GP Clinical UpdateNeurology – April 2013

Dr Fiona Chadd GPST1

Dr Sneha Lupini GPST2

Case Presentation

L.F, 28 year old female

PC: Left sided weakness, dysarthria

HPC: 32/40 pregnant, recently treated for acute

pyelonephritis Presented 2/7 later with left facial droop, left arm

weakness and slurred speech Blurring around the edges of visual fields Nausea and vomiting Developed left leg weakness whilst in A+E

Further history

Obstetric Hx: G4 P2, Ante-natal scans normal, active baby

PMHx: Depression Migraine - LOV in both eyes, dizziness and photophobia

SHx: Lived with partner and 2 children Worked as a cleaner Smoker since 14yrs old, 5 cigarettes/day

Examination findings

Obs: BP 124/92, HR 118, T 37.3, RR 18, Sats 97%

CVS, respiratory and abdominal examinations unremarkable

Calves soft non tender

No neck stiffness / photophobia / rash

Cranial nerves: Visual fields grossly normal, no visual inattention / palsy Facial sensation normal Left UMN facial weakness improving No tongue/ soft palate deviation

Peripheral nervous examination:RUL LUL RLL LLL

Tone Normal Normal Normal Normal

Power 5/5 all muscle gps

3/5 elbow flexion, 2/5 in all other muscle groups

5/5 all muscle groups

4/5 all muscle groups

Co-ordination Normal Unable to perform

Normal Normal

Sensation Normal Normal Normal Normal

Reflexes Biceps +

Ticeps +

Supinator +

Biceps(cannula)

Ticeps +

Supinator +

Knee +

Ankle +

Plantars downgoing

Knee +

Ankle +

Plantars downgoing

CT head

Initial impression and plan

Impression: Right anterior circulation infarction

Plan: Aspirin 300mg OD Anti-emetics O+G r/v MRI mane Stroke team r/v

Further review

Stroke team r/v: Subtle left facial weakness Reduced sensation left side of the face Mild left arm drift and power 4/5, sensation normal

Impression: Plan: 1.?Sagittal vein thrombosis 1. MRI + MRV 2. Exclude Stroke 2. Echo

3. O+G r/v

MRI scan

Further investigations

Lumbar puncture: Glucose 3.0 Total Prot 0.19

Serology: Anti cardiolipin Ab, Ig G, A, M and protein electrophoresis

normal

Neuro r/v: ?Acute disseminated encephalomyelitis 3/7 Methylprednisolone OP VEPs and repeat MRI in 3/12

Follow upGP post natal r/v May Discussed contraception - patient felt unable to think about it,

wanted to find out what the neurologists opinion was.

Neuro clinic r/v June Left arm power improved but slower than right arm Left leg tires when walking/shopping for half hour Repeat brain and cervical spine MRI - new lesion seen (Indicates a further non clinical event confirming diagnosis of MS)

GP r/v August Presents with PV bleeding and positive pregnancy test. Scared and upset, feels unable to deal with this on top of

diagnosis of MS. Referred to EPAC.

Key Revision on Multiple Sclerosis

Types of MS Benign MS

(10% at onset) – only a few relapses in a lifetime and none remain permanent.

Relapsing/remitting MS (80% at onset) – symptoms come and go lasting 2-6 weeks on average, with 1-2 relapses per year being typical.

Secondary progressive MS (50% of R/R → progressive in 10yrs) – gradually more or worsening symptoms with fewer remissions.

Primary progressive MS (10% at onset) – from the beginning, symptoms gradually develop and worsen over time.

Risk factors, incidence and prevalence

Risk factors Not strictly hereditary but increased chance of developing if close relatives have MS.

(General population = 1/1000, primary relative with MS 1/100) 3:1 female to male ratio More common in Caucasians Commonly presents at age 30

Incidence 3-7 people per 100,000 population are

diagnosed with MS each year In England and Wales this equates to about

1800-3400 people

Prevalence 100-120 people per 100,000 population have

MS This is approximately 52,000-62,000 people in

England and Wales

Presentation of MSSecondary symptoms Contractures Urine infections Osteoporosis Muscle wasting Reduced mobility

Specific MS presentations Transverse myelitis Acute demyelinating optic neuritis

(ADON) Bilateral internuclear ophthalmoplegia

Primary symptoms

Prognosis

Variable and unpredictable outcome.

On average the degree of disability a person experiences five years after the onset on their MS is, approximately three-quarters of the expected disability at 10-15 years.

Life expectancy is normal or nearly normal (35 years after diagnosis).

Factors predicting a better outcome include: being female <30 at age of onset infrequent attacks relapsing-remitting type

50% of deaths are due to the disease process, 15% are due to suicide.

NICE Guidance CG8 - Multiple sclerosis:

Management of multiple sclerosis in primary and secondary care

November 2003

Due to be updated in 2014!

NICE Guidance - Diagnosing MS

First presentation with neurological symptoms / demyelination signs and no reasonable alternative diagnosis - a diagnosis of MS should be considered.

Second presentation requires referral to a specialist neurology service for an appointment within 6 weeks and all investigations completed and a follow up appointment within a further 6 weeks.

