Berkala Paralyses

7/28/2019 Berkala Paralyses

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Berkala Paralyses

Author: Naganand Sripathi, MD, Director, Neuromuscular Clinic, Department of Neurology,

Henry Ford Hospital Pengarang: Sripathi Naganand, MD, Direktur, Klinik neuromuskular,

Departemen Neurologi, Rumah Sakit Henry FordContributor Information and DisclosuresKontributor Informasi dan Pengungkapan

Updated: Nov 25, 2010 Diperbarui: Nov 25, 2010

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OverviewIkhtisar Differential Diagnoses & WorkupDiferensial Diagnosa & hasil pemeriksaan

Treatment & MedicationPerawatan & Pengobatan Follow-upTindak lanjut ReferencesReferensi KeywordsKata kunci

Introduction Pengantar

Background Latar belakang

The heterogeneous group of muscle diseases known as periodic paralyses (PP) is characterized

by episodes of flaccid muscle weakness occurring at irregular intervals. Kelompok heterogenpenyakit otot yang dikenal sebagai melumpuhkan periodik (PP) yang ditandai dengan episode

kelemahan otot lembek terjadi pada interval yang tidak teratur. Most of the conditions are

hereditary and are more episodic than periodic. Sebagian besar kondisi keturunan dan lebihepisodik dari periodik. They can be divided conveniently into primary and secondary disorders.

Mereka dapat dibagi dengan mudah menjadi gangguan primer dan sekunder.

General characteristics of primary PP include the following: (1) they are hereditary; (2) most are

associated with alteration in serum potassium levels; (3) myotonia sometimes coexists; and (4)

both myotonia and PP result from defective ion channels. Karakteristik umum PP primermeliputi: (1) mereka turun temurun; (2) kebanyakan berhubungan dengan perubahan kadar

kalium serum, (3) myotonia kadang berdampingan, dan (4) baik myotonia dan hasil PP darisaluran ion rusak.

Pathophysiology Patofisiologi

A clinically useful classification of primary periodic paralyses, shown in Table 1, includeshypokalemic, hyperkalemic, and paramyotonic forms. Sebuah klasifikasi klinis yang berguna
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melumpuhkan periodik primer, ditunjukkan pada Tabel 1, termasuk bentuk hipokalemik,

hyperkalemic, dan paramyotonic.

Table 1. Tabel 1. Primary Periodic Paralysis Primer Kelumpuhan Berkala

Opentable in new windowBukatabel di jendela baru

[CLOSE WINDOW] [CLOSE WINDOW]

Table Tabel

Sodium channel Sodium

channel

Hyperkalemic PP (HyperPP) Hyperkalemic PP (HyperPP)

Hypokalemic PP (HypoPP2) Hipokalemik PP (HypoPP2)

Paramyotonia congenita Paramyotonia congenita

Calcium channel Kalsiumchannel


Potassium channel Kaliumsaluran

Andersen-Tawil syndrome Andersen-Tawil syndromeHyperkalemic PP or hypokalemic PP* Hyperkalemic PP atau PPhipokalemik *

*The deficit was described in 2 small families and has not been substantiated by others.,

*

Defisit tersebut dijelaskan dalam 2 keluarga kecil dan belum dibuktikan oleh orang lain.1 , 2

Sodium channel Sodiumchannel

Hyperkalemic PP (HyperPP) Hyperkalemic PP (HyperPP)Hypokalemic PP (HypoPP2) Hipokalemik PP (HypoPP2)

Paramyotonia congenita Paramyotonia congenita

Calcium channel Kalsium

channel


Potassium channel Kaliumsaluran

Andersen-Tawil syndrome Andersen-Tawil syndromeHyperkalemic PP or hypokalemic PP* Hyperkalemic PP atau PP

hipokalemik *

*The deficit was described in 2 small families and has not been substantiated by others.,

*Defisit tersebut dijelaskan dalam 2 keluarga kecil dan belum dibuktikan oleh orang lain.

1 , 2

The physiologic basis of flaccid weakness is inexcitability of the muscle membrane (ie,sarcolemma). Dasar fisiologis dari kelemahan lembek adalah inexcitability dari selaput otot

(yaitu, sarcolemma). Alteration of serum potassium level is not the principal defect in primary

PP; the altered potassium metabolism is a result of the PP. Perubahan kadar serum kalium

bukanlah cacat utama dalam PP primer; metabolisme kalium berubah adalah hasil dari PP

tersebut. In primary and thyrotoxic PP, flaccid paralysis occurs with relatively small changes inthe serum potassium level, whereas in secondary PP, serum potassium levels are markedly

abnormal. Dalam PP primer dan thyrotoxic, flaccid paralysis terjadi dengan perubahan yangrelatif kecil di tingkat kalium serum, sedangkan di PP sekunder, kadar kalium serum yang nyata

abnormal.

