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1.
BMC Neurol ; 24(1): 143, 2024 Apr 27.
Article in English | MEDLINE | ID: mdl-38678195

ABSTRACT

BACKGROUND: Spasticity can significantly affect a patient's quality of life, caregiver satisfaction, and the financial burden on the healthcare system. Baclofen is one of only a few options for treating spasticity. The purpose of this study is to investigate the impact of intrathecal baclofen (ITB) therapy on severe40.23 spasticity and motor function in patients with cerebral palsy. METHODS: We conducted a systematic review in PubMed, Scopus, Ovid, and the Cochrane Library in accordance with the PRISMA guidelines. We included studies based on eligibility criteria that included desired participants (cerebral palsy patients with spasticity), interventions (intrathecal baclofen), and outcomes (the Ashworth scales and the Gross Motor Function Measure [GMFM]). The within-group Cohen's d standardized mean differences (SMD) were analyzed using the random effect model. RESULTS: We screened 768 papers and included 19 in the severity of spasticity section and 6 in the motor function section. The pre-intervention average spasticity score (SD) was 3.2 (0.78), and the post-intervention average score (SD) was 1.9 (0.72), showing a 40.25% reduction. The SMD for spasticity reduction was - 1.7000 (95% CI [-2.1546; -1.2454], p-value < 0.0001), involving 343 patients with a weighted average age of 15.78 years and a weighted average baclofen dose of 289 µg/day. The SMD for the MAS and Ashworth Scale subgroups were - 1.7845 (95% CI [-2.8704; -0.6986]) and - 1.4837 (95% CI [-1.8585; -1.1088]), respectively. We found no relationship between the participants' mean age, baclofen dose, measurement time, and the results. The pre-intervention average GMFM (SD) was 40.03 (26.01), and the post-intervention average score (SD) was 43.88 (26.18), showing a 9.62% increase. The SMD for motor function using GMFM was 0.1503 (95% CI [0.0784; 0.2223], p-value = 0.0030), involving 117 patients with a weighted average age of 13.63 and a weighted average baclofen dose of 203 µg/day. In 501 ITB implantations, 203 medical complications were reported, including six new-onset seizures (2.96% of medical complications), seven increased seizure frequency (3.45%), 33 infections (16.26%), eight meningitis (3.94%), and 16 cerebrospinal fluid leaks (7.88%). Delivery system complications, including 75 catheter and pump complications, were also reported. CONCLUSION: Despite the risk of complications, ITB has a significant impact on the reduction of spasticity. A small but statistically significant improvement in motor function was also noted in a group of patients.


Subject(s)
Baclofen , Cerebral Palsy , Injections, Spinal , Muscle Relaxants, Central , Muscle Spasticity , Baclofen/administration & dosage , Humans , Muscle Spasticity/drug therapy , Muscle Spasticity/etiology , Cerebral Palsy/drug therapy , Cerebral Palsy/complications , Injections, Spinal/methods , Muscle Relaxants, Central/administration & dosage , Muscle Relaxants, Central/therapeutic use , Treatment Outcome , Severity of Illness Index , Motor Activity/drug effects , Motor Activity/physiology
2.
Acta Neurochir (Wien) ; 166(1): 308, 2024 Jul 30.
Article in English | MEDLINE | ID: mdl-39078485

ABSTRACT

BACKGROUND: Cerebral palsy (CP) is the most cause of motor dysfunction in children. Selective dorsal rhizotomy (SDR) plays a major role in long term spasticity control. However, limited data exists on the effect of SDR on postoperative spasticity treatment requirements and supraspinal effects, and the stimulation responses of dorsal nerve roots in those with CP. METHODS: The current study included the outcome for 35 individuals undergoing SDR for motor functional outcome, spasticity, baclofen dose changes, botulinum toxin injection frequency, and spasticity related orthopedic procedures. We also report on the stimulation responses in 112 individuals who underwent SDR at our institution. RESULTS: There was a significant difference in gross motor function measures (GMFM)-66 scores at last follow up that remained present when considering only ambulatory children but not with non-ambulatory children. Ashworth scores were significantly decreased for both upper and lower extremities after SDR at all follow up points. There was a significant decrease in Baclofen dose and botulinum toxin injections requirements after SDR, but no significant difference in the need for orthopedic intervention. A total of 5502 dorsal nerve roots were tested showing a decrease in stimulation intensity and increase in grade on the right side and for descending lumbosacral levels. CONCLUSIONS: SDR improves gross motor scores during short term follow up but has additional benefits in decreasing baclofen dosing and botulinum toxin injections requirements after surgery. They stimulation responses of sectioned dorsal nerve roots adds to the limited available data and our understanding of the pathological changes that occur in CP.


