NEUROLOGY

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Guillain Barre syndrome (GBS)

Assessment

Subacute (worsening over days) usually symmetrical dysasthesia and weakness of the limbs to varying degrees.  Patients may have facial and bulbar weakness and respiratory failure due to weakness of the muscles of respiration. In addition patients may also suffer from autonomic dysfunction e.g. fluctuations of heart rate and blood pressure.  On examination there may be normal/ reduced tone with weakness of limbs and absent/ depressed reflexes.  There may be sensory changes. There may be weakness of facial muscles and bulbar signs.

Was there a trigger? Recent infective illness (particularly gastroenteritis), vaccination, risk of this being HIV seroconversion illness
Check bloods for - Lyme, HIV, syphilis, B12, folate, plasma viscosity, CRP, CTD screen, TFTs (before immunoglobulin)
Nerve conduction studies can be normal early in the course of the illness and if there is dubiety then repeating them at an interval may be useful.
Lumbar puncture CSF protein may be elevated (above 0.5g/L) due to inflammation of nerve roots. Protein is elevated in approximately 49% on first day and 88% after 2 weeks.  If CSF white cells are above 50 x 10/L then another diagnosis should be considered.

Diagnosis

If Guillain Barre syndrome is not the most likely diagnosis:

Depending on symptoms consider brain or spinal pathology (particularly if a sensory level, retained reflexes or bladder/bowel involvement) neuromuscular or muscular cause of weakness.
In addition there are other causes of neuropathy

If Guillain Barre syndrome is the most likely diagnosis:

Refer to neurology and carry out the treatment plan under management

Management

If there are problems with breathing, swallow or autonomic dysfunction a referral to medical high dependency should be sought (and ITU if appropriate.)

Assess breathing
  • Is the patient breathless lying flat?
  • Is their chest wall moving normally?
  • Check FVC
    • If low:
      • check arterial blood gas (type 2 respiratory failure)
      • check 4 hourly if there is 
        •  a downward trend
        • FVC below 20ml/kg
        • type 2 respiratory failure on ABG
      • then call ITU for assessment. Invasive ventilation rather than non invasive ventilation is advised.
    • If not low:
      •  then observe and repeat FVC if it is felt that respiratory muscle weakness developing. Those with rapid disease progression, bulbar symptoms, facial weakness and dysautonomia are at particular risk
  • Abnormal respiratory rate & oxygen saturation are a late sign of respiratory failure
Autonomic dysfunction

Can occur but it is uncommonly a significant issue. However be aware that patients can have:

  • Labile blood pressure
  • Tachycardia, bradycardia or arrhythmia (rarely sudden death)
  • Urinary retention (if this is the case ensure there is no cord lesion)
  • Diarrhoea or constipation
Assess swallow
  • Has patient been choking on solids or fluids?
  • Is their voice slurred?
  • Do they have respiratory failure?

If yes - SALT referral and consider NG feeding
If no - observe

Consider IV immunoglobulin (IVIg)

If IVIg is unavailable or contraindicated then plasma exchange is just as effective.
If severe symptoms - patient unable to walk unaided, respiratory or bulbar impairment. Caution required if symptoms mild, patient at high risk of thrombotic disease or kidney injury as risks may outweigh benefits.
Note IVIg is a blood product with theoretical risk of blood borne viruses.

Check immunoglobulin levels as IgA deficiency increases risk of a severe reaction to IVIg but don't delay treatment for result if rapidly progressing.

  • Dose is 0.4g/kg per day for 5 days. Eg for 75kg person the dose would be 30g per day for 5 days. See formulary
  • Daily renal function and FBC
  • Prophylactic LMWH required
Assess breathing Autonomic dysfunction Assess swallow Consider IV immunoglobulin (IVIg)
  • Is the patient breathless lying flat?
  • Is their chest wall moving normally?
  • Check FVC
    • If low:
      • check arterial blood gas (type 2 respiratory failure)
      • check 4 hourly if there is 
        •  a downward trend
        • FVC below 20ml/kg
        • type 2 respiratory failure on ABG
      • then call ITU for assessment. Invasive ventilation rather than non invasive ventilation is advised. If patients with GBS and respiratory failure are deteriorating, short term NIV is not advised as patients are likely to need ventilated for longer. It is advised that intubation is carried out electively rather than as an emergency as GBS patients with respiratory failure are at a greater risk of dysautonomia. (Rabinstein AA.  Noninvasive ventilation for neuromuscular respiratory failure: when to use and when to avoid.  Curr Opin Crit Care. 2016 Apr;22(2):94-9) (Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EFM. Anticipating Mechanical Ventilation in Guillain-Barré Syndrome. Arch Neurol. 2001;58(6):893-898)
    • If not low:
      •  then observe and repeat FVC if it is felt that respiratory muscle weakness developing. Those with rapid disease progression, bulbar symptoms, facial weakness and dysautonomia are at particular risk
  • Abnormal respiratory rate & oxygen saturation are a late sign of respiratory failure

Can occur but it is uncommonly a significant issue. However be aware that patients can have:

  • Labile blood pressure
  • Tachycardia, bradycardia or arrhythmia (rarely sudden death)
  • Urinary retention (if this is the case ensure there is no cord lesion)
  • Diarrhoea or constipation
  • Has patient been choking on solids or fluids?
  • Is their voice slurred?
  • Do they have respiratory failure?

