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10.2 Drugs used in neuromuscular disorders

10.2 DRUGS USED IN NEUROMUSCULAR DISORDERS

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
           
        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.
                 RED     Nusinersen:  solution for injection 2.4 mg per 1 ml (Spinraza®)                        NHS England commissioned
 
        NICE TA755  Risdiplam is recommended as an option for treating 5q spinal muscular atrophy in people 2 months and older
                              with a clinical diagnosis of SMA types 1,2 or 3 or with pre-symptomatic SMA and 1 to 4 SMN2 copies. It is 
                              recommended only if the conditions of the managed access agreement are followed.
                  RED    Risdiplam oral solution 0.75mg/ml [Evrysdi]                                                          NHS England commissioned
 
       NICE TA748  Mexiletine is recommended, within its marketing authorisation, as an option for treating the symptoms of
                             myotonic disorders. It is recommended only if the company provides mexiletine according to the commercial
                             arrangement.
                  RED   Mexiletine capsules 167mg [Namuscla®]                                                              NHS England commissioned
 
     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
 
      Cannabis extract – Delta-9-Tetrahydrocannabinol (THC) 27mg and Cannabidiol (CBD) 25mg (Sativex®) oromucosal spray
      For symptom improvement in adult patients with moderate to severe spasticity due to multiple sclerosis (MS) who have
      not responded adequately to other anti-spasticity medication.
      This recommendation only covers the use of Delta-9-Tetrahydrocannabinol (THC) 27mg and Cannabidiol (CBD) 25mg 
      (Sativex) oromucosal spray for the indication of moderate to severe spasticity due to multiple sclerosis (MS) who have not 
      responded adequately to other anti-spasticity medication.
 
      SEE Cannabis-based medicinal products NG144
 
      AMBER     Cannabis extract – Delta-9-Tetrahydrocannabinol (THC) 27mg and Cannabidiol (CBD) 25mg (Sativex®) oromucosal 
                        spray. Specialist recommendation/initiation
                        INFORMATION FOR PRESCRIBERS: click here
 
      BLACK Traffic Light for all other indications
 
 
 
 


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