Intrathecal baclofen for the management of hereditary spastic paraparesis: a systematic review.

This systematic review aims to evaluate the use of intrathecal baclofen (ITB) for hereditary spastic paraparesis (HSP) treatment. An extensive search in two electronical databases was performed. We identified articles published between 1990 and 2022 (PubMed, Scopus), and applied the following inclusion criteria: diagnosis of HSP at the time of the intervention, either familial or sporadic; report on the effect of ITB in patients with HSP; test trial via either bolus injections or continuous infusion tests; and ITB pump implantation. A data extraction sheet based on the Cochrane Consumers and Communication Review Group's data extraction template was created and adapted to collect relevant data. A qualitative analysis was performed to present the results in narrative summary fashion. A total of 6 studies met our inclusion criteria. 51 patients with HSP had a pre-implantation ITB trial. The time since the diagnosis until the pump implantation ranged from 5 to 30 years. The initial bolus ranged from 20 to 50 µg and the mean doses used at steady state ranged from 65 to 705 µg. An improvement in spasticity was observed on the modified Ashworth Scale in patients treated with ITB. Although all studies reported a subjective gait improvement, not all found an objective improvement in gait. The most common side effect reported was catheter-related problems. The findings of this review support the use of ITB as an effective and a viable option for the treatment of spasticity in HSP refractory to conservative therapies.

The use of intrathecal baclofen for management of spasticity in hereditary spastic paraparesis: A case series.

Hereditary Spastic Paraparesis (HSP) causes lower limb spasticity, pain and limits ambulation resulting in a negative impact on an individual's quality of life. This case series evaluates the use of Intra-thecal Baclofen (ITB) on 5 ambulant children with HSP. Our results suggest ITB is associated with a reduction in spasticity and a trend towards improvement in patient-reported quality of life and achievement of personalised goals. This was evidenced with lower Modified Ashworth Scale (MAS) scores and increasing values using the Cerebral Palsy Quality of Life (CPQoL) tool and Goal Attainment Scale (GAS). ITB was not associated with any major immediate or longer-term adverse effects. Overall, our study supports the role of ITB, used in a goal-directed manner, in the management of children and young people with HSP where other standard treatment options have been unsuccessful.

Oculo-dento-digital Dysplasia Presenting as Spastic Paraparesis Which Was Successfully Treated by Intrathecal Baclofen Therapy.

A 42-year-old man with a history of migraine and bilateral syndactyly presented with numbness of the extremities and shaking legs, which thus prevented him from working as a carpenter. A neurological examination revealed spastic paraparesis with pathological reflexes on all four extremities. Oculo-dento-digital dysplasia (ODDD) was suspected based on his medical history and characteristic facial appearance including small eye slits, thin mouth, and pinched nose with anteverted nostrils. Genetic tests revealed a gap junction alpha 1 (GJA1) gene mutation and confirmed the diagnosis of ODDD. His spastic paraparesis was resistant to oral antispastic medication, however, his symptoms successfully improved after the initiation of intrathecal baclofen therapy, which thus allowed him to return to work.

[Intrathecal baclofen therapy in brain injury and recovery of persistent vegetative state. Apropos of a case]. / Baclofeno intratecal en daño cerebral y recuperación de estado vegetativo persistente. A propósito de un caso.

Patients with brain injury and spasticity are candidates for intrathecal baclofen therapy (ITB) when maximal doses of oral antispastic drugs fail. Some authors have described an improvement in the level of consciousness in patients with brain injury and disorder of consciousness treated with ITB for spasticity. We present the case of a 43-year-old patient with brain injury, spasticity, and permanent vegetative state (PVS) who showed an improvement in the level of consciousness after ITB for spasticity. We performed an ITB infusion test, assessing the spasticity with the Modified Ashworth Scale (MAS) and level of consciousness with the Coma Recovery Scale-Revised (CRS-R) and observed an improvement in the spasticity and the level of consciousness. Consequently, the ITB pump was implanted and the patient recovered from PVS to minimal conscious state (MCS). We conclude that ITB is indicated in patients with brain injury and spasticity. We suggest the improvement in the level of consciousness as a possible additional benefit. There is a lack of evidence to recommend ITB in patients with altered level of consciousness.

