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10.2 Drugs used in neuromuscular disorders


10.2.1 Drugs which enhance neuromuscular transmission

The anticholinesterases are used as first line treatment in myasthenia gravis. Edrophonium has brief action and is used in diagnosis.

RED           Edrophonium injection 10mg/mL
AMBER     Pyridostigmine tablets 60mg
Acetylcholine-release enhancer, 3, 4 diaminopyridine (an orphan drug) has been approved 
for use in the treatment of Lambert-Eaton myasthenic syndrome (LEMS), a rare disorder of
neuromusculsr transmission.
RED          3, 4 Diaminopyridine tablets 10mg

RED     Nusinersen:  solution for injection 2.4 mg per 1 ml (Spinraza®)                                                 NHS England commissioned
NICE TA588   Nusinersen is recommended as an option for treating 5q spinal muscular atrophy (SMA) only if:
                           > people have pre-symptomatic SMA, or SMA types 1,2 or 3 and
                           > the condition is in the managed access agreement are followed.

10.2.2 Skeletal muscle relaxants

Used for the relief of chronic muscle spasm or spasticity acting principally on the CNS.
GREEN      Baclofen              
                   tablets 10mg     
                  liquid 5mg/5mL        
GREEN      Diazepam 
                   tablets 2mg, 5mg, 10mg
                   syrup 2mg/5mL
GREEN      Tizanidine
                   tablets 2mg, 4mg

 Delta-9-tetrahydrocannabinol and cannabidiol oromucosal spray (Sativex®) - Management of spasticity associated with MS. 

In line with NICE CG 186 October 2014 Sativex should no longer be offered as an option to MS patients to treat spasticity. This does not affect people who have already started treatment with Sativex in the NHS who should be able to continue treatment until they and their NHS clinician think it appropriate to stop. Clinicians are reminded for existing patients that Sativex® should only be used strictly within its UK licence for the treatment of spasticity unresponsive to a variety of available alternatives (including tricyclic antidepressants, gabapentin & baclofen). For existing patients on this traetment  Sativex® should only be prescribed by the two specialists in the treatment of neuropathic pain associated with multiple sclerosis - Dr Tidswell & Dr Shaunak (Consultant Neurologists).  GPs should not be asked to prescribe Sativex® and should be aware of the updated guidance. 

Patients who commenced treatment before NICE guidance                                                       RED         Cannabis extract (Sativex®)                                          

All other patients

BLACK       Cannabis extract (Sativex®)