Subject(s)
Cerebral Palsy , Muscle Spasticity , Rhizotomy , Spinal Nerve Roots , Cerebral Palsy/surgery , Humans , Rhizotomy/methods , Male , Spinal Nerve Roots/surgery , Child , Female , Muscle Spasticity/surgery , Muscle Spasticity/drug therapy , Treatment Outcome , Adolescent , Baclofen/administration & dosage , Baclofen/therapeutic use , Child, Preschool , Muscle Relaxants, Central/therapeutic use , Muscle Relaxants, Central/administration & dosage
3.
Chem Pharm Bull (Tokyo) ; 72(3): 345-348, 2024.
Article in English | MEDLINE | ID: mdl-38556262

ABSTRACT

Eperisone Hydrochloride was launched in Japan in 1983 and has been used to improve muscle tone and treat spastic paralysis (Originator: Eisai Co., Ltd.). However, its biochemical mechanism of action is unknown. SB Drug Discovery was used to evaluate purinergic P2X (P2X) receptor antagonism using fluorescence. In this study, we discovered that its target protein is the P2X7 receptor. Also, P2X receptor subtype selectivity was high. This finding demonstrates the (Eperisone-P2X7-pain linkage), the validity of P2X7 as a drug target, and the possibility of drug repositioning of Eperisone Hydrochloride.


Subject(s)
Muscle Relaxants, Central , Propiophenones , Muscle Relaxants, Central/pharmacology , Muscle Relaxants, Central/therapeutic use , Purinergic P2X Receptor Antagonists/pharmacology , Propiophenones/pharmacology , Propiophenones/therapeutic use , Muscles
4.
J Craniofac Surg ; 35(4): e351-e354, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38829149

ABSTRACT

Malignant hyperthermia (MH) is a fatal hyperthermia with a high mortality, which usually occurs during induction of general anesthesia. Dantrolene sodium is a wonder drug currently used for treating malignant hyperthermia. However, preparing, storing, and maintaining dantrolene sodium are crucially expensive, thus making it financially unsatisfactory and difficult for clinicians to acquire in time. Monitoring patients' condition closely and intervening promptly when early signs of malignant hyperthermia occur can effectively prevent the condition from worsening and win over time for the arrival of dantraline sodium. This article is to report a case in which we successfully rescued a child occurring malignant hyperthermia without using dantrolene sodium.


Subject(s)
Anesthesia, General , Dantrolene , Malignant Hyperthermia , Muscle Relaxants, Central , Child, Preschool , Humans , Dantrolene/therapeutic use , Muscle Relaxants, Central/therapeutic use
5.
Medicina (Kaunas) ; 60(5)2024 May 01.
Article in English | MEDLINE | ID: mdl-38792938

ABSTRACT

Klippel-Feil syndrome (KFS) is characterized by the congenital fusion of the cervical vertebrae and is sometimes accompanied by anomalies in the craniocervical junction. In basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the brainstem and cervical cord results in neurological defects and surgery is required. A 16-year-old boy diagnosed with KFS and severe BI presented with spastic tetraplegia, opisthotonus and dyspnea. CT scans showed basilar impression, occipitalization of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Posterior occipitocervical reduction and fusion along with decompression were performed. Paralysis gradually improved postoperatively over 3 weeks. However, severe spasticity and opisthotonus persisted and intrathecal baclofen (ITB) therapy was initiated. Following this, opisthotonus disappeared and spasticity of the extremities improved. Rehabilitation therapy continued by controlling the dose of ITB. Five years after the surgery, self-propelled wheelchair driving was achieved and activities of daily life improved. The treatment strategy for patients with BI and congenital anomalies remains controversial. Posterior reduction and internal fixation using instrumentation were effective techniques in this case. Spasticity control achieved through a combination of surgery and ITB treatment enabled the amelioration of therapeutic efficacy of rehabilitation and the improvement of ADL.