Eficácia e segurança da toxina botulínica no tratamento da paraparesia espástica: revisão sistemática / Efficacy and safety of botulinum toxin in the treatment of spastic paraparesis: systematic review

A paraparesia espástica é caracterizada pela perda de função total ou parcial dos membros inferiores associado ao aumento do tônus muscular velocidade-dependente. A toxina botulínica é utilizada no tratamento de diversos padrões de espasticidade, sejam em flexão, extensão ou adução. Objetivo: determinar a eficácia e segurança do bloqueio químico com toxina botulínica em pacientes com paraparesia espástica. Método: foi realizada uma revisão sistemática com busca nas bases de dados do PUBMED, MEDLINE, LILACS e SCIELO. Os critérios de inclusão foram: ensaios clínicos que utilizaram a toxina botulínica para o tratamento de pacientes com paraparesia espástica e publicados em inglês a partir da década de 1980. Os desfechos considerados foram: a pontuação na Escala de Ashworth Modificada, a amplitude de movimento passiva e ativa e os efeitos adversos da toxina botulínica. Resultados: foram incluídos cinco artigos. Todos mostraram melhora da espasticidade nos pacientes estudados. Quatro artigos mostraram aumento da amplitude de movimento passivo e três relataram aumento da amplitude de movimento ativo. Três artigos trouxeram relatos de efeitos adversos após o uso da toxina botulínica, mas a maioria deles não eram graves e cessaram espontaneamente. Conclusão: os estudos analisados mostraram que a toxina botulínica é eficaz e segura em pacientes com paraparesia espástica.(AU)

Spastic paraparesis is the loss of total or partial lower limb function associated with increased speed-dependent muscle tone. Botulinum toxin is used in the treatment of several spasticity presentations that include flexion, extension and adduction. Objective: To determine both safety and efficacy of botulinum toxin as a blocking agent in the treatment of spastic paraparesis. Method:A systematic review was carried out with a search on PUBMED, MEDLINE, LILACS and SCIELO databases. The inclusion criteria were: clinical trials that used botulinum toxin for the treatment of patients with spastic paraparesis and published in English from the 1980s. The following outcomes were assessed by the studies: the Ashworth Modified scale score, the range of passive and active motion and botulinum toxin adverse effects. Results:Five articles were included. All of them showed spasticity improvements in the patients. Four studies showed increases in passive range of motion and three articles showed increase in active range of motion. Three papers reported adverse effects after botulinum toxin use but they were mostly mild and ceased spontaneously. Conclusion: Most analyzed studies indicated that botulinum toxin is safe and efficient inthe treatment of spastic paraparesis. (AU)

A comprehensive person-centered approach to adult spastic paresis: a consensus-based framework.

Spastic paresis is a common feature of an upper motor neuron impairment caused by stroke, brain injury, multiple sclerosis and other central nervous system (CNS) disorders. Existing national and international guidelines for the treatment of adult spastic paresis tend to focus on the treatment of muscle overactivity rather than the comprehensive approach to care, which may require life-long management. Person-centered care is increasingly adopted by healthcare systems in a shift of focus from "disease-oriented" towards "person-centered" medicine. The challenge is to apply this principle to the complex management of spastic paresis and to include an educative process that engages care providers and patients and encourages them to participate actively in the long-term management of their own disease. To address this issue, a group of 13 international clinicians and researchers used a pragmatic top-down methodology to evaluate the evidence and to formulate and grade the strength of recommendations for applying the principles of person-centered care to the management of spastic paresis. There is a distinct lack of clinical trial evidence regarding the application of person-centered medicine to the rehabilitation setting. However, the current evidence base supports the need to ensure that treatment interventions for spastic paresis should be centered on as far as reasonable on the patient's own priorities for treatment. Goal setting, negotiation and formal recording of agreed SMART goals should be an integral part of all spasticity management programs, and goal attainment scaling should be recorded alongside other standardized measures in the evaluation of outcome. When planning interventions for spastic paresis, the team should consider the patient and their family's capacity for self-rehabilitation, as well as ways to enhance this approach. Finally, the proposed intervention and treatment goals should consider the impact of any neuropsychological, cognitive and behavioral deficits on rehabilitation. These recommendations support a person-centric focus in the management of spastic paresis.

Uso compasivo de cannabidiol en espray para el tratamiento de paraparesia espástica en un paciente con adrenomieloneuropatía / Compassionate use of cannabidiol in spray form for the treatment of spastic paraparesis in a patient with adrenomyeloneuropathy

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[Compassionate use of cannabidiol in spray form for the treatment of spastic paraparesis in a patient with adrenomyeloneuropathy]. / Uso compasivo de cannabidiol en espray para el tratamiento de paraparesia espastica en un paciente con adrenomieloneuropatia.

TITLE: Uso compasivo de cannabidiol en espray para el tratamiento de paraparesia espastica en un paciente con adrenomieloneuropatia.

Serial Casting as an Adjunct to Botulinum Toxin Type A Treatment in Children With Cerebral Palsy and Spastic Paraparesis With Scissoring of the Lower Extremities.

