Introduction: Treatment with intrathecal baclofen (ITB) is a therapeutic option in the management of severe spasticity in patients with hereditary spastic paraparesis (HSP). However, information on the impact of ITB on the natural course of disease, especially the effect of ITB on functional parameters over time is limited. Materials and Methods: We evaluated seven patients with HSP retrospectively who were treated with an ITB device. The following parameters were measured before (pre-implantation) and after implantation (post-implantation) of the ITB device at steady state dosage of ITB and annually until last follow-up: modified Ashworth Scale, Reflex Scale, modified Rankin Scale, and Rivermead Mobility Index. The ITB dosages were assessed after reaching steady state as well as annually until last follow-up. Results: The ITB device was implanted 13 ± 6 (range 9–16) years after diagnosis of HSP on average. Severe spasticity was controlled in all patients by a mean baclofen dosage of 188 ± 60 (range 145–230) μg per day at steady state post-implantation. The modified Ashworth Scale improved significantly from 3 (interquartile range [IQR] 3–3.25) to 1 (IQR 1–1.25; p = 0.046), as did the Reflex Scale from 5 (IQR 4.75–5) to 3 (IQR 2.75–3; p = 0.046) at steady state dosage of ITB. The modified Rankin Scale improved from 2 (IQR 2–2) to 1 (IQR 1–1.5; p = 0.083) and the Rivermead Mobility Index remained 14 (IQR 13.5–14 pre-implantation, IQR 14–14 post-implantation; p = 0.18). Post-implantation, spasticity improved for 2–3 years, followed by a stable phase of ambulatory and other mobility functions for 4–5 years. Thereafter, the maintenance or progressive loss of mobility depended on individual courses of the disease. No ITB-related severe side effects occurred. Discussion: Our data further support the role of ITB in the treatment of severe spasticity in patients with deteriorated walking performance suffering HSP. ITB therapy may initially improve spasticity and stabilize mobility functions for the first 6–8 years in patients with HSP.

No abstract

ObjectiveTreatment with intrathecal baclofen (ITB) is commonly used in patients with severe spasticity. However, complications may occur after implantation of the ITB‐device, albeit mainly procedure‐ and device‐related problems. The aim of the study was to assess surgical‐ as well as catheter‐ and pump‐related complications and define their risk factors.MethodsWe retrospectively evaluated all patients with an implanted ITB‐device who were treated at the Department of Neurology, Hochzirl Hospital, Zirl, Austria, between 2006 and 2016.ResultsTwenty‐nine of 116 (25%) patients experienced 32 complications: 5 procedure‐ and 27 device‐related (4 pump‐ and 23 catheter‐associated) problems occurred. Risk factors for sustaining any complication were a spinal localization of lesion (odds ratio [OR] OR 2.71, p = .021), other catheter types than an Ascenda® catheter (OR 3.87, p = .041), a lower modified Rankin Scale (median 4 vs. 5; OR 2.86, p = .015) and a higher Barthel Index (median 53 vs. 0; OR 2.84, p = .006). The median time from the last ITB‐related surgery to the first complication was 18 (IQR 1‐57) months. Overall, 47% complications occurred within the first year after any surgical procedure regarding the ITB‐device, thereof 25% within the first month.ConclusionsProcedure‐ and device‐related complications are frequent after implantation of an ITB‐device with catheter‐associated complications as the most frequently encountered problems. Patients with a spinal origin of spasticity, a lower modified Rankin Scale and a higher Barthel Index have a higher risk to sustain a complication.

Background Neurogenic dysphagia is common and has no definitive treatment. We assessed whether pharyngeal electrical stimulation (PES) is associated with reduced dysphagia. Methods The PHAryngeal electrical stimulation for treatment of neurogenic Dysphagia European Registry (PHADER) was a prospective single-arm observational cohort study. Participants were recruited with neurogenic dysphagia (comprising five groups – stroke not needing ventilation; stroke needing ventilation; ventilation acquired; traumatic brain injury; other neurological causes). PES was administered once daily for three days. The primary outcome was the validated dysphagia severity rating scale (DSRS, score best-worst 0–12) at 3 months. Findings Of 255 enrolled patients from 14 centres in Austria, Germany and UK, 10 failed screening. At baseline, mean (standard deviation) or median [interquartile range]: age 68 (14) years, male 71%, DSRS 11·4 (1·7), time from onset to treatment 32 [44] days; age, time and DSRS differed between diagnostic groups. Insertion of PES catheters was successfully inserted in 239/245 (98%) participants, and was typically easy taking 11·8 min. 9 participants withdrew before the end of treatment. DSRS improved significantly in all dysphagia groups, difference in means (95% confidence intervals, CI) from 0 to 3 months: stroke ( n = 79) –6·7 (–7·8, –5·5), ventilated stroke ( n = 98) –6·5 (–7·6, –5·5); ventilation acquired ( n = 35) –6·6 (–8·4, –4·8); traumatic brain injury ( n = 24) -4·5 (–6·6, –2·4). The results for DSRS were mirrored for instrumentally assessed penetration aspiration scale scores. DSRS improved in both supratentorial and infratentorial stroke, with no difference between them ( p = 0·32). In previously ventilated participants with tracheotomy, DSRS improved more in participants who could be decannulated ( n = 66) –7·5 (–8·6, –6·5) versus not decannulated ( n = 33) –2·1 (–3·2, –1·0) ( p <0·001). 74 serious adverse events (SAE) occurred in 60 participants with pneumonia (9·2%) the most frequent SAE. Interpretation In patients with neurogenic dysphagia, PES was safe and associated with reduced measures of dysphagia and penetration/aspiration. Funding Phagenesis Ltd.

Objective To develop an algorithm for the selection of adults with disabling spasticity for treatment with intrathecal baclofen (ITB) and/or botulinum toxin type A (BoNT A). Methods A European Advisory Board of 4 neurologists and 4 rehabilitation specialists performed a literature review on ITB and BoNT A treatment for disabling spasticity. An online survey was sent to 125 physicians and 13 non-physician spasticity experts. Information on their current clinical practice and level of agreement on proposed selection criteria was used to inform algorithm design. Consensus was considered reached when ≥75% of respondents agreed or were neutral. Results A total of 79 experts from 17 countries completed the on-line survey (57%). Agreement was reached that patients with multi-segmental or generalized disabling spasticity refractory to oral drugs are the best candidates for ITB (96.1% consensus), while those with focal/segmental disabling spasticity are ideal candidates for BoNT A (98.7% consensus). In addition the following are good candidates for ITB (% consensus): bilateral disabling spasticity affecting lower limbs only (97.4%), bilateral (100%) or unilateral (90.9%) disabling spasticity affecting lower limbs and trunk, and unilateral or bilateral disabling spasticity affecting upper and lower extremities (96.1%). Conclusion This algorithm will support the management of adult patients with disabling spasticity by aiding patient selection for ITB and/or BoNT A treatments. LAY ABSTRACT Involuntary muscle overactivity or spasticity is the increase in muscle tone caused as a consequence of a brain or spine lesion. The muscle overactivity can become disabling spasticity and adequate treatment or combination of treatments are essential to reduce or eliminate the problems and disability caused by the involuntary muscle overactivity. An European expert consensus on the treatment of disabling spasticity, with Intrathecal Baclofen (an implanted pump that delivers baclofen directly near the spinal cord) or injection of Botulinum Toxin into muscles, was made using an online survey with 79 experts completing the survey. This algorithm supports the future management of adult patients with disabling muscle overactivity by aiding patient selection for Intrathecal Baclofen and/or Botulinum Toxin treatment.