No single mechanism is responsible for this group of disorders. Tidak ada mekanisme tunggal

bertanggung jawab untuk kelompok gangguan. Thus, they are heterogeneous but share some


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common traits. Dengan demikian, mereka heterogen namun berbagi beberapa ciri umum. The

weakness usually is generalized but may be localized. Kelemahan biasanya umum tetapi bisa

dilokalisasi. Cranial musculature and respiratory muscles usually are spared. Cranial otot danpernapasan otot biasanya diselamatkan. Stretch reflexes are either absent or diminished during

the attacks. Stretch refleks baik tidak ada atau berkurang selama serangan. The muscle fibers are

electrically inexcitable during the attacks. Serat otot elektrik inexcitable selama serangan.Muscle strength is normal between attacks but, after a few years, some degree of fixed weaknessdevelops in certain types of PP (especially primary PP). kekuatan otot adalah normal antara

serangan tetapi, setelah beberapa tahun, beberapa derajat kelemahan tetap berkembang di

beberapa jenis PP (PP terutama primer). All forms of primary PP (except Becker myotoniacongenita [MC]) are either autosomal dominant inherited or sporadic (most likely arising from

point mutations). Semua bentuk PP primer (kecuali myotonia congenita Becker [MC]) baik

autosomal dominan warisan atau sporadis (yang paling mungkin timbul dari mutasi titik).

Voltage-sensitive ion channels closely regulate generation of action potentials (brief and

reversible alterations of the voltage of cellular membranes). saluran ion Voltage-sensitif erat

mengatur potensi generasi tindakan (perubahan singkat dan reversibel dari tegangan membranselular). These are selectively and variably permeable ion channels. Ini adalah selektif dan

variabel saluran ion permeabel. Energy-dependent ion transporters maintain concentrationgradients. transporter ion Energi-tergantung mempertahankan gradien konsentrasi. During thegeneration of action potentials, sodium ions move across the membrane through voltage-gated

ion channels. Selama generasi potensial aksi, ion natrium bergerak melintasi membran melalui

saluran tegangan-gated ion. The resting muscle fiber membrane is polarized primarily by themovement of chloride through chloride channels and is repolarized by movement of potassium.

Membran serat otot istirahat terpolarisasi terutama oleh pergerakan klorida melalui saluran

klorida dan repolarized oleh pergerakan kalium. Sodium, chloride, and calcium channelopathies,

as a group, are associated with myotonia and PP. Natrium, klorida, dan kalsium channelopathies,sebagai kelompok, yang berhubungan dengan myotonia dan PP. The functional subunits of

sodium, calcium, and potassium channels are homologous. Sub-unit fungsional natrium, kalsium,

dan saluran kalium yang homolog. Sodium channelopathies are better understood than calcium

or chloride channelopathies. Sodium channelopathies lebih baik dipahami daripadachannelopathies kalsium atau klorida. All forms of familial PP show the final mechanistic

pathway involving aberrant depolarization, inactivating sodium channels, and muscle fiber

inexcitability. Semua bentuk PP keluarga menunjukkan jalur depolarisasi mekanistik akhirmelibatkan menyimpang, menonaktifkan saluran natrium, dan inexcitability serat otot.

Discussion in this article primarily addresses the sodium, calcium, and potassium

channelopathies as well as secondary forms of PP. Pembahasan dalam artikel ini terutama alamat

natrium, kalsium, dan kalium channelopathies serta bentuk sekunder PP. Chloride

channelopathies are not associated with episodic weakness and are discussed in more detail inthe articles on myotonic disorders. channelopathies Klorida tidak berhubungan dengan

kelemahan episodik dan dibahas secara lebih rinci dalam artikel di gangguan myotonic.

Muscle sodium channel geneOtot saluran natrium gen


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The sodium channel has an alpha subunit and a beta subunit. Saluran natrium memiliki subunit

alfa dan subunit beta. The alpha subunit of the sodium channel is a 260-kd glycoprotein

comprising about 1800-2000 amino acids. Subunit alfa saluran natrium adalah glikoprotein 260-kd terdiri sekitar 1800-2000 asam amino. This channel is highly conserved evolutionarily from

Drosophila to human. Saluran ini sangat kekal evolusi dariDrosophila ke manusia. It has 4

homologous domains (I-IV) that fold to form a central pore, each with 225-325 amino acids. Iamemiliki 4 homolog domain (I-IV) yang flip untuk membentuk pori-pori pusat, masing-masingdengan 225-325 asam amino. Each domain consists of 6 hydrophobic segments (S1-S6)

traversing the cell membrane. Setiap domain terdiri dari 6 segmen hidrofobik (S1-S6) melintasi

membran sel. The main functions of the channel include voltage-sensitive gating, inactivation,and ion selectivity. Fungsi utama saluran termasuk gating tegangan-sensitif, inaktivasi, dan

selektivitas ion. The extracellular loop between S5 and S6 dips into the plasma membrane and

participates in the formation of the pore. Loop ekstraseluler antara S5 dan S6 dips ke dalam

membran plasma dan berpartisipasi dalam pembentukan pori-pori. The S4 segment containspositively charged amino acids at every third position and functions as a voltage sensor. Segmen