If yes - SALT referral and consider NG feeding
If no - observe

If IVIg is unavailable or contraindicated then plasma exchange is just as effective.
If severe symptoms - patient unable to walk unaided, respiratory or bulbar impairment. Caution required if symptoms mild, patient at high risk of thrombotic disease or kidney injury as risks may outweigh benefits. 
Note IVIg is a blood product with theoretical risk of blood borne viruses.

Check immunoglobulin levels as IgA deficiency increases risk of a severe reaction to IVIg but don't delay treatment for result if rapidly progressing.

  • Dose is 0.4g/kg per day for 5 days. Eg for 75kg person the dose would be 30g per day for 5 days. See formulary
  • Daily renal function and FBC
  • Prophylactic LMWH required

Sumner CJ, Sheh S, Griffin JW, et al. The spectrum of neuropathy in diabetes and impaired glucose tolerance. Neurology 2003; 60: 108-11

Length dependent neuropathy

Presentation

Chronic 'length dependent neuropathies' is the most common neuropathy.
Presentation is in adults over 50 years old who have a symmetrical, slowly progressive neuropathy. The symptoms begin in feet and work their way upwards. There may be mild weakness of feet, dysasthesia and mild proprioceptive problems. It can be due to a number of underlying disorders. It is often due to diabetes (including impaired glucose tolerance which can cause a mild and painful neuropathy) but may be idiopathic if blood tests looking for causes prove negative.

Assessment

Nerve conduction studies in these patients are unlikely to be useful because they will show an axonal neuropathy in the vast majority (and won't explain the underlying cause of the neuropathy.)
A set of 'screening' blood tests is below. Some tests are 'general health bloods' which may point to a systemic issue causing the neuropathy rather than direct pathology. In addition (in those who may be at risk) checking HIV, syphilis and borrelia serology may be helpful.

Blood Test Looking for Outcome
Full blood count General health screen
MCV - vitamin B12 deficiency
Investigate underlying abnormality.
Renal function General health screen 
Neuropathy can be caused by chronic renal failure
Investigate underlying abnormality. Consider renal failure as cause in those with long standing renal failure
Liver function General health screen Investigate underlying abnormality. Can be associated with hepatitis B infection. Can be associated with hepatitis C infection due to cryoglobulinaemia (refer patient)
Thyroid function Hypothyroidism Treat.
Vitamin B12 Low vitamin B12 Investigate and treat. Consider trial of treatment in those with a low normal vitamin B12 level.
Immunoglobulin protein, electropheresis and plasma viscosity Monoclonal gammopathy is more common in those with peripheral neuropathy, particularly IgM paraprotein.
IgG (light chain usually lambda) associates with POEMs.
Follow local haematology guidelines. 
Refer/discuss cases if it is unclear whether result is relevant to neuropathy.
Plasma viscosity and CRP Elevated - consider vasculitis or active autoimmune condition ANA, ANCA, rheumatoid factor in those with an unexplained elevates plasma viscosity and refer patient.
Serum glucose HbAlC Diabetes Treat which may slow progression of neuropathy.
Glucose tolerance test Even 'pre-clinical diabetes' can cause a mild, usually painful neuropathy Healthy lifestyle may slow progression neuropathy and diabetes.

In addition ask

  • Drug history - some do cause neuropathies/ neuritis.
  • Family history - if there are first degree relatives with unexplained neuropathies and there are concerns this is an inherited neuropathy, refer.

Diagnosis

There are many causes of neuropathy, two ways of trying to work out the underlying cause is to look at speed of onset and the predominant problem ie weakness or sensory symptoms.

