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clonazepam and Stiff-Person Syndrome

clonazepam has been researched along with Stiff-Person Syndrome in 20 studies

Clonazepam: An anticonvulsant used for several types of seizures, including myotonic or atonic seizures, photosensitive epilepsy, and absence seizures, although tolerance may develop. It is seldom effective in generalized tonic-clonic or partial seizures. The mechanism of action appears to involve the enhancement of GAMMA-AMINOBUTYRIC ACID receptor responses.
clonazepam : 1,3-Dihydro-2H-1,4-benzodiazepin-2-one in which the hydrogens at positions 5 and 7 are substituted by 2-chlorophenyl and nitro groups, respectively. It is used in the treatment of all types of epilepsy and seizures, as well as myoclonus and associated abnormal movements, and panic disorders. However, its use can be limited by the development of tolerance and by sedation.

Stiff-Person Syndrome: A condition characterized by persistent spasms (SPASM) involving multiple muscles, primarily in the lower limbs and trunk. The illness tends to occur in the fourth to sixth decade of life, presenting with intermittent spasms that become continuous. Minor sensory stimuli, such as noise and light touch, precipitate severe spasms. Spasms do not occur during sleep and only rarely involve cranial muscles. Respiration may become impaired in advanced cases. (Adams et al., Principles of Neurology, 6th ed, p1492; Neurology 1998 Jul;51(1):85-93)

Research Excerpts

ExcerptRelevanceReference
"Oral clonazepam is an effective therapy besides behavioral and safety interventions for hereditary cases."2.66Hyperekplexia and other startle syndromes. ( Pandey, S; Saini, AG, 2020)
"Her diagnosis was confirmed as hereditary hyperekplexia with GlRA1 hybrid gene mutations based on the sequencing results."1.56C.292G>A, a novel glycine receptor alpha 1 subunit gene (GLRA1) mutation found in a Chinese patient with hyperekplexia: A case report. ( Lin, CM; Wu, LL; Zhang, Y; Zheng, XL, 2020)
"Treatment with clonazepam markedly reduced the threshold and intensity of the startle response, enabling him to recover independence."1.40Sporadic hyperekplexia presenting with an ataxic gait. ( Bilbao, I; Losada, J; Maestro, I; Rouco, I; Zarranz, JJ, 2014)
"The prognosis is generally good in hereditary hyperekplexia."1.33Hyperekplexia in two siblings. ( Kannan, B; Kulkarni, ML; Mathadh, P, 2006)
" For treatment, the dosage and way of administration of diazepam should be judged according to the severeness of the disease; nitrazepam and clonazepam are effective, too."1.28[Clinical analysis of 30 cases of stiff-man syndrome]. ( Chen, BJ, 1992)

Research

Studies (20)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's5 (25.00)18.2507
2000's4 (20.00)29.6817
2010's8 (40.00)24.3611
2020's3 (15.00)2.80

Authors

AuthorsStudies
Zhang, Y1
Wu, LL1
Zheng, XL1
Lin, CM1
Saini, AG1
Pandey, S1
Cai, Q1
Wu, C1
Xu, W1
Liang, Y1
Liao, S1
Moriyoshi, H1
Hata, Y1
Inagaki, R1
Suzuki, J1
Nishida, S1
Nishida, N1
Ito, Y1
Zhang, C1
Wang, SG1
Wang, Y1
Liu, XL1
Cao, L1
Derksen, A1
Stettner, M1
Stöcker, W2
Seitz, RJ1
Rouco, I1
Bilbao, I1
Losada, J1
Maestro, I1
Zarranz, JJ1
Eisermann, M1
Lardeux, C1
Nicloux, M1
Bahi-Buisson, N1
Vanbellinghen, JF1
Magny, JF1
Kaminska, A1
Lapillonne, A1
McAbee, GN1
Rønne, MS1
Nielsen, PB1
Mogensen, CB1
Saigal, R1
Goyal, L1
Yadav, R1
Agrawal, A1
Mital, P1
Patel, B1
Poon, WT1
Au, KM1
Chan, YW1
Chan, KY1
Chow, CB1
Tong, SF1
Lam, CW1
Kulkarni, ML1
Kannan, B1
Mathadh, P1
Garg, R1
Ramachandran, R1
Sharma, P1
Vieregge, P1
Branczyk, B1
Barnett, W1
Soyka, D1
Kömpf, D1
Prevett, MC1
Brown, P1
Duncan, JS1
Fiol, M1
Cammarota, A1
Rivero, A1
Pardal, A1
Nogués, M1
Correale, J1
Chen, BJ1
Ryan, SG2
Dixon, MJ1
Nigro, MA1
Kelts, KA1
Markand, ON1
Terry, JC2
Shiang, R1
Wasmuth, JJ1
O'Connell, P1
Sherman, SL1
Sparkes, RS1
Torres, MC1
Mackey, RW1

Reviews

1 review available for clonazepam and Stiff-Person Syndrome

ArticleYear
Hyperekplexia and other startle syndromes.
    Journal of the neurological sciences, 2020, Sep-15, Volume: 416

    Topics: Adult; Child; Clonazepam; Humans; Hyperekplexia; Infant; Infant, Newborn; Muscle Rigidity; Receptors

2020

Other Studies

19 other studies available for clonazepam and Stiff-Person Syndrome

ArticleYear
C.292G>A, a novel glycine receptor alpha 1 subunit gene (GLRA1) mutation found in a Chinese patient with hyperekplexia: A case report.
    Medicine, 2020, Volume: 99, Issue:17

