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Stiff-Person Syndrome, Penyakit Langka yang Memicu Kejang Otot dan Kelumpuhan

Stiff-Person Syndrome, Penyakit Langka yang Memicu Kejang Otot dan Kelumpuhan
Ilustrasi stiff-person syndrome. Credit: Freepik

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Setiap hari manusia menggunakan otot untuk bergerak. Namun pada kondisi tertentu, otot tidak dapat berfungsi dengan normal sehingga menyebabkan kejang otot. Kondisi tersebut bisa jadi merupakan gejala stiff-person syndrome, sebuah penyakit autoimun langka yang menyerang saraf.

 

Apa Itu Stiff-Person Syndrome?

Stiff-person syndrome adalah penyakit autoimun dan gangguan saraf yang menyebabkan otot di batang tubuh dan tungkai mengalami kaku dan kejang. Orang dengan kondisi ini pertama-tama mengalami kekakuan otot tubuh, yang menyebar ke bagian tubuh lainnya seiring berjalannya waktu.

Penyakit ini juga dikenal sebagai sindrom Moersch-Woltman atau sindrom orang kaku. Kondisi ini menyebabkan kejang otot yang menyakitkan. Kejang otot yang muncul dapat dipicu oleh kebisingan, sentuhan fisik dan tekanan emosional. 

Stiff-person syndrome adalah kondisi yang sangat langka. Dilansir dari Cleveland Clinic, hanya 1 dari 1 juta orang yang didiagnosis sindrom ini. Kondisi ini umumnya lebih banyak dialami wanita daripada pria. Gejala dapat muncul pada usia berapa pun namun umumnya berkembang antara usia 30-60 tahun.

Baca Juga: Mengenal Bell's Palsy, Kelumpuhan Otot Wajah yang Sering Dikira Stroke

 

Penyebab Stiff-Person Syndrome

Dilansir dari Yale Medicine, penyebab penyakit langka ini hingga kini masih belum diketahui. Namun peneliti menduga hal tersebut merupakan reaksi autoimun di mana tubuh menyerang sel saraf di sistem saraf pusat yang mengontrol pergerakan otot.

Pada sebagian besar pengidap stiff-person syndrome memiliki antibodi yang menargetkan enzim glutamic acid decarboxylase (GAD). GAD berperan dalam membuat neurotransmitter yang disebut gamma-aminobutyric acid (GABA) yang membantu mengontrol pergerakan otot.

Para ahli menduga sistem kekebalan tubuh pada orang dengan stiff-person syndrome secara keliru menyerang enzim GAD sehingga menurunkan jumlah GABA dalam tubuh. Meskipun demikian, masih dibutuhkan penelitian lebih lanjut karena ada orang dengan sindrom tersebut yang tidak memiliki antibodi terhadap GAD yang dapat dideteksi.

 

Gejala Stiff-Person Syndrome

Gejala utama stiff-person syndrome adalah otot terasa kaku di batang tubuh bersamaan dengan kejang otot yang hebat. Rasa kaku ini dapat dipicu oleh rangsangan dari lingkungan seperti suara keras atau tekanan emosional. Jika kejang otot cukup parah, hal ini dapat menyebabkan orang tersebut jatuh. Setelah rangsangan tersebut berlalu, otot secara bertahap akan mengendur.

Gejala stiff-person syndrome bisa berkembang dalam berbulan-bulan atau bertahun-tahun. Beberapa pasien tetap stabil selama bertahun-tahun, namun beberapa lainnya memburuk. Pada awalnya, otot kaku bersifat hilang dan timbul tetapi lama kelamaan kekakuan ini akan menjadi konstan. Seiring waktu, otot yang mengalami kekakuan menjadi lebih banyak termasuk di lengan dan wajah.

Pada beberapa orang, otot kaku yang dialami dapat menyebabkan postur membungkuk. Pada kasus parah, otot yang kaku dapat menyebabkan kesulitan untuk berjalan atau bergerak.