Subject(s)
Baclofen , Cervical Vertebrae , Klippel-Feil Syndrome , Humans , Baclofen/therapeutic use , Baclofen/administration & dosage , Male , Klippel-Feil Syndrome/complications , Adolescent , Cervical Vertebrae/abnormalities , Cervical Vertebrae/surgery , Spinal Fusion/methods , Injections, Spinal/methods , Muscle Relaxants, Central/therapeutic use , Muscle Relaxants, Central/administration & dosage , Occipital Bone/abnormalities , Occipital Bone/surgery , Treatment Outcome , Decompression, Surgical/methods
7.
J Pediatr Rehabil Med ; 17(1): 57-66, 2024.
Article in English | MEDLINE | ID: mdl-38552121

ABSTRACT

PURPOSE: The use of intrathecal medications for the management of spasticity and various pain syndromes in the adult population has been previously reported. However, no evidence-based guidelines currently exist in the pediatric population. This case series presents patients (n = 8) with pediatric-onset disability who underwent placement of intrathecal baclofen pumps initially for management of severe spasticity. Despite titration of dose and use of oral analgesia, their uncontrolled pain persisted. Each patient was transitioned to a combination of baclofen and analgesic intrathecal therapy. The outcome in pain improvement and quality of life, as reported by patients and/or caregivers, were retrospectively reviewed. METHODS: Retrospective review of the background and decision-making process regarding transition to combination intrathecal therapy identified patient selection characteristics. Each patient and/or their caregivers completed a survey regarding improvements in pain, spasticity, function, and quality of life following initiation of combination intrathecal medications. RESULTS: Survey results revealed improvements in functional and pain assessments after initiation of combination baclofen and analgesic intrathecal medication. Patients and caregivers reported decreases in pain and oral spasticity medications. CONCLUSION: Use of pumps with antispasmodic and analgesic medication for combination intrathecal medication delivery should be considered in the management of patients with childhood-onset disabilities who have both severe spasticity and pain.


Subject(s)
Baclofen , Muscle Relaxants, Central , Adult , Humans , Child , Baclofen/therapeutic use , Muscle Relaxants, Central/therapeutic use , Retrospective Studies , Quality of Life , Muscle Spasticity/drug therapy , Muscle Spasticity/etiology , Pain , Analgesics/therapeutic use , Infusion Pumps, Implantable
8.
Int J Rehabil Res ; 47(1): 3-9, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38251093

ABSTRACT

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.


Subject(s)
Baclofen , Injections, Spinal , Muscle Relaxants, Central , Baclofen/therapeutic use , Baclofen/administration & dosage , Humans , Muscle Relaxants, Central/therapeutic use , Muscle Relaxants, Central/administration & dosage , Spastic Paraplegia, Hereditary/drug therapy , Infusion Pumps, Implantable
9.
CNS Neurol Disord Drug Targets ; 23(7): 917-926, 2024.
Article in English | MEDLINE | ID: mdl-38213169

ABSTRACT

BACKGROUND: Cerebral Palsy (CP) is a group of permanent, but not unchanging, disorders of movement and/or posture and motor function, which are due to a non-progressive interference, lesion, or abnormality of the developing/immature brain. One clinical presentation is muscle spasticity, which leads to a significant impact on the individual's functionality and quality of life. Spasticity treatment is multidisciplinary and includes pharmacological and physical intervention; intrathecal baclofen shows a positive effect in severe spasticity and suboptimal response to oral drugs, while local injection of Botulinum toxin type A (BTXA) improves muscle tone, motion and pain. OBJECTIVE: The aim of this study was to evaluate the efficacy of the combined intrathecal baclofen infusion (ITB) - botulinum toxin treatment in the management of spasticity in CP. METHODS: 8 patients with spastic tetraparesis were enrolled. All patients were treated with intrathecal Baclofen; in lower limbs, no spastic symptoms appeared, while marked spasticity was noted in upper limbs. We injected the right and left Biceps Brachial (BB) and Flexor Digitorum Superficialis (FDS) muscles with botulinum toxin type A. All patients underwent Myometric measurement, Ashworth Scale, Numerical Rating Scale, and Visual Analogic Scale evaluation before infiltration (T0), 30 days after injection (T1), 60 days after injection (T2), and 90 days after treatment (T3). RESULTS: All data demonstrated an improvement in spasticity, pain, quality of life, and self-care during the study, with p < 0.05. No side effects appeared. CONCLUSION: This study demonstrated the efficacy and safety of intrathecal baclofen infusion and botulinum toxin combined treatment in the management of spasticity, pain, quality of life, and selfcare in CP patients.


