Prospective Randomized Study of Oral Diazepam and Baclofen on Spasticity in Cerebral Palsy

Goyal, Vinay; Laisram, Nonica; Wadhwa, Ranjan Kumar; Kothari, S Y

doi:10.7860/jcdr/2016/17067.7975

Cited by 10 publications

(11 citation statements)

References 14 publications

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“…The medications of choice for spasticity are baclofen and botulinum toxin (consensus; LE: 5). Baclofen treats generalized spasticity, 54,55 and a high dose is usually necessary for patients with CLN2 due to the intensity of spasticity. Botulinum toxin reduces local spasticity and improves muscle tone, 56 and it is available in the Brazilian public healthcare.…”

Section: Approach To Other Clinical Manifestationsmentioning

confidence: 99%

“…Benzodiazepines should not be used as long-term treatment as they increase respiratory secretion, muscle weakness, and ataxia. 54…”

Section: Managementmentioning

confidence: 99%

“…Benzodiazepines should not be used as long-term treatment as they increase respiratory secretion, muscle weakness, and ataxia. 54 Movement disorders, such as myoclonus, ataxia, dystonia, parkinsonism, and chorea, are frequent in patients with NCLs 57 and have been described in patients with CLN2. 16,58 According to our experts, the drug used for myoclonus is levetiracetam and for parkinsonism is dopamine; trihexyphenidyl and biperiden are indicated for dystonia with no preference among them (consensus; LE: 5).…”

Section: Approach To Other Clinical Manifestationsmentioning

confidence: 99%

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Clinical management and diagnosis of CLN2 disease: consensus of the Brazilian experts group

et al. 2023

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Neuronal ceroid lipofuscinosis type 2 (CLN2) is a rare neurodegenerative genetic disease that affects children in early life. Its classic form is rapidly progressive, leading to death within the first 10 years. The urge for earlier diagnosis increases with the availability of enzyme replacement therapy. A panel of nine Brazilian child neurologists combined their expertise in CLN2 with evidence from the medical literature to establish a consensus to manage this disease in Brazil. They voted 92 questions including diagnosis, clinical manifestations, and treatment of the disease, considering the access to healthcare in this country. Clinicians should suspect CLN2 disease in any child, from 2 to 4 years old, with language delay and epilepsy. Even though the classic form is the most prevalent, atypical cases with different phenotypes can be found. Electroencephalogram, magnetic resonance imaging, molecular and biochemical testing are the main tools to investigate and confirm the diagnosis. However, we have limited access to molecular testing in Brazil, and rely on the support from the pharmaceutical industry. The management of CLN2 should involve a multidisciplinary team and focus on the quality of life of patients and on family support. Enzyme replacement therapy with Cerliponase α is an innovative treatment approved in Brazil since 2018; it delays functional decline and provides quality of life. Given the difficulties for the diagnosis and treatment of rare diseases in our public health system, the early diagnosis of CLN2 needs improvement as enzyme replacement therapy is available and modifies the prognosis of patients.

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Section: Approach To Other Clinical Manifestationsmentioning

confidence: 99%

“…Benzodiazepines should not be used as long-term treatment as they increase respiratory secretion, muscle weakness, and ataxia. 54…”

Section: Managementmentioning

confidence: 99%

Section: Approach To Other Clinical Manifestationsmentioning

confidence: 99%

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Clinical management and diagnosis of CLN2 disease: consensus of the Brazilian experts group

et al. 2023

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“…Published regimens use maximum doses of levodopa as high as 400 mg/day for the treatment of dystonia in CP 16 , but the most common maximum daily dose used by respondents was 100 mg/day. Noting that dystoniaspecific dosing information is lacking for baclofen [17][18][19] , clonazepam 20 , and diazepam 18,21 , respondents used dosing regimens in line with those published for spasticity or seizure management.…”

Section: Most Common Medications Used Indications and Dosingmentioning

confidence: 99%

Physician Approaches to the Pharmacologic Treatment of Dystonia in Cerebral Palsy

