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  1. Article ; Online: Safety and efficacy of edaravone in well-defined Iranian patients with amyotrophic lateral sclerosis: A parallel-group single-blind trial.

    Eishi-Oskouei, Alireza / Basiri, Keivan

    Current journal of neurology

    2023  Volume 20, Issue 1, Page(s) 1–7

    Abstract: Background: ...

    Abstract Background:
    Language English
    Publishing date 2023-11-27
    Publishing country Iran
    Document type Journal Article
    ISSN 2717-011X
    ISSN (online) 2717-011X
    DOI 10.18502/cjn.v20i1.6373
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Comparison of ultrasound findings in Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy for differential diagnosis.

    Basiri, Keivan / Abdollahian, Mahsa / Ghasemi, Majid

    Current journal of neurology

    2023  Volume 20, Issue 2, Page(s) 56–63

    Abstract: Background: ...

    Abstract Background:
    Language English
    Publishing date 2023-11-27
    Publishing country Iran
    Document type Journal Article
    ISSN 2717-011X
    ISSN (online) 2717-011X
    DOI 10.18502/cjn.v20i2.6740
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Pompe Disease Screening in a Sample of Iranian Patients

    Keivan Basiri / Behnaz Ansari

    Acta Medica Iranica, Vol 59, Iss

    2021  Volume 1

    Abstract: The reason why the experiments were done: previous studies have shown that the incidence rates vary in different populations. Now we report the preliminary results of the screening study. Pompe disease is a rare but potentially treatable disorder caused ... ...

    Abstract The reason why the experiments were done: previous studies have shown that the incidence rates vary in different populations. Now we report the preliminary results of the screening study. Pompe disease is a rare but potentially treatable disorder caused by the deficiency of the lysosomal enzyme acid-α-glucosidase (GAA). GAA activity was measured on DBS in 65 patients with undiagnosed myopathies presenting to the hospital of the research setting in Isfahan, Iran, from 2016 to 2017 and then was confirmed by genetic analysis. Of the total of 65 patients, 29 (44.6%) were male, and 36 (55.4%) were female. The mean age of the patients was 29±12.55 years, and their mean age at the disease onset was 17±12.75. Two patients (one male and one female) were diagnosed with a low acid alpha-glucosidase activity. Only one patient (female) showed a compound heterozygotic mutation of the GAA gene (c.-32-13T>G). Early diagnosis of Pompe disease is important for improving the outcome.
    Keywords Pompe disease ; Lysosomal enzyme ; Proximal myopathy ; HyperCKemia ; Medicine (General) ; R5-920
    Subject code 610
    Language English
    Publishing date 2021-02-01T00:00:00Z
    Publisher Tehran University of Medical Sciences
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  4. Article ; Online: On genotype-phenotype relationship of dystrophinopathies among Iranian population.

    Basiri, Keivan / Alizadeh, Maryam / Ansari, Behnaz / Ghasemi, Majid / Kheradmand, Mohsen / Sedghi, Maryam

    Current journal of neurology

    2022  Volume 22, Issue 4, Page(s) 231–237

    Abstract: Background: ...

    Abstract Background:
    Language English
    Publishing date 2022-12-19
    Publishing country Iran
    Document type Journal Article
    ISSN 2717-011X
    ISSN (online) 2717-011X
    DOI 10.18502/cjn.v22i4.14528
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Gabapentin versus Pregabalin for management of chronic inflammatory demyelinating polyradiculoneuropathy.

    Rayani, Moulood / Ansari, Behnaz / Boroujeni, Sajad Asadi / Veshnavei, Hossein Abdollahi / Basiri, Keivan

    American journal of neurodegenerative disease

    2021  Volume 10, Issue 4, Page(s) 50–56

    Abstract: Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic autoimmune demyelinating peripheral neuropathy that leads to symmetrical muscular weakness, sensory deficit, hyporeflexia, chronic fatigue, and impaired quality ... ...