Subject(s)
Baclofen , Botulinum Toxins, Type A , Cerebral Palsy , Muscle Relaxants, Central , Muscle Spasticity , Humans , Baclofen/administration & dosage , Baclofen/therapeutic use , Muscle Spasticity/drug therapy , Muscle Spasticity/etiology , Cerebral Palsy/complications , Cerebral Palsy/drug therapy , Male , Female , Muscle Relaxants, Central/administration & dosage , Muscle Relaxants, Central/therapeutic use , Botulinum Toxins, Type A/administration & dosage , Botulinum Toxins, Type A/therapeutic use , Adult , Treatment Outcome , Young Adult , Injections, Spinal , Adolescent , Neuromuscular Agents/administration & dosage , Neuromuscular Agents/therapeutic use , Quality of Life , Drug Therapy, Combination , Infusions, Spinal
10.
Biochem Pharmacol ; 224: 116244, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38685280

ABSTRACT

Traumatic brain injury (TBI) triggers a bevy of changes including mitochondrial dysfunction, apoptosis, oxidative stress, neurobehavioural impairment, and neuroinflammation, among others. Dantrolene (DNT), a muscle relaxant which inhibits intracellular Ca2+ signaling from the ER, has been repurposed as a potential neuroprotective agent in various neurological diseases. However, there have been limited studies on whether it can mitigate TBI-induced deficits and restore impaired mitochondrial dynamics. This study sought to evaluate whether Dantrolene can potentially provide neuroprotection in an in vivo model of TBI. Male wistar rats subjected to TBI were treated with DNT (10 mg/kg) 1 h and 12 h post surgery. Animals were assessed 24 h post-TBI to evaluate neurobehavioural deficits and cerebral edema. We evaluated the protein expressions of apoptotic, autophagic, and neuroinflammatory markers by immunoblotting, as well as Mitochondrial Membrane Potential (MMP) and Reactive Oxygen Species (ROS) via Flow Cytometry to ascertain the effects of DNT on TBI. We further analysed immunofluorescence staining with Glial Fibrillary Acidic Protein (GFAP) and immunohistochemistry with NF-κß to investigate neuroinflammation. H&E staining was also performed post-TBI. Our findings revealed DNT administration inhibits mitochondria-mediated apoptotis and reduces heightened oxidative stress. DNT treatment was also found to reverse neurobehavioural impairments and offer neuroprotection by preserving neuronal architechture. We also demonstrated that DNT inhibits neuronal autophagy and alleviates neuroinflammation following TBI by modulating the NF-κß/Akt signaling pathway. Thus, our results suggest a novel application of DNT in ameliorating the multitude of deficits induced by TBI, thereby conferring neuroprotection.


Subject(s)
Brain Injuries, Traumatic , Dantrolene , Mitochondria , NF-kappa B , Neuroinflammatory Diseases , Proto-Oncogene Proteins c-akt , Rats, Wistar , Animals , Dantrolene/pharmacology , Dantrolene/therapeutic use , Brain Injuries, Traumatic/drug therapy , Brain Injuries, Traumatic/metabolism , Brain Injuries, Traumatic/pathology , Male , Rats , Proto-Oncogene Proteins c-akt/metabolism , Neuroinflammatory Diseases/drug therapy , NF-kappa B/metabolism , Mitochondria/drug effects , Mitochondria/metabolism , Signal Transduction/drug effects , Neuroprotective Agents/pharmacology , Neuroprotective Agents/therapeutic use , Muscle Relaxants, Central/pharmacology , Muscle Relaxants, Central/therapeutic use
11.
Arq. bras. neurocir ; 40(3): 238-244, 15/09/2021.
Article in English | LILACS | ID: biblio-1362120