A diagnosis of MS should be made clinically: by a doctor with specialist neurological experience primarily on the basis of the history and examination on the basis of evidence of CNS lesions scattered in space and time

(MRI and/or visual evoked potentials)

NICE Guidance - Treatments(the same regardless of first presentation or relapse)

Acute treatment – any episode causing distressing symptoms or increased limitation of activities can be treated with high dose corticosteroids started ASAP. IV methylprednisolone 500mg –1g daily, for 3 - 5 days

or Oral methylprednisolone 500mg – 2g daily, for 3 - 5 days

Frequent (> 3x/year) or prolonged (> 3/52) use of corticosteroids should be avoided)

Treatment to reduce disease progression – All patients - Linoleic acid 17–23 g/day may reduce progression of

disability (sunflower, corn and soya). Relapsing remitting MS – Beta interferon (Avonex, Rebif and Betaferon)

and Glatiramer acetate (Copaxone) – if they can walk unaided for 100m, had 2 significant relapses in 2 years, aged 18+

Secondary progressive MS – Beta interferon – if they can walk unaided for 10m, had 2 disabling relapses in 2 years, had minimal increase in disability over the last 2 years, aged 18+

NICE Guidance – Management of MS related conditions Depression/emotionalism

Tricyclic antidepressants or SSRIs CBT

Anxiety Antidepressants or benzodiazapines

Fatigue No treatment, but small clinical benefit from Amantadine 200 mg OD Treat underlying causes (chronic pain, medication side effects, problems

sleeping)

Cognitive losses Formal cognitive assessment Depression assessment Medication review Advice about financial vulnerabilities

Visual problems – more due to loss of control of eye movement than optic neuritis Refer to optometrist for glasses If no improvement refer to ophthalmologist For nystagmus a specialist can try oral Gabapentin Adaptive technology Register as partially sighted

Neuropathic pain Carbamazepine, Gabapentin, or Amitriptyline Pain referral

Swallowing difficulties SALT assessment Advice about food consistencies and dietary intake Weight and nutritional monitoring Chest physiotherapy Short term NG feeding Long term PEG (recurrent chest infections, inadequate intake, prolonged or

distressing feeding, NG in situ for > 1/12)

NICE Guidance – Management of MS related conditions

NICE Guidance – Management of MS related conditions Urinary symptoms

Oxybutynin or Tolterodine for bladder dysfunction Desmopressin 100–400μg orally or 10–40μg intranasally nocte for nocturia or

daily control of urinary frequency when travelling Specialist referral if incontinent > 1x/week Referral to continence service if > 3 UTIs/year Intermittent catheterisation if high residual volume Long term catheter as a last resort

Bowel symptoms Constipation - dietary advice

- oral laxatives - suppositories or enemas Incontinence - likely overflow, consider constipation treatment

NICE Guidance – Management of MS related conditions MSS problems – neuro-physiotherapy referral

Muscular weakness – strengthening exercises - specialist equipment and orthoses

Spasticity and spasms – stretching exercises - Baclofen or Gabapentin

Joint contractures – stretching exercises - local Botox injections

- plaster casts- surgery

Ataxia and tremor – exercises - specialist equipment - surgery

MSS pain – TENS- antidepressants- CBT

NICE Guidance – Additional considerations

Vaccinations – patients should have influenza immunisations and all other vaccinations as normal, as relapses can be precipitated by infections.

Pregnancy - the risk of relapse decreases during pregnancy, but increases transiently postpartum.

Surgery – all types of stress can worsen MS, but stress due to operations is not proven to trigger relapses so surgical procedures should not be postponed.

AKT Questions

1. Which TWO statements regarding multiple sclerosis are correct?

a. Annual incidence in UK is 1 in 1,000,000 b. Common in temperate climates c. Efferent papillary defect noted d. Bilateral internuclear ophthalmoplegia is a typical feature of MS e. Steroids reduce frequency of attacks f. Oligoclonal bands are pathognomic of MS

2. Choose the most appropriate cause of double vision in each patient from the list below; a. Berry aneurysm e. Multiple sclerosis b. Cerebral glioma f. Myasthenia gravis c. Drug induced g. Stroked. Graves’ disease

A 35-year-old man who is a non-smoker, suddenly develops a severe headache and double vision. His right pupil is fixed and dilated.

A 27-year-old woman who is a non-smoker, suddenly develops double vision. She had an episode of reduced visual acuity in her left eye whilst on holiday 18 months previously, for which no cause was identified. She has no other significant past medical history.

A 48-year-old woman has transitory double vision towards the end of most days. She smokes 10 cigarettes/day. She has vitiligo and hypothyroidism.

3. Your Practice Manager, has been off work for the last six months, with a diagnosis of MS. She wishes to return to work and realises that getting to her upstairs office could be problematical and a whole day’s work could be overtiring. As her employer, which two decisions would be most appropriate?

a. Take her back as Practice Manager on a flexible, part-time basis b. Enforce her to take early ill-health retirement c. Revamp her working place environment d. Take her back as a Telephonist on a flexible, part-time basis e. Take her back as Practice Manager only on a full time basis f. Tell her to keep getting sickness certificates from her Doctor g. Terminate her employment