      • Anti-spasmodics
        • Baclofen, tizanidine
        • IM botulinum
        • Intrathecal baclofen/phenol (more used for relapses with severe spasticity)
      • Nerve blocks
  • Lower Urinary Tract Dysfunction
    • Anticholinergics
      • Oxybutynin
    • Catheterisation (autonomous intermittent or permanent)
    • Desmopressin may be used to reduce the risk of over-filling symptoms (less commonly used)
    • Rarely, bladder drilling
  • Fatigue
    • Amantadine
    • Modafinil if sleepy
    • Hyperbaric oxygen
  • Sensory Symptoms
    • Anticonvulsants
      • Gabapentin
    • Antidepressants
      • Amitriptyline
    • TENS
• Moderate attacks
  • Oral prednisolone
• Severe attacks
  • IV Prednisolone

Disease modifying therapy (long-term tx)

• Interferon Beta (Avonex, Rebif, Betaseron)
  • Decrease relapse rate by 1/3
  • Decrease severity of relapses by 50%

Mode of action for interferon beta: multiple antiinflammatory actions

• Glitiramer Acetate (Copaxone)
  • Slower mode of onset (6-9 months); S/C injection
  • Similar effect on relapses as interferon but better tolerated
  • (Effect on MRI less pronounced)
• Tysabri (natalizumab)
  • 3rd line
  • Works as an antibody that antagonises integrins that allow inflammatory cells to cross the endothelium and by deactivating inflammatory cells
  • Much more effective; much more expensive
  • Possible association with prion diseases and progressive multifocal leukoencephalopathy
• Others
  • Mitoxantrone
    • Used in RPMS
    • 12 infusions over 2 years
    • Cardiotoxicity is dose related
  • Fingolimod (awaiting NICE/SMC guidance)
    • S1P modulator
    • >50% reduction in relapse rate; oral agent

Migraine

Epidemiology/Demographics

• 1 year prevalence – 10-15%
• Women > Men (2.5:1)
• On average: 1 attack/month
  • Aura in 20%; (without aura 80%)

Aura is a fully reversible visual, sensory, motor or language symptom that usually lasts 20-60 mins.  Headache usually follows <1hr later but the two can occur simultaneously.  Visual aura is the most common type.  Positive monochromatic symptoms e.g. central scomata; central fortification; hemianopic loss

Diagnosis

• This is a clinical diagnosis
  • the patient must have had at least 5 attacks lasting a total of 4-72 hours
    • 2 must have been moderate/severe, unilateral, throbbing pain, made worse with movement
    • 1 must have had some autonomic component (normally photo/phonophobia)

Pathophysiology

• Both vascular and neural influences cause migraines in susceptible individuals
  • Theory is that migraine is a primary neurogenic condition but secondary vascular changes cause worsening symptoms
    • Stress triggers changes in the brain, these changes cause release of serotonin (can be affected by sleep, diet, stress, hormones, exercise etc)
    • Blood vessels constrict and dilate
    • Chemicals including substance P irritate the nerves and blood vessels causing pain
  • Aura is caused by
    • Cortical spreading depolarisation (spread of excitation)
    • Activation of the trigeminal vascular system- dilation of cranial blood vessels
    • Release of substance P, neurokinin A and CGRP

Subtypes

• Typical
• Acephalgic (relatively rare: symptoms of migraine without headache i.e. aura, photophobia, nausea etc)
• Basilar (migraine + vertigo / dizziness / confusion / dysarthria / tingling / incoordination)
• Retinal (migraine + retinal/optic ischaemia- monocular blindness; papilloedema and retinal haemorrhage)
• Ophthalmic (migraine + extraocular palsy / dilated pupil / eye pain)
• Hemiplegic (familial vs sporadic type: rare; causes hemiplegia +/- numbness/tingling +/- speech impairment)
• Abdominal (migraine + abdo pain + anorexia/ nausea / vomiting / pallor)

Investigations

• None usually required
• If late onset (>55), known malignancy or acephalgic (i.e. just focal autonomic signs), imaging (MRIb) should be done

Treatment

• Non pharmacological
  • Set realistic goals, education, diary/triggers etc
  • Relaxation and stress management
  • Lifestyle factors : avoid triggers; healthy diet; hydration; low caffeine etc etc
• Pharmacological
  • Acute
    • NSAIDs
      • Aspirin 900mg (Naproxen 250mg; Ibuprofen 400mg are suitable alternatives and can be tried 2nd line)
      • +/- antiemetic is nausea/gastroparesis is a problem
      • Take ASAP (i.e. before aura if possible)
      • 60% significant reduction in headache at 2 hours but only 25% complete relief
    • Triptans e.g. rizatriptan > sumatriptan
      • 5-HT agonist; various modalities
      • Take at the start of headache
      • Efficacy similar to NSAIDs- but far more expensive
      • Side effects include: odd sensations e.g. tingling; heat; tightness of the throat/chest; heaviness; pressure
      • Contraindications include patients with a history of heart disease, or with significant risk factors
  • Prophylaxis
    • Consider if >3months or if very severe
    • Aim is to titrate drug to achieve lowest possible therapeutic dose
    • Must trial each for a minimum of 4 months
    • Consider non-pharmacological methods too e.g. relaxation, acupuncture (can be as effective, especially if stress is a major trigger)
      • Propanolol
        • β-blocker; reduction in migraine in around 60-80%
        • Avoid in asthma, PVD, heart failure
      • Topiramate
        • CA inhibitor; poor side effect profile (start slow)
          • Weight loss; paraesthesia; impaired concentration; enzyme inducer
      • Others e.g. amitriptyline; gabapentin; pizotifen; sodium valproate (NOT clonidinde; carbamazepine; ergotamine)

Autonomic Cephalgia and Primary Headache syndromes

The trigeminal autonomic cephalgias are a group of primary headache disorders characterised by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features e.g.