ABSTRACT

Spasticity is amotor disorder that leads to a resistance to passive jointmovement. Cerebral palsy is the most important cause of spasticity and can be caused by several factors, including multiple gestations, alcoholism, infections, hemorrhages, drowning, and traumatic brain injuries, among others. There aremany scales that help tomeasure andmonitor the degree of impairment of these patients. The initial treatment should focus on the causal factor, such as tumors, inflammation, degenerative diseases, hydrocephalus, etc. Subsequently, the treatment of spastic musculature includes oral or intrathecal myorelaxants, spinal cord electrostimulation, neurotomies, Lissauer tract lesion, dentatotomy and selective dorsal rhizotomy. The latter is a safetechnique, possibleto beperformed inmost centers with neurosurgical support, and it is effective in the treatment of severe spasticity. In this article, the authors describe the surgical technique and conduct a review the literature.


Subject(s)
Motor Neuron Disease/surgery , Rhizotomy/rehabilitation , Muscle Spasticity/surgery , Muscle Spasticity/etiology , Cerebral Palsy/complications , Minimally Invasive Surgical Procedures/methods , Rhizotomy/methods , Laminoplasty/methods , Muscle Relaxants, Central/therapeutic use
12.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 38: e2018267, 2020. tab, graf
Article in English | LILACS | ID: biblio-1092130

ABSTRACT

ABSTRACT Objective: To report on a case of malignant hyperthermia in a child after a magnetic resonance imaging of the skull was performed using sevoflurane anesthesia. Case description: A 3-year-old boy admitted to the pediatric intensive care unit after presenting clinical and laboratory findings consistent with unspecified viral meningoencephalitis. While the patient was sedated, a magnetic resonance imaging of the skul was performed using propofol followed by the administration of sevoflurane through a laryngeal mask in order to continue anesthesia. Approximately three hours after the start of the procedure, the patient presented persistent tachycardia, tachypnea, generalized muscular stiffness and hyperthermia. With a diagnostic hypothesis of malignant hyperthermia, dantrolene was then administered, which immediately induced muscle stiffness, tachycardia, tachypnea and reduced body temperature. Comments: Malignant hyperthermia is a rare pharmacogenetic syndrome characterized by a severe hypermetabolic reaction after the administration of halogenated inhalational anesthetics or depolarizing muscle relaxants such as succinylcholine, or both. Although it is a potentially fatal disease, the rapid administration of continued doses dantrolene has drastically reduced the morbidity and mortality of the disease.


RESUMO Objetivo: Relatar um caso de hipertermia maligna em criança após exame de ressonância magnética de crânio realizada sob efeito anestésico de sevoflurano. Descrição do caso: Menino de três anos de idade, admitido na Unidade de Terapia Intensiva Pediátrica (UTIP) após apresentar quadros clínico e laboratorial compatíveis com meningoencefalite viral não especificada. Foi realizado um exame de ressonância magnética de crânio com sedação utilizando, na indução anestésica, o propofol seguido pela administração de sevoflurano por meio de máscara laríngea para manutenção anestésica. Aproximadamente três horas após o início do procedimento, o paciente apresentou taquicardia, taquipneia, rigidez muscular generalizada e hipertermia persistentes. Com hipótese diagnóstica de hipertermia maligna, foi então administrado dantrolene, que fez ceder de forma imediata a rigidez muscular, a taquicardia, a taquipneia e reduziu a temperatura corporal. Comentários: A hipertermia maligna é uma síndrome farmacogenética rara, que se caracteriza por reação hipermetabólica grave após administração de anestésicos inalatórios halogenados e/ou relaxantes musculares despolarizantes, como a succinilcolina. Apesar de ser uma doença potencialmente fatal, a rápida administração de dantrolene, junto às doses de manutenção, tem reduzido drasticamente a morbimortalidade da doença.