The purpose of this study was to examine whether combination therapy of serial casting and botulinum toxin type A injection can further enhance the effects of botulinum toxin type A in children with cerebral palsy with scissoring of both legs. This study was a prospective and randomized trial. The children were divided into 2 groups, one of which received serial casting after botulinum toxin type A (n = 40), and the other which only received botulinum toxin type A (n = 40). Serial casting started 3 weeks after the botulinum toxin type A. Both groups received physiotherapy. Groups were assessed at baseline then compared at 6 and 12 weeks following the intervention. Significant improvements in Gross Motor Function Measure-66 and Caregiver Health Questionnaire were recorded in both groups ( P < .001). The modified Ashworth scale improved significantly following botulinum toxin type A in the serial casting group ( P < .05), but not in botulinum toxin type A only group. These results suggest that serial casting after botulinum toxin type A can enhance the benefits of botulinum toxin type A in children with cerebral palsy.

Longstanding spastic paraparesis in a patient infected with hepatitis C virus and seropositive for aquaporin-4 antibody - Case report and review of the literature.

Nervous system involvement in Hepatitis C virus infection (HCV) has been associated to neuro-immunological deregulation, particularly in interferon-alpha treated patients. We present a case of optic and brainstem demyelinating disorder associated with aquaporin-4 (AQP4) antibodies. A 48 year-old woman, with previous diagnosis of non-treated hepatitis C, presented with a 10-year history of long-standing gait disturbance. Neurological examination disclosed a grade 4 spastic paraparesis, lower limb hyperreflexia, right positive Hoffmann sign, bilateral Babinski sign and spastic gait only possible with bilateral support. Spinal cord magnetic resonance imaging (MRI) was normal. Brain MRI showed an asymmetric, bilateral pontine and left mesencephalic hypersignal in T2 and FLAIR, with no gadolinium enhancement. Visual evoked potential revealed bilateral pre-chiasmatic conduction delay. Blood tests showed a positive anti-HCV antibody and a positive AQP4 antibody. Cerebrospinal fluid (CSF) analysis was normal, with no oligoclonal bands. The patient started intravenous (IV) methylprednisolone followed by oral prednisolone; simultaneously, interferon-alpha and ribavirin. There was a slight clinical improvement within the first weeks. There are 7 cases describing association between HCV infection and central nervous system (CNS) demyelination with positive AQP4 antibody, 4 patients under interferon-α. AQP4 antibodies should be tested in patients infected with HCV and CNS demyelination.

Subfascial expansive plasty for intrathecal baclofen pump replacement. A technical note.

PURPOSE: Although intratechal pump replacement is a common technique, it can be sometimes complicated when aiming to replace a 20 ml pump with a bigger one (40 ml). We developed a simple and straightforward technique to relax the wall of the pocket of the pump, preserving its fascial layer. METHODS: A 20-year-old boy with spastic tetraparesia was admitted for pump replacement. After scar opening and pump removal, various lineal incisions were performed in the posterior layer of the subfascial pocket in a cranio-caudal direction. RESULTS: A 40 ml pump was placed without skin incision ampliation and preserving subfascial plane. CONCLUSIONS: This easy, expansive technique for infusion pump replacement preserves subfascial plane and prevents the need for more extensive surgeries.

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl.

Neuromyelitis optica (NMO) and myasthenia gravis (MG) are rare autoimmune disorders. The coexistence of the two disorders, although rare, has been documented. This is a case report of a 16-year-old student who presented with recurrent episodes of transverse myelitis and optic neuritis, 8 years after diagnosis of MG. She presented with visual impairment, relapsing and remitting weakness, numbness and paraesthesia of her lower limbs, with bladder and bowel incontinence. Her examination revealed bilateral optic atrophy, spastic paraparesis of the lower limbs and patchy sensory loss up to thoracic level (T4-5). She had a positive acetylcholine receptor antibody, a positive aquaporin-4 antibody and chest CT finding of thymic enlargement. We therefore confirmed the previous diagnosis of MG and performed a recent diagnosis of background NMO. A high index of suspicion is needed to make a diagnosis of this rare coexistence of NMO and MG in resource-limited settings such as Nigeria.

Paraparesia espástica progresiva y siringomielia estática: síndrome de Silver/SPG17 / Progressive spastic paraparesis and static syringomyelia: Silver syndrome/SPG17

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Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).

Disappearance of white matter lesions on MRI and clinical recovery after initiating antiretroviral therapy in a case of HIV infection presenting as spastic paraparesis.