    Topics: China; Clonazepam; Female; Humans; Infant; Mutation; Receptors, Glycine; Reflex, Startle; Stiff-Pers

2020
Stiff-person syndrome coexisting with critical illness polyneuropathy: A case report.
    Medicine, 2020, Dec-11, Volume: 99, Issue:50

    Topics: Baclofen; Clonazepam; Diagnosis, Differential; Electromyography; Humans; Immunoglobulins, Intravenou

2020
[A pedigree of hereditary hyperekplexia].
    Rinsho shinkeigaku = Clinical neurology, 2018, Jul-27, Volume: 58, Issue:7

    Topics: Accidental Falls; Administration, Oral; Adult; Clonazepam; Female; Humans; Male; Pedigree; Point Mut

2018
Teaching Video NeuroImages: Cautious walking gait in siblings with hereditary hyperekplexia.
    Neurology, 2019, 04-23, Volume: 92, Issue:17

    Topics: Adult; Clonazepam; Female; GABA Modulators; Gait; Gait Disorders, Neurologic; Humans; Male; Middle A

2019
Antiglycine receptor-related stiff limb syndrome in a patient with chronic lymphocytic leukaemia.
    BMJ case reports, 2013, May-20, Volume: 2013

    Topics: Aged; Autoantibodies; Baclofen; Clonazepam; Electromyography; GABA Agents; Humans; Leukemia, Lymphoc

2013
Sporadic hyperekplexia presenting with an ataxic gait.
    Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 2014, Volume: 21, Issue:2

    Topics: Clonazepam; Diagnosis, Differential; Electroencephalography; Electromyography; GABA Modulators; Gait

2014
Not all myoclonic jerking and tonic posturing in the neonate is epilepsy.
    The Journal of pediatrics, 2014, Volume: 164, Issue:3

    Topics: Anticonvulsants; Clonazepam; Consanguinity; Diagnosis, Differential; Electroencephalography; Epileps

2014
Clobazam-clonazepam combination effective for stimulus-induced falling in hyperekplexia.
    Journal of child neurology, 2015, Volume: 30, Issue:1

    Topics: Accidental Falls; Anticonvulsants; Benzodiazepines; Child; Clobazam; Clonazepam; Drug Therapy, Combi

2015
[Stiff baby syndrome is a rare cause of neonatal hypertonicity].
    Ugeskrift for laeger, 2014, Feb-24, Volume: 176, Issue:9A

    Topics: Anticonvulsants; Clonazepam; Female; Humans; Infant, Newborn; Rare Diseases; Stiff-Person Syndrome

2014
Stiff Person Syndrome.
    The Journal of the Association of Physicians of India, 2015, Volume: 63, Issue:8

    Topics: Adult; Antibodies; Anticonvulsants; Autoimmunity; Baclofen; Clonazepam; Electromyography; Glutamate

2015
Novel missense mutation (Y279S) in the GLRA1 gene causing hyperekplexia.
    Clinica chimica acta; international journal of clinical chemistry, 2006, Volume: 364, Issue:1-2

    Topics: Anticonvulsants; Base Sequence; Child, Preschool; Clonazepam; DNA Mutational Analysis; Female; Follo

2006
Hyperekplexia in two siblings.
    Indian journal of pediatrics, 2006, Volume: 73, Issue:12

    Topics: Anticonvulsants; Child, Preschool; Clonazepam; Female; Humans; Infant, Newborn; Male; Reflex, Startl

2006
Anaesthetic implications of hyperekplexia--'startle disease'.
    Anaesthesia and intensive care, 2008, Volume: 36, Issue:2

    Topics: Anesthesia; Anesthetics, Inhalation; Anticonvulsants; Child; Clonazepam; Electroencephalography; Hum

2008
[Stiff-man syndrome. Report of 4 cases].
    Der Nervenarzt, 1994, Volume: 65, Issue:10

    Topics: Adult; Animals; Autoantibodies; Cerebellum; Clonazepam; Dose-Response Relationship, Drug; Drug Admin

1994
Improvement of stiff-man syndrome with vigabatrin.
    Neurology, 1997, Volume: 48, Issue:4

    Topics: Adult; Anticonvulsants; Carbamazepine; Clonazepam; Drug Therapy, Combination; Female; gamma-Aminobut

1997
Focal stiff-person syndrome.
    Neurologia (Barcelona, Spain), 2001, Volume: 16, Issue:2

    Topics: Adult; Anticonvulsants; Autoantibodies; Clonazepam; Diagnosis, Differential; Electromyography; Femal

2001
[Clinical analysis of 30 cases of stiff-man syndrome].
    Zhonghua shen jing jing shen ke za zhi = Chinese journal of neurology and psychiatry, 1992, Volume: 25, Issue:6

    Topics: Adolescent; Adult; Clonazepam; Diazepam; Female; Humans; Male; Middle Aged; Nitrazepam; Reference St

1992
Genetic and radiation hybrid mapping of the hyperekplexia region on chromosome 5q.
    American journal of human genetics, 1992, Volume: 51, Issue:6

    Topics: Base Sequence; Cell Line, Transformed; Chromosome Mapping; Chromosomes, Human, Pair 5; Clonazepam; D

1992
Startle disease, or hyperekplexia: response to clonazepam and assignment of the gene (STHE) to chromosome 5q by linkage analysis.
    Annals of neurology, 1992, Volume: 31, Issue:6

    Topics: Adult; Child, Preschool; Chromosomes, Human, Pair 5; Clonazepam; Female; Genes, Dominant; Genetic Ma

1992