Diagnosis on the basis of symptom onset.
Acute Relapsing Subacute/Chronic
Guillian Barre syndrome
Vasculitis
Paraneoplastic sensory neuropathy
Diabetic lumbosacral radiculoplexus neuropathy (used to be known as diabetic amyotrophy)

Chronic inflammatory demyelinating polyneuropathy (CIDP)
Vasculitis
Porphyria
Vitamin B12 deficiency
Uraemic neuropathy
Neuropathy due to hypothyroidism
Neuropathy associated with paraproteins
Diabetes and impaired glucose tolerance
Hereditary neuropathies eg Charcot Marie Tooth

 

Diagnosis on basis of most prominent symptom
Pure motor weakness.
No sensory symptoms or signs on examination
Mixes sensory and motor neuropathy Predominantly proprioceptive loss
causing sensory ataxia
Predominantly pain

Multifocal motor neuropathy 
Some hereditary neuropathies

Consider other causes:
Motor Neurone Disease
Myasthenia gravis
Myopathies

Most common - see blood screening for more common causes

Paraneoplastic (check paraneoplastic antibodies and look for underlying cancer)
Sjogrens syndrome
Cisplatin toxic neuropathy
Pyridoxine toxicity
IgM paraprotein demyelinating neuropathies (check anti GD1b antibodies)
Guillian Barre syndrome uncommonly
Diabetes or impaired glucose tolerance
Vasculitic neuropathy
Guillian Barre syndrome
HIV related neuropathy
Toxic neuropathy eg arsenic
Amyloid (familial or primary)
Fabry's disease
Inherited syndromes

Referral

Refer/ discuss with neurology (or if acute issue to medical team)

  • Rapidly progressive neuropathy.
  • 'Neuropathy' which only has motor and no sensory symptoms/ signs.
  • Neuropathy where proprioceptive loss is the prominent symptom.
  • Asymmetrical neuropathy, particularly if mononeuritis multiplex is considered.
  • Patient with hepatitis C, elevated plasma viscosity or paraproteins.
  • Strong family history.

Urgent referral

Any neuropathy which is worsening on a daily or weekly basis should be referred to neurology clinic as urgent. If there is a concern the neuropathy may be Gullain Barre syndrome due to rapid worsening; refer to medical team for inpatient assessment.

Management

If a patient has neuropathic pain amitriptyline, nortriptyline, gabapentin, pregabalin or duloxetine could be trialled. See formulary and neuropathic non-malignant pain guidelines
Physiotherapy for review of mobility and consideration of aids may be useful and give an opinion as to whether aids eg foot splints may be useful for patients with foot drop.

References:
Overell J.  Peripheral neuropathy: pattern recognition for the pragmatist.  Practical neurology 2011; 11: 62-70
Sumner CJ, Sheh S, Griffin JW, et al. The spectrum of neuropathy in diabetes and impaired glucose tolerance. Neurology 2003; 60: 108-11
Headache

Presentation

First presentation of headache to clinician

If the history is suggestive of subarachnoid haemorrhage, meningitis or encephalitis, or there is papilloedema on fundoscopy or focal neurological signs on examination - contact medical team with a view for assessment.

Subarachnoid haemorrhage is characterised by a sudden, severe headache and may be associated with meningism, focal neurology or reduced GCS.
Meningitis - headache with photophobia, meningism and pyrexia/ signs of sepsis. Please remember presentation is varied and the immunocompromised and elderly may not be overtly meningitic.
Encephalitis - headache, seizures, change of behaviour, focal neurology and there may be pyrexia/signs of sepsis.

Assessment

  • Check blood pressure to ensure not due to hypertension.
  • Check CRP and plasma viscosity in those you suspect are at risk of temporal arteritis/ giant cell arteritis on basis of symptoms, past medical history and over age of 50yrs. If suspected please refer to the acute medical receiving team for assessment.
  • Refer optician for assessment in those who may have glaucoma or have optic discs which are difficult to visualise.
  • Environmental triggers - is patient sleeping well, keeping hydrated, eating regularly, drinking too much caffeine or alcohol or are they stressed?
  • Medication overuse - are they taking analgesics for headache more than 3 days per week? If yes advise to cut down and headache may worsens before it improves.
  • Request CT brain those over the age of 16 with a new headache or one whose character has changed. NICE clinical Guideline 150 (endorsed by the Royal College of Radiologists) have listed symptoms which should prompt further investigation in patients with a headache.

CT brain referral criteria

Symptoms which would suggest a patient is at high risk of pathology and would be best seen as an inpatient/ ambulatory care unit referral:

  • worsening headache with fever
  • sudden-onset headache reaching maximum intensity within 5 minutes
  • new-onset neurological deficit
  • new-onset cognitive dysfunction
  • impaired level of consciousness

Symptoms which would suggest a moderate risk of pathology and an outpatient scan may be considered:

  • change in personality
  • recent (typically within the past 3 months) head trauma
  • headache triggered by cough, valsalva (trying to breathe out with nose and mouth blocked) or sneeze
  • headache triggered by exercise
  • orthostatic headache (headache that changes with posture)
  • a substantial change in the characteristics of their headache.

