▶Child:Initially 12 mg for 4 doses on days 0 , 14 , 28 and
63 , then 12 mg every 4 months, for advice on missed
doses—consult product literaturelUNLICENSED USELicensed for use in children (age range
not specified by manufacturer).
lCAUTIONSRisk factors for renal toxicity—monitor urine
protein (preferably using afirst morning urine specimen).
risk factors for thrombocytopenia and coagulation
disorders—monitor platelet and coagulation profile before
treatment
lPREGNANCYManufacturer advises avoid—no information
available.
lBREAST FEEDINGManufacturer advises avoid—no
information available.
lRENAL IMPAIRMENTManufacturer advises close
monitoring—safety and efficacy not established.
lHANDLING AND STORAGEManufacturer advises store in a
refrigerator ( 2 – 8 °C); may be stored (in the original carton,
protected from light) at or below 30 °C, for up to 14 days.
lNATIONAL FUNDING/ACCESS DECISIONS
Scottish Medicines Consortium (SMC) Decisions
SMC No. 1318/18
TheScottish Medicines Consortiumhas advised (May 2018 )
that nusinersen (Spinraza®) is accepted for restricted use
within NHS Scotland for the treatment of 5 q spinal
muscular atrophy (SMA) in patients with symptomatic
type 1 SMA (infantile onset). This advice is contingent
upon the continuing availability of the patient access
scheme in NHS Scotland or a list price that is equivalent or
lower.lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug.
Solution for injection
▶Spinraza(Biogen Idec Ltd)A
Nusinersen (as Nusinersen sodium) 2.4 mg per 1 mlSpinraza
12 mg/ 5 ml solution for injection vials| 1 vialP£ 75 , 000. 002.2 Myasthenia gravis and
Lambert-Eaton myasthenic
syndrome
ANTICHOLINESTERASES
Anticholinesterases f
lDRUG ACTIONThey prolong the action of acetylcholine by
inhibiting the action of the enzyme acetylcholinesterase.
lCONTRA-INDICATIONSIntestinal obstruction.urinary
obstruction
lCAUTIONSArrhythmias.asthma (extreme caution).
atropine or other antidote to muscarinic effects may be
necessary (particularly when neostigmine is given by
injection) but not given routinely because it may mask
signs of overdosage.bradycardia.epilepsy.
hyperthyroidism.hypotension.parkinsonism.peptic
ulceration.recent myocardial infarction.vagotonia
lSIDE-EFFECTSAbdominal cramps.diarrhoea.excessive
tearing.gastrointestinal disorders.hypersalivation.
muscle complaints.nausea.rash.vomiting
Overdose Signs of overdosage include
bronchoconstriction, increased bronchial secretions,
lacrimation, excessive sweating, involuntary defaecation,
involuntary micturition, miosis, nystagmus, bradycardia,
heart block, arrhythmias, hypotension, agitation,excessive dreaming, and weakness eventually leading to
fasciculation and paralysis.
lPREGNANCYManufacturer advises use only if potential
benefit outweighs risk.
lBREAST FEEDINGAmount probably too small to be
harmful.eiiiiFaboveNeostigmine
(Neostigmine methylsulfate)
lINDICATIONS AND DOSE
Treatment of myasthenia gravis
▶BY MOUTH
▶Neonate:Initially 1 – 2 mg, then 1 – 5 mg every 4 hours,
given 30 minutes before feeds.▶Child 1 month–5 years:Initially 7. 5 mg, dose repeated at
suitable intervals throughout the day, total daily dose
15 – 90 mg
▶Child 6–11 years:Initially 15 mg, dose repeated at
suitable intervals throughout the day, total daily dose
15 – 90 mg
▶Child 12–17 years:Initially 15 – 30 mg, dose repeated at
suitable intervals throughout the day, total daily dose
75 – 300 mg, the maximum that most patients can
tolerate is 180 mgdaily
▶BY SUBCUTANEOUS INJECTION, OR BY INTRAMUSCULAR
INJECTION
▶Neonate: 150 micrograms/kg every 6 – 8 hours, to be
given 30 minutes before feeds, then increased if
necessary up to 300 micrograms/kg every 4 hours.▶Child 1 month–11 years: 200 – 500 micrograms, dose
repeated at suitable intervals throughout the day
▶Child 12–17 years: 1 – 2. 5 mg, dose repeated at suitable
intervals throughout the day
Reversal of non-depolarising (competitive) neuromuscular
blockade
▶BY INTRAVENOUS INJECTION
▶Neonate: 50 micrograms/kg, to be given over 1 minute
after or with glycopyrronium or atropine, followed by
25 micrograms/kg if required.▶Child 1 month–11 years: 50 micrograms/kg (max. per dose
2. 5 mg), to be given over 1 minute after or with
glycopyrronium or atropine, then 25 micrograms/kg if
required
▶Child 12–17 years: 50 micrograms/kg (max. per dose
2. 5 mg), to be given over 1 minute after or with
glycopyrronium or atropine, then 25 micrograms/kg
(max. per dose 2. 5 mg) if requiredlUNLICENSED USE
▶In neonatesDose for treatment of myasthenia gravis by
subcutaneous or intramuscular injection is unlicensed.
lCAUTIONS
▶With intravenous useGlycopyrronium or atropine should
also be given when reversing neuromuscular blockade
lINTERACTIONS→Appendix 1 : neostigmine
lRENAL IMPAIRMENT
Dose adjustmentsMay need dose reduction.
lDIRECTIONS FOR ADMINISTRATIONForintravenous
injection, give undilutedordilute with Glucose 5 %or
Sodium Chloride 0. 9 %.648 Neuromuscular disorders BNFC 2018 – 2019
Musculoskeletal system10