• Ptosis; miosis; Nasal stuffiness; Nausea/vomiting; Tearing; eye lid oedema

NB IMPORTANT: Anyone with new onset, unilateral cranial autonomic features requires imaging with MRIb/MRIangio to exclude vascular/mass causes that could have severe consequences

Cluster Headache

• Men>Women
• 30-40 years old
• Circadian and seasonal
• Severe unilateral headache lasting 45-90 mins (20mins – 3hrs)
  • Occurring 1-8 times/day
  • Occur in ‘clusters’ that can last weeks/months
• Treat with
  • Acutely: high flow oxygen (100%) and subcut sumatriptan (6mg)
  • Short-term: Steroids (2 weeks- reducing dose)
  • Long-term: Verapamil (prophylaxis)

Paroxysmal Hemicrania

• Women > Men
• 50-60 years old
• Severe unilateral headache with pronounced unilateral autonomic features
  • Lasting 10-30mins (2-45mins)
  • Occurring 1-40 times/day
  • i.e. shorter duration but more frequent than cluster
• Treat with indomethacin (patients usually have an absolute response)

Hemicrania continua

• Essentially the same as P/H but features are persistent
• There is an equally good response to indomethacin

Short lasting Unilateral Neuralgiform headache with Conjunctival Tearing/injection- SUNCT

• Male > Female
• Short lived (15-20 sec), unilateral neuralgiform (i.e. nerve pain) headache
  • With conjunctival tearing or injection (dominant autonomic feature)
• Treat with gabapentin

Trigeminal Neuralgia (technically not a TAC as there are rarely any autonomic features)

• Women>Men
• More common in the elderly
• Pain is triggered by touch and usually occurs in a particular facial distribution (V2 or V3)
  • Pain is severe and stabbing; brief (1-90sec) but can occur 10-100 times/day
  • Bouts of pain may last weeks/months before any sort of remission
• Treat with carbemazepine/gabapentin/phenytoin/baclofen
  • Surgery may also be used to ablate/decompress the affected nerve
• MRI is only indicated if there are focal signs, atypical features, poor response to medication or prior to surgery

Persistent Idiopathic Facial Pain (Stabbing Headache)

• This is a diagnosis of exclusion and is usually treated with indomethacin (see above table)

Weakness

CCP relating to Guillain-Barre; Myasthenia; neuropathy; Myopathy; Cord pathology; ALS; Chronic fatigue syndrome.

History

• Make sure to differentiate between focal weakness, generalised weakness and lethargy/malaise (e.g. anaemia)
  • NB Local weakness (i.e. unilateral and of specific muscle groups) suggest a neuropathy of some description (this could be radiculopathy, but could also be due to a compressive lesion/trauma or even early on in ALS)
• Ask about where the weakness is worst?
  • Proximally vs Distally vs everywhere (MG/polymyositis vs GB/myelopathy vs ?CFS)
• How long has it been going on for?
  • Hours/Days (GBS/myelopathy)
  • Longer?
• Any time/exertions make it worse?
  • Exertion (better in LES; worse in MG/polymyositis)
  • End of day (MG) vs All the time (CFS) vs start of day (CFS)
  • Do they have trouble doing ADLs?
    • Reading
    • Dressing
    • Walking
    • Washing etc etc
• Are there any other symptoms?
  • In particular pain or loss of sensation?
    • Where- dermatomal/nerve root distribution / proximally / random non-specific
  • Fatigue
    • ? Cancer syndrome; hypothyroidism; anaemia
  • Ask about problem of weakness elsewhere e.g. dysphagia, dysarthria, gait problems; and impact on daily life

The rest of the history is as expected i.e. systemic enquiry (important); PMHx; RHx/ FHx/ SHx etc.  Be sure to ask about recent illness e.g. gastroenteritis (GBS); history of cancer (LES); drugs (STATINS); family history (DMD); social history, etc etc

Examination

Nervous motor examination

See Motor examination

Also Nervous Sensory Examination

• In contrast to motor examination, begin distally and work proximally for sensation
• Before testing a mode of sensation show the patient what you are going to do by testing it on the top of the sternum with their eyes open
• Begin by using a cotton wool bud to test light touch (dorsal column).  Ask the patient to close their eyes.
  • Touch the limbs with the cotton wool beginning at the tips of the fingers/toes and then working up
  • You then want to test the dermatome regions
• Repeat with a neurotip pin, test pain (spinothalamic)
• With a 128Hz tuning fork, test vibration sense on bony prominences, again beginning distally
• Holding the joint at the sides and with the patient’s eyes initially open, demonstrate upward and downward movement of the distal finger (or other joint).  Ask the patient to close their eyes and move the joint up or down and ask the patient to tell you which way you are moving it.
  • If the patient cannot do this, move proximally until they can.

Some indicative tests of Myaesthenia

• Ask the patient to count to 100.
  • If the patient becomes dysarthric after a while, this points towards MG
• Ask the patient to look at your finger and raise it up so they have to look up at it.  Keep their gaze there for a minute or two.
  • Again, patients with MG lose the ability to hold gaze upwards and eyelids begin to droop.
• Have a general look at the patient – do they look ‘flat’?

MSK examination of the limbs/neck/back may also be required.