Pengidap stiff-person syndrome juga cenderung menunjukkan gejala depresi dan kecemasan yang sebagian besar dipicu oleh kondisi perkembangan penyakit yang tidak diketahui. Di sisi lain, pengidap sindrom kaku ini juga memiliki tingkat neurotransmitter GABA lebih rendah yang juga bertugas mengatur kecemasan.

Baca Juga: Hernia Diskus, Masalah Tulang Belakang yang Sering Disebut Sebagai Saraf Terjepit

 

Pengobatan Stiff-Person Syndrome

Hingga saat ini belum ditemukan penanganan khusus untuk stiff-person syndrome. Penanganan sementara melibatkan pemberian obat untuk menangani gejala yang muncul. Dokter dapat meresepkan obat penenang, pelemas otot dan obat-obatan steroid. Selain itu pasien juga akan membutuhkan beberapa jenis fisioterapi untuk memaksimalkan gerak tubuh.

 

Stiff-person syndrome adalah penyakit langka yang masih membutuhkan penelitian lebih lanjut. Apabila Anda merasakan kejang otot yang semakin lama semakin parah, sebaiknya segera periksakan ke dokter.

Jika Anda memerlukan saran atau konsultasi medis, Anda dapat mengunjungi dokter atau memanfaatkan fitur konsultasi yang tersedia di aplikasi Ai Care dengan mengunduh aplikasi Ai Care dari App Store atau Play Store.

 

Mau tahu informasi seputar penyakit lainnya? Cek di sini, ya!

Writer : Ratih AI Care
Editor :
  • Sean Edbert Lim, MBBS
Last Updated : Kamis, 20 Juni 2024 | 06:16

Cleveland Clinic. Stiff Person Syndrome. Available from: https://www.yalemedicine.org/conditions/stiff-person-syndrome#

National Institute of Neurological Disorders and Stroke. Stiff-Person Syndrome. Available from: https://www.ninds.nih.gov/health-information/disorders/stiff-person-syndrome

 

Yale Medicine. Stiff-Person Syndrome. Available from: https://www.yalemedicine.org/conditions/stiff-person-syndrome#

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        • vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
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        • view::web.v2.partials.article-popular:8
        • vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
        • vendor/livewire/livewire/src/Mechanisms/ExtendBlade/ExtendedCompilerEngine.php:22
        • vendor/laravel/framework/src/Illuminate/View/Engines/CompilerEngine.php:72
      • article-popular.blade.php#8aicare_prod_new10.31msselect `categories`.*, `category_post`.`post_id` as `pivot_post_id`, `category_post`.`category_id` as `pivot_category_id`, `category_post`.`created_at` as `pivot_created_at`, `category_post`.`updated_at` as `pivot_updated_at` from `categories` inner join `category_post` on `categories`.`id` = `category_post`.`category_id` where `category_post`.`post_id` = 1007 and `categories`.`deleted_at` is null
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        Backtrace
        • view::web.v2.partials.article-popular:8
        • vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
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        Backtrace
        • view::web.v2.partials.article-popular:11
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        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
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        • vendor/laravel/framework/src/Illuminate/View/Engines/CompilerEngine.php:72
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        Backtrace
        • view::web.v2.partials.article-popular:8
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        • view::web.v2.partials.article-popular:8
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        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
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        • view::web.v2.partials.article-popular:8
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        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
        • vendor/livewire/livewire/src/Mechanisms/ExtendBlade/ExtendedCompilerEngine.php:22
        • vendor/laravel/framework/src/Illuminate/View/Engines/CompilerEngine.php:72
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        • view::web.v2.partials.related-article:8
        • vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
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        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
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        Backtrace
        • view::web.v2.partials.related-article:8
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        Hints
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        Backtrace
        • view::web.v2.partials.related-article:8
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        • vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
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        Backtrace
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        • vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
        • vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
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