    Abstract Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic autoimmune demyelinating peripheral neuropathy that leads to symmetrical muscular weakness, sensory deficit, hyporeflexia, chronic fatigue, and impaired quality of life (QoL). The current study aims to investigate the effects of gabapentin versus pregabalin on pain, sleep disturbances, and QoL in CIDP patients.
    Methods: This clinical trial was conducted on 40 patients diagnosed with CIDP randomly allocated to treatment with 100-500 mg gabapentin (n=20) or 50-300 mg pregabalin (n=20) both co-medicated with 37.5 mg venlafaxine. The dose of gabapentin/pregabalin was adjusted based on the patient's tolerability/response to the treatment. Visual analogue scale (VAS), Pittsburg Sleep Quality Questionnaire and Short Form Health Survey (SF-36) were filled at baseline, within three, six, nine and 12 months after the interventions to assess pain severity, sleep quality and QoL, respectively. The Iranian Registry of Clinical Trials (IRCT) code: IRCT20200217046523N16, https://fa.irct.ir/search/result?query=IRCT20200217046523N16.
    Results: Gabapentin revealed a dose-dependent efficacy in pain severity (
    Conclusion: Based on this study, the co-medication of pregabalin and venlafaxine led to remarkable superior outcomes compared to venlafaxine plus gabapentin in the management of pain, sleep quality, and QoL due to CIDP.
    Language English
    Publishing date 2021-08-15
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2695563-5
    ISSN 2165-591X
    ISSN 2165-591X
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Causative variants linked with limb girdle muscular dystrophy in an Iranian population: 6 novel variants.

    Mianesaz, Hamidreza / Ghalamkari, Safoura / Salehi, Mansoor / Behnam, Mahdiyeh / Hosseinzadeh, Majid / Basiri, Keivan / Ghasemi, Majid / Sedghi, Maryam / Ansari, Behnaz

    Molecular genetics & genomic medicine

    2022  Volume 11, Issue 2, Page(s) e2101

    Abstract: Background: Limb-girdle muscular dystrophy (LGMD) is a non-syndromic muscular dystrophy caused by variations in the genes involved in muscle structure, function and repair. The heterogeneity in the severity, progression, age of onset, and causative ... ...

    Abstract Background: Limb-girdle muscular dystrophy (LGMD) is a non-syndromic muscular dystrophy caused by variations in the genes involved in muscle structure, function and repair. The heterogeneity in the severity, progression, age of onset, and causative genes makes next-generation sequencing (NGS) a necessary approach for the proper diagnosis of LGMD.
    Methods: In this article, 26 Iranian patients with LGMD criteria were diagnosed with disease variants in the genes encoding calpain3, dysferlin, sarcoglycans and Laminin α-2. Patients were referred to the hospital with variable distribution of muscle wasting and progressive weakness in the body. The symptoms along with biochemical and EMG tests were suggestive of LGMD; thus the genomic DNA of patients were investigated by whole-exome sequencing including flanking intronic regions. The target genes were explored for the disease-causing variants. Moreover, the consequence of the amino acid alterations on proteins' secondary structure and function was investigated for a better understanding of the pathogenicity of variants. Variants were sorted based on the genomic region, type and clinical significance.
    Results: In a comprehensive investigation of previous clinical records, 6 variations were determined as novel, including c.1354-2 A > T and c.3169_3172dupCGGC in DYSF, c.568 G > T in SGCD, c.7243 C > T, c.8662_8663 insT and c. 4397G > C in LAMA2. Some of the detected variants were located in functional domains and/or near to the post-translational modification sites, altering or removing highly conserved regions of amino acid sequence.
    MeSH term(s) Humans ; Iran ; Muscular Dystrophies, Limb-Girdle/genetics ; Muscular Dystrophies
    Language English
    Publishing date 2022-11-14
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2734884-2
    ISSN 2324-9269 ; 2324-9269
    ISSN (online) 2324-9269
    ISSN 2324-9269
    DOI 10.1002/mgg3.2101
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Practical approach to electrodiagnosis of the carpal tunnel syndrome: A review.

    Basiri, Keivan / Katirji, Bashar

    Advanced biomedical research

    2015  Volume 4, Page(s) 50

    Abstract: Despite being the most common entrapment neuropathy and the most common reason for referral to the electromyography (EMG) laboratory, the diagnosis of carpal tunnel syndrome (CTS) continues to be challenging due to a large number of electrodiagnostic ( ... ...

    Abstract Despite being the most common entrapment neuropathy and the most common reason for referral to the electromyography (EMG) laboratory, the diagnosis of carpal tunnel syndrome (CTS) continues to be challenging due to a large number of electrodiagnostic (EDX) tests available. We present a flowchart and propose a practical approach to the diagnosis of CTS using the available literature and the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) guidelines and the Practice Parameter for Electrodiagnostic Studies in Carpal Tunnel Syndrome.
    Language English
    Publishing date 2015-02-17
    Publishing country India
    Document type Journal Article ; Review
    ZDB-ID 2672524-1
    ISSN 2277-9175
    ISSN 2277-9175
    DOI 10.4103/2277-9175.151552
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Practical approach to electrodiagnosis of the carpal tunnel syndrome

    Keivan Basiri / Bashar Katirji

    Advanced Biomedical Research, Vol 4, Iss 1, Pp 50-

    A review

    2015  Volume 50

    Abstract: Despite being the most common entrapment neuropathy and the most common reason for referral to the electromyography (EMG) laboratory, the diagnosis of carpal tunnel syndrome (CTS) continues to be challenging due to a large number of electrodiagnostic ( ... ...

