Hospital Based Practice – Dysphagia. Always requires investigation to exclude malignancy. If symptoms are progressive or prolonged then urgent investigation is required. Causes.

o Mechanical blockage Malignant stricture.

Oesophageal cancer Gastric cancer Pharyngeal cancer

Benign strictures. Oesophageal web or ring Peptic stricture.

Extrinsic pressure. Lung cancer Mediastinal lymph nodes Retrosternal goitre Aortic aneurysm Left atrail enlargement.

Pharyngeal pouch. Oral

o Motility disorders Achalasia Diffuse oesophageal spasm Systemic sclerosis Myasthenia gravis Bulbar palsy Pseudobulbar palsy Syringobulbia Bulbar poliomyelitis Chagas’ disease

o Others. Oesophagitis

Infectiono Candidao Herpes simplex

Reflux Globus hystericus

Clinical features.o There are a number of key questions to ask.

1. Was it difficult to swallow liquids and solids from the start. Yes: Motility disorder. No: Stricture if solids became difficult first.

2. Is it difficult to initiate swallowing movement? If yes, suspect bulbar palsy. Especially if coughing on swallowing.

3. Is swallowing painful? If yes, suspect.

o Cancero Severe oesophagitiso Achalasiao Oesophageal spasm

4. Is dysphagia intermittent or constant, and is it getting worse? Intermittent: Oesophageal spasm Constant & worsening Suspect malignant stricture

5. Does the neck bulge or gurgle on drinking. If yes, suspect pharyngeal pouch.

Signs.o Look for

Cachecsia Anaemia Signs of systemic disease.

Systemic sclerosis CNS disease

o Examine mouth.o Feel for supraclavicular nodes.

Virchow’s node (left supraclavicular) suggests intra – abdominal malignancy.

Investigations.o FBC.

Anaemiao U&E

Dehydrationo CXR.

Mediastinal fluid level. Absent gastric bubble Aspiration

o Barium swallow ± video fluoroscopy.o GI endoscopy + biopsy.o Further investigations

Oesophageal manometry If barium swallow is normal

ENT opinion If suspected pharyngeal cause.

Diffuse oesophageal spasm. Intermittent dysphagia. Chest pain Barium swallow.

o Abnormal contraction.o Eg. Corkscrew oesophagus.

Achalasia. Failure of relaxion of lower oesophagus sphincter. Due to degeneration of myenteric plexus. Clinical picture.

o Dysphagiao Regurgitationo Substernal crampso Weihgt loss

Barium swallow.o Dilated tapering oesophagus.

Treatment.o Endoscopic balloon dilatation.o Heller’s cardiomyotomy. + PPIso Botulism toxin.

Benign oesophageal stricture. Caused by.

o GORDo Corrosiveso Surgeryo Radiotherapy.

Management.o Endoscopic balloon dilatation.

Paterson – Brown – Kelly/ Plummer – Vinson syndrome Post – cricoids web + iron – deficiency.

Anti – emetics.Receptor Antagonist NotesH1 Cyclizine GI causes

Cinnarizine Vestibular disordersD2 Metoclopramide GI causes

ProkineticDomperidones ProkineticProchlorperazine Vestibular & GI causesHaloperidol Chemical causes, eg. Opioids

5HT3 Ondansetron Can use high doses.Eg. For emetogenic chemotherapy.

Others Hyoscine butylbromide Antimuscarinic.Hence, antispasmodic and antisecretory.Don’t give with a prokinetic.

Dexamethasone Unknown mode of actionUsed as adjuvant therapy.

Midazolam Unknown action.Anti – emetic effect outlasts sedative effect.

Gastro – oesophageal Reflux Disease. Dysfunction of the lower oesophageal sphincter predisposes to acid reflux. If reflux is prolonged or excessive, it may cause:

o Oesophaitiso Benign oesophageal strictureo Barrett’s oesophagus.

Associations.o Smokingo Alcoholo Hiatus herniao Pregnancyo Obesityo Large mealso Achalasia surgery.o Drugs.

Tricyclics Anticholinergics Nitrates

o Systemic sclerosiso H. pylori

Symptoms.o Heartburn.

Burning Retrosternal discomfort. Related to meals Exacerbated by.

Lying down Stooping STraining

Relieved by antacids.o Belchingo Acid brush.

Acid or bile regurgitation.o Waterbrush.

Excessive salivation.o Odynophagia.

Painful swallowingo Nocturnal asthma.

Cough Wheeze Minimal aspiration of gastric contents.

Differential diagnoses.o Oesophagitis

Corrosives NSAIDs

o Infection. CMV Herpes Candida

o Peptic ulcero Gastric cancero Non – ulcer dyspepsia.

Complicationso Oesophagitiso Ulcerso Benign strictureo Barrett’s oesophaguso Oesophageal adenocarcinomao Iron deficiency anaemia.

Rarely.

Investigations.o Isolated symptoms don’t require investigation.o Indication for upper GI endoscopy.

Age > 55 Symptoms > 4 weeks Dysphagia Persistent symptomsin spite of treatment Relapsing symptoms Weight loss

o Barium swallow may show hiatus hernia.o 24 hour oesophageal pH monitoring ± oesophageal manometry help diagnose GORD.

Use if endoscopy is normal

o Endoscopic classification. According to Los Angeles classification. The term mucosal break describes a well demarcated area of slough or erythema

Covers old classification of erosion and ulceration.1. One or more mucosal breaks < 5 mm long.

Doesn’t extend beyond 2 mucosal folds.2. Mucosal breaks > 5 mm long.

