Botox effects on kids and adults with HSP

The findings of two studies


Botox injection

Botox reduces fatigue in adults with HSP, but no improvement in mobility.


No significant improvement in mobility was found with Botox injections in a study of 33 adult HSPers, despite there being a decrease in spasticity in the adductor muscles. However there was a significant reduction in fatigue scores with the Botox.



Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients.

Little is known about the effects of Btx-A on non-motor symptoms in HSP patients.

Objective: To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients.

Methods: Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections.

Results: Mean age was 41.7 ± 13.6 years. There were 18 women and 15 men. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections.

Conclusion: Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.


SOURCE: Arq Neuropsiquiatr. 2018 Mar;76(3):183-188. doi: 10.1590/0004-282×20180013. PMID: 29809239


Botulinum toxin for hereditary spastic paraplegia: effects on motor and non-motor manifestations.

Servelhere KR1, Faber I1, Martinez A1, Nickel R2, Moro A2, Germiniani FMB2, Moscovich M2, Blume TR2, Munhoz RP2, Teive HAG2, França MC Jr1.

1 Departamento de Neurologia, Hospital de Clínicas, Universidade Estadual de Campinas, Campinas, SP, Brasil.

2 Departamento de Medicina Interna, Serviço de Neurologia, Unidade de Distúrbios do Movimento, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brasil.



Botox for treating spasticity in children

Botox useful for kids with HSP


But not for all, and occasional mild adverse effects.


Botox is a useful and safe treatment of spasticity in children with HSP, both in the short and long-term.


It improved spasticity universally and some degree of functionality in more than one third of cases.


Adverse effects are mild and occur in about 10% of cases, while small numbers had poorer scores on some of the measures with treatment.



Introduction and Objectives: BoNT-A has proved to have an important role in the treatment of spasticity produced by nonprogressive disorders, such as cerebral palsy (CP). There are few reports about the use of BoNT-A in HSP, and these are rarely about children. The objective was to investigate the results of treatment with BoNT-A in a large series of children with HSP.


Methods: The children were diagnosed using neuroimaging (magnetic resonance imaging), neurophysiologic (motor nerve conduction studies, sensory nerve conduction studies, somatosensory evoked potentials, visual evoked potentials, and transcranial magnetic stimulation) and genetic studies, and other etiologies were excluded. BoNT-A was injected using electromyography, electrostimulation, or palpation and anatomic localization, always with conscious analgesia using nitrous oxide 50%, ethyl chloride spray, or EMLA cream. All the patients were assessed at each injection and 2-3 months later, using scales of function and spasticity and a questionnaire to collect details of adverse events (AE). The Physician Rating Scale (PRS) was used to assess the type of foot support, hip adduction, and popliteal angle during gait, and the active foot dorsiflexion. The spasticity scales used were the Modified Ashworth Scale (MAS) and the Tardieu Scale.


Results: Sixty-three cases of HSP were treated. Genetic studies were done in 46. Different causal mutations were found in 15 cases (SPG4, SPG3A, SPG11, PLA2G6). There were four cases with 2 family members affected, but no mutations have been found so far; and there were 23 other sporadic cases with only partial genetic studies, but with negative results so far.


The average age at onset was 7 years (y) 5 months (mo), with reinjections every 4.8 mo on average. The data are shown in Tables 1 and 2. Ambulant (Gross Motor Function Classification System ([GMFCS]) level I or II) patients represented 63.5% of the total number of patients. The total doses/kg of body weight (bw) of BoNT-A injected by session were in the range of recommended doses in childhood spasticity but slightly lower than those used by our team in spasticity due to CP. All patients showed improved spasticity in most of the muscles. With regard to functionality, comparing the first and last examinations, 42.2% of patients improved and 4.3% had worsened foot support during gait; 35.5% improved and 5.9% had worsened adduction of the hips; 32.2% improved and 8.5% had a worsened popliteal angle; and 40.5% improved and 5.1% had worsened active foot dorsiflexion. The proportion of injections that produced AEs was 10.8%. The AEs consisted of minimal or slight tiredness or weakness that did not prevent any of the activities of daily living (80%), moderate weakness (13.8%), and significant weakness (2%). AEs lasted less than 5 days in 44% of cases and more than a week in 56%. These data indicate a good safety profile although less safe than the data that we have observed in CP (3% of AEs).


Conclusions: BoNT-A is a useful and safe treatment of spasticity in HSP, both in the short and long term. It improved spasticity in all cases and some degree of functionality in more than a third of cases. The doses injected must be carefully calculated and are lower than those for nonprogressive disorders. AEs are infrequent and mild, but must be expected in a proportion of cases.


SOURCE: Abstracts / Toxicon 123 (2016) S2eS90 


Effectiveness of botulinum toxin type a (BONT-A) in long-term treatment of hereditary spastic paraplegia in children: review of a series of 63 cases

Mar Garcia-Romero a, Mercedes Martinez-Moreno b, Samuel Ignacio Pascual-Pascual a.

a Neuropediatric Department, Hospital Universitario La Paz, IDIPAZ, Universidad Autonoma de Madrid, Madrid, Spain

b Pediatric Rehabilitation Department, Hospital Universitario La Paz, IDIPAZ, Universidad Autonoma de Madrid, Madrid, Spain


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