Lott,

Fehlings,

Gelineau-Morel

et al. 2024

Preprint

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Objective: To determine how physicians approach pharmacologic dystonia treatment in people with CP and assess physician readiness to participate in a randomized trial comparing existing pharmacologic dystonia treatments. Methods: We administered a REDCap survey to physician members of the American Academy of Cerebral Palsy and Developmental Medicine and of the Child Neurology Society to assess which pharmacologic agents they use to treat dystonia in CP and their preferred indications and dosing. Results: Of 479 physicians surveyed, 240 (50%) responded. Respondents treated functionally limiting (95%) and generalized (57%) dystonia and most commonly used six medications: baclofen (95%), trihexyphenidyl (79%), gabapentin (67%), carbidopa/levodopa (55%), clonazepam (55%), and diazepam (54%). Baclofen was preferred in people with co-existing spasticity (81%), gabapentin was preferred in people with co-existing pain (49%), and trihexyphenidyl was avoided in people with constipation (34%) or urinary retention (42%). Preferred dosing regimens followed published regimens for dystonia, when available, but otherwise followed published regimens for other CP symptoms (spasticity and seizures). Baclofen was preferred by 64% of respondents as first line treatment, but there was no clear consensus on second or third-line medications. Most respondents (51%) were comfortable randomizing their patients to receive any of the six most commonly used medications used to treat dystonia in CP. Conclusions: This study summarizes current indications and dosing for the six most commonly used medications to treat dystonia in CP as per treating physicians in the US and Canada and also demonstrates physician support for a randomized trial comparing the effectiveness of these treatments.

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“…It was on this basis decided to discontinue the medication prior to the intervention as treatment with antispastic medication can cause potential challenges in relation to motor learning (Willerslev-Olsen, Lundbye-Jensen, Petersen, & Nielsen, 2011). Furthermore, the medication may cause unwanted side effects such as general muscle weakness, sedation, fatigue, and drowsiness (Chang et al, 2013;Goyal, Laisram, Wadhwa, & Kothari, 2016;Hulme, MacLennan, Ritchie, John, & Shotton, 1985;Willerslev-Olsen et al, 2011).…”

Section: Discontinuation Of Medicationmentioning

confidence: 99%

Motor-learning based activities may improve functional ability in adults with severe cerebral palsy: A controlled pilot study

Larsen

Frisk

Willerslev-Olsen

et al. 2021

NRE

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BACKGROUND: Cerebral palsy (CP) is a neurodevelopmental disturbance characterized by impaired control of movement. Function often decreases and 15% of adults are classified as severely affected (Gross Motor Function Classification Scale III-V). Little is known about interventions that aim to improve functional abilities in this population. OBJECTIVE: To evaluate a 12-week intervention based on motor learning principles on functional ability in adults with severe CP. METHODS: 16 adults (36±10 years, GMFCS III-V) were enrolled and divided into an intervention group (Active group) and a standard care group (Control group). Primary outcome measure was Gross Motor Function Measure (GMFM-88). Secondary measures were neurological status. The Active group were measured at baseline, after the intervention and at one-month follow-up. The Control group were measured at baseline and after one month. RESULTS: Analysis showed statistically significant improvement in GMFM-88 for the Active group from baseline to post assessment compared with the Control group (group difference: 5 points, SE 14.5, p = 0.008, CI: 1.2 to 8.7). Improvements were maintained at follow-up. Results from the neurological screening showed no clear tendencies. CONCLUSIONS: The study provides support that activities based on motor learning principles may improve gross motor function in adults with severe CP.

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Prospective Randomized Study of Oral Diazepam and Baclofen on Spasticity in Cerebral Palsy

Cited by 10 publications

References 14 publications

Clinical management and diagnosis of CLN2 disease: consensus of the Brazilian experts group

Clinical management and diagnosis of CLN2 disease: consensus of the Brazilian experts group

Physician Approaches to the Pharmacologic Treatment of Dystonia in Cerebral Palsy

Motor-learning based activities may improve functional ability in adults with severe cerebral palsy: A controlled pilot study

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