Subject(s)
Humans , Male , Child, Preschool , Magnetic Resonance Imaging , Anesthetics, Inhalation/adverse effects , Sevoflurane/adverse effects , Malignant Hyperthermia/etiology , Antiviral Agents/therapeutic use , Acyclovir/therapeutic use , Propofol/administration & dosage , Anesthetics, Intravenous/administration & dosage , Anesthetics, Inhalation/administration & dosage , Dantrolene/therapeutic use , Sevoflurane/administration & dosage , Malignant Hyperthermia/drug therapy , Meningoencephalitis/drug therapy , Meningoencephalitis/virology , Muscle Relaxants, Central/therapeutic use
14.
Arq. neuropsiquiatr ; 71(3): 171-173, mar. 2013. tab, graf
Article in English | LILACS | ID: lil-668763

ABSTRACT

Objective To compare the adherence between monotherapy and politherapy in prophylactic migraine treatment. Method Five hundred consecutive patients with migraine from a tertiary center were retrospectively studied as to the number of preventive medications prescribed during the first visit. Adherence, defined as returning for the next consultation after 4 to 6 weeks and following the prescribed regimens, were also evaluated and compared between patients. Results 71.8% were women, and 6% of the patients did not receive any preventive medication, 11.4% received one drug, 22.2% two drugs, 41.4% three drugs, and 19% four drugs for the prevention of migraine. The overall adherence was 79.6%. Respectively, 73.7, 71.8, 82.6 and 86.3% of those who received the prescription of one, two, three and four drugs returned, complying with the treatment. Conclusion There is no difference in adherence to monotherapy or politherapy (one to four drugs) for the prophylaxis of migraine. .


Objetivo Comparar a adesão entre monoterapia e politerapia no tratamento profilático da migrânea. Método Foram analisados retrospec-tivamente 500 pacientes com migrânea de um centro terciário, com vistas ao número de medicações preventivas prescritas na primeira consulta e adesão ao tratamento após 4 a 6 semanas. Resultados 71,8% da amostra era composta de mulheres. Em relação ao tratamento preventivo da migrânea, 6% não receberam medicação preventiva; 11,4% receberam uma droga; 22,2% duas drogas; 41,4% três drogas e 19% quatro drogas. A adesão média foi de 79,6%. Em relação à prescrição de uma, duas, três ou quatro drogas, essa adesão foi respectiva-mente de 73,7, 71,8, 82,6 e 86,3%. Conclusão Não houve diferenças na adesão à prescrição de medicações em monoterapia ou politerapia (uma a quatro drogas) para o tratamento preventivo da migrânea. .


Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Medication Adherence/statistics & numerical data , Migraine Disorders/drug therapy , Prescription Drugs/therapeutic use , Adrenergic beta-1 Receptor Antagonists/therapeutic use , Antidepressive Agents/therapeutic use , Brazil , Drug Therapy, Combination/methods , Flunarizine/therapeutic use , Muscle Relaxants, Central/therapeutic use , Retrospective Studies , Time Factors , Treatment Outcome
15.
Arq. neuropsiquiatr ; 69(2b): 288-291, 2011. ilus
Article in English | LILACS | ID: lil-588085

ABSTRACT

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disorder characterized by late-infantile onset spastic ataxia and other neurological features. ARSACS has a high prevalence in northeastern Quebec, Canada. Several ARSACS cases have been reported outside Canada in recent decades. This is the first report of typical clinical and neuroimaging features in a Brazilian family with probable diagnosis of ARSACS.


A ataxia espástica autossômica recessiva de Charlevoix-Saguenay (ARSACS) é doença degenerativa do sistema nervoso, caracterizada por ataxia associada a espasticidade, entre outras manifestações neurológicas, de início na infância. A doença tem alta prevalência na região de Quebec, no Canadá. Muitos relatos de ARSACS têm sido descritos fora do Canadá nas últimas décadas. Nesse artigo, relatamos a primeira descrição dos aspectos clínicos e de neuroimagem típicos em uma família brasileira com provável diagnóstico de ARSACS.