Limited to the space between 2 mucosal fold tops.3. Mucosal break continuous between the tops of 2 or more mucosal folds.

Involves less than 75% of the oesophageal circumference.4. Mucosal break involving > 75% of oesophageal circumference.

Grading can be useful, but it is important to also document the qualitative findings of endoscopy.

Also record any complications of GORD.

Treatment.o Lifestyle.

Encourage. Weight loss Smoking cessation Small, regular meals

Avoid. Hot drinks Alcohol Eating within 3 hours of bed. Drugs reducing oesophageal motility.

o Nitrateso Anticholinergicso Tricyclicso Caclium channel blockers

Drugs that damage oesophageal mucosa.o NSAIDs.o Potassium saltso Bisphosphonates.

Raise head of bed.o Drugs.

Antacids. Magnesium trisilicate mixture (Gaviscon).

PPI If oesophagitis confirmed on endoscopy. Eg. Lanzoprazole.

o Refer for endoscopy if. Symptoms persist for > 4 weeks. Weight loss Dysphagia Excessive vomiting GI bleeding

o Prokinetic drugs. Encourage gastric emptying Eg. Metoclopramide.

o Surgery Eg. Nissen fundoscopy. Not indicated unless

Symptoms are severe Resistant to medical therapy. Severe reflux on pH monitoring.

Laparoscopic repairs are gaining favour.

Motor Neurone Disease. Caused by degeneration of neurones

o Motor cortexo Cranial nerve nucleio Anterior horn cells.

Upper and lower motor neurones are affected. No sensory loss or sphincter disturbances.

o Distinguishes from MS and polyneuropathies. No affect on external eye movements.

o Distinguishes from myasthenia gravis. Fronto – temporal dementia is seen in 10 – 35%.

Prevelence of 7:10000o Male: Female ratio of 3:2o Up to 10% of cases are familial.

Cause is unknown.o As affects anterior horn cells in the same way as polio, is thought that viruses may be

implicated.o Higher levels of oxidative damage from free radicals are found in MND brains than normal

brains. Also, MND more sensitive to free radicals causing damage than normal brian. Affect motor neurons preferentially due to

Long length free to be attacked Lower levels of protective calcium – buffering proteins

o Oxidative damage has not yet been fully shown to be the cause of MND.o No diagnostic tests, but 3 clinical patterns.

o Bulbar palsy. Palsy of.

Tongue Chewing muscles Swallowing muscles Facial muscles

Due to loss of motor nuclei function in the medulla. Signs include.

LMN lesions.o Flaccidityo Fasiculating tongue.

Like “sack of worms” Jaw jerk may be normal or absent. Speech is.

o Quieto Hoarseo Nasal

Causes. MND Gullian – Barre Polio Syringobulbia Brainstem tumours Central pontine myelinolysis

o Malnourished alcoholicso Rapid correction of hyponatraemiao Causes

Progressive and fatal quadriparesi Mutism

Dysarthria Bulbar palsy

o Recovery can occuro Amyotrophic lateral sclerosis.

50% of cases. Combined LMN wasting and UMN signs.

Loss of fine finger movement. Spasticity

o Arm flexorso Leg extensors

Hyperreflexia Upgoing plantars Clonus

Risk doubled in Gulf war veterans Possibly due to copper/ zinc superoxide dismutase (SOD1) mutations.

o Progressive muscular atrophy. 25% of cases. Anterior horn cell lesions Affect distal muscles before proximal ones. Better prognosis than ALS.

Think of MND in those > 40 with stumbling.o May be due to spastic gait or foot drop.

Also consider in unexplained aspiration pneumonia. Look for UMN signs.

o Weaknesso Spasticityo Brisk reflexeso Upgoing plantars

Look for LMN signs.o Weaknesso Muscle wastingo Fasiculation.

Tongue Abdomen Back Thighs

Consider if speech or swallow is affected. MND strongly suggested in patients with a mixture of progressive UMN and LMN signs.

o If affecting 2 limbso If affecting 1 limb and bulbar muscles.o Fasiculation alone is not enough to diagnose LMN lesion, also requires weakness.

Investigations.o Tend to rule out other differentials.o MRI of brain and cord helps eliminate structural causes.o Lumbar puncture helps eliminate inflammatory causes.o Neurophysiology helps exclude motor neuropathies.

Can detect subclinical denervation.

Treatment.o MDT approach is best due to MND rapid course and relative rarity.o Antiglutamate drugs.

Riluzole. Inhibits glutamate release. Prolongs life by about 3 months. Expensive. Be cautious in liver disease.

o Check LFTs monthly for 3 months, then 3 monthly for 9 months, then annually.

Side effects.o Vomitingo Weaknesso Tachycardiao Somnolenceo HEadahceo Dizzinesso Vertigoo Paino Raised LFTs.

o Vitamin E As antioxidant, may reducerisk of developing MND by 50%

o Anti – drooling. Propantheline Amitriptyline

o Anti – dysphagia. Pureed foods Consider NG tube or PEG.

o Anti – spasticity. As for MS.

o Respiratory failure & sleep apnoea. NIV at home may give valuable palliation.

Why do anti – glutamate drugs work in MND. Glutamate is chief excitatory neurotransmitter. MND brains tend to have higher than normal levels of glutamate.

o Reduced activity of excitatory amino acid transporter, EAAT2, which ‘mops up’ glutamate.o High levels may be neurotoxic.

Prognosiso Incurableo Often fatal within 5 years.o Median age at death in UK is 60 years.o With bulbar disease, prognosis is to survive only about 1.5 years.o Death from choking is rare/

Reassure patients that a dignified death is the norm.