10.3 Neuromuscular disorders

First line drugs

Second line drugs

Specialist drugs

Secondary care drugs

Traffic light status (TLS) explained:

Green: Routine prescribing within licensed indication
Amber 1: specialist recommendation followed by GP initiation and continuation
Amber 2: specialist or GP initiation in line with local guideline after 1st line failure followed by GP continuation
Amber 3: specialist initiation and stabilisation followed by GP continuation
Amber SCG: specialist initiation and stabilisation followed by GP continuation in line with an agreed shared care guideline
Red: Hospital or specialist prescribing only
Double Red: These medicines have been evaluated and rejected by MKPAG and are NOT approved for use within MK. They are not recommended for use because of lack of clinical effectiveness, cost effectiveness or safety.

10.3.1 Neuroprotective Drugs

Notes:

Riluzole is used to extend life or the time to mechanical ventilation in patients with motor neurone disease (MND) who have amyotrophic lateral sclerosis (ALS).

Treatment should be initiated and stabilised by a specialist in MND for the first three months

GPs will continue monitoring the patient and prescribing riluzole thereafter. The specialist will advise the GP on the estimated duration of treatment, when to continue prescribing and when to stop. The specialist will also alert GPs to potential side effects.

Note:

Notes:

Always include the salt (i.e. quinnine sulphate) on the prescription
Start at 200mg dose.
Quinine should only be used when cramps regularly disrupt sleep
The risks should be carefully considered relative to the potential benefits
After a trial of at least 4 weeks, treatment should be stopped if there is no benefit. If treatment continues, the benefits should be assessed around every 3 months
Patients should be monitored closely during the early stages for adverse effects as well as for benefit.
Patients should be warned not to exceed the recommended dose. Serious side effects including irreversible blindness and death may occur with overdose
Thrombocytopenia is a rare but potentially life-threatening adverse reaction associated with quinine. Patients should be instructed to stop treatment and consult a physician if signs of thrombocytopenia occur, such as unexplained petechiae, bruising, or bleeding
Quinine should not be prescribed or given to patients who have previously experienced any adverse reaction to quinine, including that found in beverages

Traffic light status (TLS) explained:

Green: Routine prescribing within licensed indication
Amber 1: specialist recommendation followed by GP initiation and continuation
Amber 2: specialist or GP initiation in line with local guideline after 1st line failure followed by GP continuation
Amber 3: specialist initiation and stabilisation followed by GP continuation
Amber SCG: specialist initiation and stabilisation followed by GP continuation in line with an agreed shared care guideline
Red: Hospital or specialist prescribing only
Double Red: These medicines have been evaluated and rejected by MKPAG and are NOT approved for use within MK. They are not recommended for use because of lack of clinical effectiveness, cost effectiveness or safety.