Subject(s)
Adult , Female , Humans , Male , Muscle Spasticity/diagnosis , Spinocerebellar Ataxias/congenital , Amitriptyline/analogs & derivatives , Amitriptyline/therapeutic use , Baclofen/therapeutic use , Magnetic Resonance Imaging , Muscle Relaxants, Central/therapeutic use , Muscle Spasticity/drug therapy , Pedigree , Spinocerebellar Ataxias/diagnosis , Spinocerebellar Ataxias/drug therapy
16.
Acta cient. venez ; 57(4): 125-129, 2006. tab
Article in Spanish | LILACS | ID: lil-537098

ABSTRACT

Existen varios métodos para el estudio de la interacción entre bloqueadores musculares y otros tantos para elcálculo de la curva dosis respuesta. El presente trabajo analiza la interacción entre el vecuronio y rocuronio comparando las potencias respectivas obtenidas del control con el de las mezclas, calculadas para cada paciente. En primer lugar se concluyó la relación dosis-respuesta con el uso de dosis subparalíticas únicas, administrando vecuronio 10, 20 y 30 ug. Kg-¹ o rocuronio 50, 100 y 200 ug.Kg-¹. Utilizando el máximo efecto se determinaron las dosis efectivas-50 y 95 (DE50 – 95) resolviendo la ecuación de Hill para cada paciente considerando 4,75 como coeficiente. Seguidamente se repitieron los cálculos después de administrar fracciones equipotenciales que representan 0.2, 0.3 y 0.4 x DE50 de ambas drogas. Con ayuda del análisis algebraico se identificó el tipo de interacción: [dv / (DE50-95) v + dr/(DE50-95) r] y que demostró un valor ligeramente menor de uno [1] lo cual indica aditivismo. Todas las cifras que se obtuvieron guardan gran similitud con los provenientes de otros métodos y sugieren que el presente es una herramienta sencilla, confiable y útil para el cálculo de la relación dosis-respuesta en general y para estudiar la interacción entre vecuronio y rocuronio en particular.


There are several methods for the study of the interaction between neuromuscular blockers and as many tocalculate their dose-response curve. The present trial deals with the interaction between vecuronium and rocuronium assessed by comparison of their respective potencies obtained for a control with that of their mixtures, both calculated for each patient. Drug potency was obtained first after the administration of single no paralytic doses: vecuronium 10, 20 and 30 ug. Kg-¹ or rocuronium 50, 100 and 200 ug. Kg-¹. Using maximal effect, 50 and 95 effective doses (ED50 – 95) were determined by solving Hill equation for each subject, considering 4.75 as the coefficient. The same procedure was repeated after the administration of 0.2, 0.3 or 0.4 x ED50 of both drugs. Algebraic analysis was used to identified the type of interaction according to [dv/ (DE50-95) v + dr/ (DE50-95) r] leading to a value less than one [1] indicating additivism. All numerical values actually obtained are in close agreement with that coming from other methods, suggesting that this is an easy, consistent and useful tool for the calculation of the doseresponserelationship in general and for the study of the vecuronium-rocuronium interaction in particular.


Subject(s)
Vecuronium Bromide/analysis , Vecuronium Bromide/therapeutic use , Dose-Response Relationship, Drug , Muscle Relaxants, Central/analysis , Muscle Relaxants, Central/therapeutic use , Anesthesiology , Pharmacology
17.
Article in Spanish | LILACS | ID: lil-437751

ABSTRACT

La hipertermia maligna (HTM) es una enfermedad fármaco-genética de carácter autosómico dominante de baja incidencia pero potencialmente mortal, caracterizada por un síndrome hipermetabólico del músculo estriado esquelético, que puede desencadenar la destrucción completa de éste, resultando en aumento de la temperatura corporal, alto consumo de oxígeno, hiperpotasemia, falla renal y arritmias. A continuación se da a conocer el caso de un paciente de 6 años portador de una distrofia muscular de Duchenne con antecedentes familiares de hipertermia maligna y que se sometió a cirugía adenoamigdaliana en la cual se tomaron las medidas anestésicas para prevenir la aparición de una crisis.