We present a case of a 30-year-old Polish female who presented with increasing for about 2 years spastic paraparesis and urinary incontinence. She denied any risky sexual behaviors, drug abuse, there was no history of surgery or blood transfusions. MRI of the brain showed diffuse, hyperintensive in T2, poorly defined lesions in the white matter. About 3 months later paraparesis increased and control MRI showed progression of previously described lesions. She was then diagnosed with HIV infection. There was a suspicion of progressive multifocal leucoencephalopathy (PML) or vacuolar myelopathy in the course of HIV infection. Antiretroviral treatment was initiated leading, together with rehabilitation, to a progressive improvement of symptoms. Pathological lesions on brain MRI completely disappeared. In conclusion, HIV test should be done in every patient with neurological signs of unknown cause.

Hyperargininemia: 7-month follow-up under sodium benzoate therapy in an Italian child presenting progressive spastic paraparesis, cognitive decline, and novel mutation in ARG1 gene.

BACKGROUND: Hyperargininemia due to mutations in ARG1 gene is an autosomal recessive inborn error of metabolism caused by a defect in the final step of the urea cycle. Common clinical presentation is a variable association of progressive spastic paraparesis, epilepsy, and cognitive deficits. METHODS: We describe the clinical history of an Italian child presenting progressive spastic paraparesis, carrying a new homozygous missense mutation in the ARG1 gene. A detailed clinical, biochemical, and neurophysiological follow-up after 7 months of sodium benzoate therapy is reported. RESULTS: Laboratory findings, gait abnormalities, spastic paraparesis, and electroencephalographic and neurophysiological abnormalities remained quite stable over the follow-up. Conversely, a mild cognitive deterioration has been detected by means of the neuropsychologic assessment. CONCLUSIONS: Further longitudinal studies by means of longer follow-up and using clinical, biochemical, radiological, and neurophysiological assessments are needed in such patients to describe natural history and monitor the effects of treatments.

Potential beneficial effects of granulocyte colony-stimulating factor therapy for spastic paraparesis in a patient with kyphoscoliosis: a case report.

Congenital kyphosis and kyphoscoliosis are much less common than congenital scoliosis and more serious because these curves can progress rapidly and can lead to spinal cord compression and paraplegia. A 15-year-old boy presented with congenital kyphoscoliosis along with spastic paraparesis (American Spinal Injury Association Impairment Scale grade C). We examined the safety and effectiveness of a low dose of analog granulocyte colony-stimulating factor (G-CSF) in this patient. G-CSF 5 µg/kg was given subcutaneously, daily for 5 days per month for 3 months. Laboratory tests, including blood, biochemical tests, and CD34+ cells (marker hematopoietic progenitor cells) were performed, in addition to clinical examination. Clinical examination revealed an increase of muscle strength in the upper limbs and decrease spasticity in the lower limbs between baseline and day 90 and day 180. We found no serious adverse event, drug-related platelet reduction, or splenomegaly. Leukocyte levels remained below 21,000/µL. CD34+ increased significantly at day 5 of G-CSF administration. Low-dose G-CSF was safe and well tolerated by the patient. A significant increase in muscle strength in this patient with spastic paraparesis after 3 months of treatment may indicate beneficial effects of G-CSF factor in this disorder. These results are inspiring and warrant further studies.

Systematic review of the effectiveness of pharmacological interventions in the treatment of spasticity of the hemiparetic lower extremity more than six months post stroke.

BACKGROUND: The long-term management of lower limb spasticity after stroke is an important aspect of an individual's physical recovery and quality of life. OBJECTIVE: To examine the effectiveness of pharmacological interventions in reducing spasticity of the lower limb in chronic stroke survivors. METHODS: PubMed, CINAHL, and EMBASE were searched for studies in which (1) ≥50% of the sample size had sustained a stroke; (2) the research design was a randomized controlled trial (RCT); (3) the mean time since stroke was ≥6 months for both the treatment and control groups, at the time treatment was initiated; (4) the treatment group received a pharmacological intervention aimed at treating lower limb spasticity; and (5) spasticity was assessed pre and post treatment. Methodological quality of each study was assessed using the PEDro tool. RESULTS: Nine RCTs (PEDro scores, 4-9) met inclusion criteria and included a pooled sample size of 605 individuals with a mean age of 54.8 years (range, 14-86). Four RCTs provided evidence that botulinum toxin type A was effective in reducing spasticity compared to persons receiving placebo or a phenol neurolytic. One study provided evidence that both alcohol and phenol neurolytics were effective in reducing spasticity. Finally, 4 studies provided evidence that oral and intrathecal medications were effective in reducing lower limb spasticity compared to placebo. CONCLUSIONS: Pharmacological treatment initiated 6 months post stroke reduced lower limb spasticity. Relevant areas of exploration for future research could include the period of effectiveness, long-term complications, and a cost-benefit analysis of such treatments.