George Brown:  60 year old male  Diagnosed with MND 2 years ago (58 years)  Occupation: Self-employed builder  Initial symptoms: mild weakness in left leg with foot drop .

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Amyotropic Lateral Scelrosis (ALS) Prevalence Most Common ≈ 70% Progressive Bulbar Palsy (PBL) ≈ 25% Commoner in F Both Motor neurones of CN IX – XII from medulla oblongata Progressive Muscular Atrophy (PMA) ≈ 5% LMN Brainstem and spinal cord Primary Lateral Sclerosis ≈ 0. there is no involvement of the sensory system. brainstem and spinal cord Progressive spastic Clinical tetraparesis / Features parapesis Weakness Muscle wasting Fasciculation Life 2 – 5 years Expectancy Dysarthria Dysphagia Wasting. motor nerves to the eyes and sphincters . weakness + fasciculation of lower facial muscle + tongue Reduced cough + gag reflex 6 months – 3 years Weakness Muscle Wasting Fasciculation Decreased tone + reflexes Progressive tetraparesis > 5 years Normal unless it develops into ALS Note: With all forms of MND.5% UMN Affects precentral cortex = primary motor cortex UMNL / Both LMNL Affects the lateral corticospinal tract.

SIGN Weakness Muscle Wasting Fasciculations Reflexes Tone Babinski’s Reflex Clonus > 5 * May occur mildly due to disuse UMNL  X* X ↑ ↑   LMNL    ↓ ↓ X X .

SOB and dysphagia. .Case continued • Initial symptoms: – Mild weakness in left leg with foot drop • Current symptoms and problems: – – – – Increasing pressure on his wife Reluctant to have carers Transferring George from bed to chair Admission to hospice is brought forward secondary to drooling.

• Affects are social.Drooling • Well known symptom of MND particularly when the brainstem is involved. botulinum toxin. – Non-pharmacological: SALT referral (physical therapy and positioning) Dand and Sackell. • Treatment: – Pharmacological: Antimuscarinics (eg Hyoscine hydrobromide transdermally). radiotherapy.16(11):560-4 . physical and psychological.The management of drooling in motor neurone disease. surgery. Int J PalliatNurs. 2010 Nov.

.SOB • Early stages: • Refer to specialist respiratory physician early in the disease process for regular assessment. • Later stages: • • • • Consult with palliative care physician Relieve anxiety (re-assurance and/or opiates/ benzodiazepines) improve secretion control “Early” non-invasive positive pressure ventilation decreases FVC decline.

• MDT decision .Dysphagia • 2/3 of patients with MND experience dysphagia. PEG or RIG. • Main Problems: starvation and aspiration pneumonia (major cause of death in MND!) • Food blending • Tube feeding: NG.

Common Fears • • • • • • • • Severity Progression Reversal Quality of life affected Life expectancy altered Work/ Finance Hereditary Social/ Family fears .

Fears • • • • • Choking and breathlessness Pain Cognitive changes Incontinence Loss of sexual power Ref: Death and Dying by The Motor Neurone Disease Association .

How can these fears be helped? • Build a relationship with trust for holistic care • Give appropriate time to allow people to express fears and concerns • Information giving – leaflets. support groups etc • Easy access to information • Early introduction to local hospice and services .

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