    Abstract Despite being the most common entrapment neuropathy and the most common reason for referral to the electromyography (EMG) laboratory, the diagnosis of carpal tunnel syndrome (CTS) continues to be challenging due to a large number of electrodiagnostic (EDX) tests available. We present a flowchart and propose a practical approach to the diagnosis of CTS using the available literature and the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) guidelines and the Practice Parameter for Electrodiagnostic Studies in Carpal Tunnel Syndrome.
    Keywords Carpal tunnel syndrome ; electrodiagnosis ; nerve entrapment ; Medicine ; R ; Biology (General) ; QH301-705.5
    Language English
    Publishing date 2015-01-01T00:00:00Z
    Publisher Wolters Kluwer Medknow Publications
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article: Life-threatening misdiagnosis of bulbar onset myasthenia gravis as a motor neuron disease: How much can one rely on exaggerated deep tendon reflexes.

    Basiri, Keivan / Ansari, Behnaz / Okhovat, Ali Asghar

    Advanced biomedical research

    2015  Volume 4, Page(s) 58

    Abstract: The autoimmune disease myasthenia gravis (MG), can mimic a variety of neurological disorders leading to a delay in diagnosis and treatment. On occasions, misdiagnosis of MG could lead to unnecessary therapeutic interventions. We report the case of a 50 ... ...

    Abstract The autoimmune disease myasthenia gravis (MG), can mimic a variety of neurological disorders leading to a delay in diagnosis and treatment. On occasions, misdiagnosis of MG could lead to unnecessary therapeutic interventions. We report the case of a 50 year-old man, in whom MG was mistaken for motor neuron disease (MND). Subsequently, correct diagnosis and optimal management resulted in saving his life and significant improvement in his functional status. We discuss the importance of considering MG as one of the potential differential diagnoses among cases of new onset or recurrent unexplained bulbar symptoms, despite exaggerated deep tendon reflexes. Also, a literature review on the misdiagnosis of MG and the potential pitfalls in MG diagnosis are discussed.
    Language English
    Publishing date 2015-02-23
    Publishing country India
    Document type Case Reports
    ZDB-ID 2672524-1
    ISSN 2277-9175
    ISSN 2277-9175
    DOI 10.4103/2277-9175.151874
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article: The Schwartz-Jampel syndrome: Case report and review of literature.

    Basiri, Keivan / Fatehi, Farzad / Katirji, Bashar

    Advanced biomedical research

    2015  Volume 4, Page(s) 163

    Abstract: Schwartz-Jampel syndrome (SJS), first described in the United States in 1962, is a hereditary disorder characterized by facial dysmorphism and muscle stiffness. We describe the first case of a Persian 9-year-old boy with SJS and review the literature. ... ...

    Abstract Schwartz-Jampel syndrome (SJS), first described in the United States in 1962, is a hereditary disorder characterized by facial dysmorphism and muscle stiffness. We describe the first case of a Persian 9-year-old boy with SJS and review the literature. The child had a short neck, blepharophimosis, flattened face, hypertrichosis of the eyelids, prominent eyebrows, high arched palate, low set ears, micrognathia, short stature, and skeletal deformities. He had proximal muscle hypertrophy, distal muscle wasting and generalized hyporeflexia. Bone X-ray revealed pseudofracture of humerus. Needle electromyography revealed continuous myotonic discharges at rest with no waxing and waning in all tested muscles. Based on clinical and electrodiagnostic findings, the diagnosis of SJS type 1B was made and procainamide was started which resulted in clinical improvement. The diagnosis of SJS should be suspected when a child presents with the triad of myotonia, facial dysmorphism and skeletal deformities.
    Language English
    Publishing date 2015-08-10
    Publishing country India
    Document type Case Reports
    ZDB-ID 2672524-1
    ISSN 2277-9175
    ISSN 2277-9175
    DOI 10.4103/2277-9175.162538
    Database MEDical Literature Analysis and Retrieval System OnLINE

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