Consider further investigations and/or referral for people who present with new-onset headache and any of the following:

  • compromised immunity, caused, for example, by HIV or immunosuppressive drugs
  • age under 20 years and a history of malignancy
  • a history of malignancy known to metastasise to the brain
  • vomiting without other obvious cause

If criteria are met:

Use RADIOLOGY INTERACTIVE REFERRAL FORM. 
On request write 'outpatient headache pathway' and the reason the patient fulfills the criteria.

If assistance is required to interpret the scan or advice required to manage of headache email high-uhb.neurology@nhs.net

Episodic migraine

International Headache Society definition:
Recurrent headache disorder manifesting in attacks lasting 4-72 hours. Typical characteristics of the headache are unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical activity and association with nausea and/or photophobia and phonophobia.
See:

Asking the patient to keep a headache diary can be useful to see if treatment has been effective

Trigeminal autonomic cephalalgias

Eg cluster, paroxysmal hemicrania, CUNCT, SUNA (groups of severe, stabbing, unilateral headache commonly in trigeminal distribution with autonomic features e.g. eye tearing, facial flushing etc). If description in keeping with IHS definition of paroxysmal hemicrania or hemicrania continua please give a trial of indomethacin to see if headache is eliminated. Prescribe INDOMETHACIN 75mg twice daily with a PPI for 3 weeks. If there is partial response it can be increased to up to 225mg daily. If not effective please stop.
Refer for neurology clinic appt. To discuss acute treatment before the appointment, call/ email high-uhb.neurology@nhs.net.

International Headache Society definitions: https://www.ichd-3.org/3-trigeminal-autonomic-cephalalgias/
Cluster headache - ' Attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination of these sites, lasting 15-180 minutes and occurring from once every other day to eight times a day. The pain is associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and/or eyelid oedema, and/or with restlessness or agitation .'
Paroxysmal hemicrania - 'attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination of these sites, lasting 2-30 minutes and occurring several or many times a day. The attacks are associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and/or eyelid oedema. They respond absolutely to indomethacin.'
Short-lasting unilateral neuralgiform headache attacks (includes SUNCT/ SUNA) - ' Attacks of moderate or severe, strictly unilateral head pain lasting seconds to minutes, occurring at least once a day and usually associated with prominent lacrimation and redness of the ipsilateral eye.
Hemicrania continua - ' Persistent, strictly unilateral headache, associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and/or eyelid oedema, and/or with restlessness or agitation. The headache is absolutely sensitive to indomethacin.

Chronic daily headache

Most of these are tension headache, chronic migraine or medication overuse headache. It is possible to have two chronic headache conditions simultaneously. If the headache does not fit this history a referral and further imaging should be considered.
Headache diary
Consider trial of amitriptyline, topiramate, gabapentin

Definitions: 
Chronic tension type headache
Diagnostic criteria:

  1. Headache occurring on ≥15 days per month on average for >3 months (≥180 days per year), fulfilling criteria B-
  2. Lasting hours to days, or unremitting
  3. At least two of the following four characteristics:
    1. bilateral location
    2. pressing or tightening (non-pulsating) quality
    3. mild or moderate intensity
    4. not aggravated by routine physical activity such as walking or climbing stairs
  4. Both of the following:
    1. no more than one of photophobia, phonophobia or mild nausea
    2. neither moderate or severe nausea nor vomiting
  5. Not better accounted for by another ICHD-3 diagnosis.

Chronic migraine - Headache occurring on 15 or more days per month for more than three months, which, on at least 8 days per month, has the features of migraine headache.
Diagnostic criteria:

  1. Headache (tension-type-like and/or migraine-like) on ≥15 days per month for >3 months 2 and fulfilling criteria B and C
  2. Occurring in a patient who has had at least five attacks fulfilling criteria B-D for 1.1 Migraine without aura and/or criteria B and C for 1.2 Migraine with aura
  3. On ≥8 days per month for >3 months, fulfilling any of the following 3:
    1. criteria C and D for 1.1 Migraine without aura
    2. criteria B and C for 1.2 Migraine with aura
    3. believed by the patient to be migraine at onset and relieved by a triptan or ergot derivative

Medication overuse headache - as headache which is present for ≥ 15 days per month, and which has developed or worsened during regular overuse for > 3 months of one or more acute headache drugs (any analgesic but triptans and opiates are the worst offenders.)

Headache induced by a cough, bending forward, defaecating or exercise

Many headaches can be worsened by these activities. But if it causes a headache the patient will be assessed in clinic and considered for an MRI.

Editorial Information

Last reviewed: 05 July 2019

Next review: 05 July 2021

Author(s): Consultant Neurologist

Approved By: TAM Subgroup of ADTC

Reviewer Name(s): Consultant Neurologist