Subject(s)
Humans , Male , Child , Intraoperative Complications/prevention & control , Muscular Dystrophy, Duchenne/complications , Malignant Hyperthermia/etiology , Malignant Hyperthermia/prevention & control , Anesthetics/adverse effects , Dantrolene/therapeutic use , Acute Disease , Precipitating Factors , Malignant Hyperthermia/genetics , Malignant Hyperthermia/drug therapy , Muscle Relaxants, Central/therapeutic use , Succinylcholine/adverse effects
18.
Arq. neuropsiquiatr ; 62(2b): 403-405, jun. 2004.
Article in English | LILACS | ID: lil-362202

ABSTRACT

OBJETIVO: Estudar a eficiência do uso da infusão de morfina intratecal, no tratamento da espasticidade. MÉTODO: Doze pacientes com espasticidade refratária a tratamento clínico foram estudados. Dez eram do sexo masculino e dois do feminino. A idade média foi 34,4 anos (20 a 61 anos). A média inicial da escala de Ashworth foi 4,6. Todos foram submetidos a implante de bomba de infusão para infusão Intratecal de morfina. RESULTADOS: Ao final do tratamento o valor da escala de Ashworth foi 2,2. A dose média de infusão intratecal de morfina foi 0,95 mg, com freqüência de aplicação de 1,8 vezes/dia. Como complicações, quatro pacientes desenvolveram prurido, dois náuseas, dois retenção urinária e todos melhoraram com diminuição da concentração da morfina intratecal. Um paciente foi submetido à revisão da bomba de morfina por extrusão do cateter. CONCLUSAO: O uso de morfina intratecal para o tratamento de espasticidade refratária a tratamento clínico é eficaz e com poucas complicações.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Analgesics, Opioid/therapeutic use , Infusion Pumps, Implantable , Morphine/therapeutic use , Muscle Spasticity/drug therapy , Injections, Spinal , Muscle Relaxants, Central/therapeutic use
19.
Rev. Fac. Odontol. (B.Aires) ; 12(29): 10-5, abr. 1992.
Article in Spanish | LILACS | ID: lil-115303

ABSTRACT

Se define el trismus como una contractura de los músculos masticadores de etiología variada. Se lo encuadra como uno de los síntomas comunes a varias afecciones. Las infecciones, los traumatismos, los tumores y algunas afecciones generales son capaces de provocar trismus. Es importante hacer un buen diagnóstico de la afección para poder instituir el tratamiento correcto. La mayoría de los trismus ceden con tratamiento kinésicos acompañado de tratamiento general con relajantes musculares y analgésicos. Cuando fracasan los tratamientos conservadores, la cirugía está indicada, teniendo especial cuidado en el trazado de las incisiones por la brida cicatrizal que puede originar


Subject(s)
Muscle Relaxants, Central/therapeutic use , Masticatory Muscles/injuries , Trismus/therapy , Central Nervous System/injuries , Massage
20.
Compend. invest. clin. latinoam ; 8(2): 54-8, nov.-dic. 1988. ilus
Article in Spanish | LILACS | ID: lil-71665

ABSTRACT

En un estudio abierto, prospectivo a corto plazo se incluyeron 25 pacientes de uno u otro sexo, con edades comprendidas entre 16 a 60 años y con rigidez por cocntractura muscular consecutiva a traumatismo o ejercicio. A cada paceinte se le administró un nuevo mioespasmolítico: tizanidina por vía oral a dosis de 4 mg cada 8 horas durante 5 días. Se valoró en todos los casos, al inicio del estudio y a los 3 y 5 días de tratamiento el dolor espontáneo, el dolor a la palpación, la tensión muscular y la incapacidad funcional. También se valoraron los efectos seccundarios que se presentaron durante la administración de la tizanidina. Se notó una importante mejoría en los cuatro parámetros estudiados desde el tercer día de tratamiento, misma que se mantuvo hasta el final del estudio. El medicamento fue bien tolerado a dosis de 12 mg ya que el 68% de los pacientes no presentaron ningún efecto secundario. Se concluye que la tizanidina es una buena alternativa para el tratamiento de las contracturas musculares postraumáticas y por ejercicio


Subject(s)
Adolescent , Adult , Middle Aged , Humans , Male , Female , Clonidine/analogs & derivatives , Exercise/adverse effects , Muscle Relaxants, Central/therapeutic use , Muscle Rigidity/drug therapy , Muscles/injuries , Muscle Rigidity/etiology , Prospective Studies
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