S4 mengandung asam amino yang bermuatan positif pada setiap posisi ketiga dan fungsi sebagai

sensor tegangan. Conformation changes may occur during depolarization, resulting in activationand inactivation of the channel. perubahan konformasi mungkin terjadi selama depolarisasi,

mengakibatkan aktivasi dan inaktivasi saluran. The cellular loop between domain III-S6 and

domain IV-S1 acts as an inactivating gate. Loop selular antara domain III-S6 dan bertindak

domain IV-S1 sebagai gerbang menonaktifkan.

The sodium channel has 2 gates (activation and inactivation) and can exist in 3 states. Salurannatrium memiliki 2 gerbang (aktivasi dan inaktivasi) dan dapat ada di 3 negara bagian. At rest

with the membrane polarized, the activation gate is closed and the inactivation gate is opened.

Saat istirahat dengan membran terpolarisasi, aktivasi gerbang ditutup dan gerbang inaktivasi

dibuka. With depolarization, the activation gate opens, allowing sodium ions to pass through theion channel and also exposing a docking site for the inactivation gate. Dengan depolarisasi,

gerbang aktivasi terbuka, yang memungkinkan ion natrium melewati saluran ion dan juga

memperlihatkan sebuah situs docking untuk gerbang inaktivasi. With continued depolarization,

the inactivation gate closes, blocking the entry of sodium into the cell and causing the channel toenter the fast-inactivation state. Dengan depolarisasi melanjutkan, gerbang inaktivasi menutup,

menghalangi masuknya natrium ke dalam sel dan menyebabkan saluran untuk memasuki negara

cepat-inaktivasi. This inactivation of the channel allows the membrane to become repolarized,resulting in a return to the resting state with the activation gate closed and the inactivation gate

opened. Inaktivasi ini memungkinkan membran saluran untuk menjadi repolarized, sehingga

kembali ke keadaan istirahat dengan aktivasi gerbang ditutup dan gerbang inaktivasi dibuka.

Two inactivation processes occur in mammalian skeletal muscle: Fast inactivation involvesterminating the action potential and acts on a millisecond time scale. Dua proses inaktivasi

terjadi pada otot rangka mamalia: inaktivasi Cepat melibatkan mengakhiri potensial aksi dan

bertindak pada skala waktu milidetik. Slow inactivation takes seconds to minutes and canregulate the population of excitable sodium channels. inaktivasi Lambat memerlukan beberapa

detik untuk menit dan dapat mengatur populasi saluran natrium bersemangat.

Sodium channel mutations that disrupt fast and slow inactivation are usually associated with a

phenotype of HyperPP and myotonia, where as mutations that enhance slow or fast inactivation


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producing loss of sodium channel function cause HypoPP. mutasi yang mengganggu saluran

Natrium inaktivasi cepat dan lambat biasanya berhubungan dengan fenotip HyperPP dan

myotonia, sedangkan mutasi yang meningkatkan inaktivasi lambat atau cepat menghasilkanhilangnya fungsi saluran natrium menyebabkan HypoPP.

Mutations of the sodium channel gene ( SCN4A ) have several general features. Mutasi gensaluran natrium (SCN4A) memiliki beberapa fitur umum. Most of the mutations are in the"inactivating" linker between repeats III and IV, in the "voltage-sensing" segment S4 of repeat

IV or at the inner membrane where they could impair the docking site for the inactivation gate.

Sebagian besar mutasi berada di linker "menonaktifkan" antara mengulangi III dan IV, di S4segmen "tegangan-sensing" dari mengulang IV atau pada membran dalam mana mereka bisa

merusak situs docking untuk gerbang inaktivasi. The clinical phenotype differs by specific amino

acid substitution and, while some overlap may occur between hyperkalemic PP, paramyotonia

congenita (PC), and potassium-aggravated myotonias (PAM), the 3 phenotypes are generallydistinct (as described below). Fenotip klinis berbeda dengan substitusi asam amino dan spesifik,

sementara beberapa tumpang tindih mungkin terjadi antara PP hyperkalemic, congenita

paramyotonia (PC), dan potasium-diperparah myotonias (PAM), 3 fenotip umumnya berbeda(seperti yang dijelaskan di bawah). Nearly all mutant channels have impaired fast-inactivation of

sodium current. Hampir semua saluran mutan merugikan cepat-inaktivasi natrium saat ini. Most

patients are sensitive to systemic potassium or to cold temperature. Kebanyakan pasien yang

sensitif terhadap kalium sistemik atau suhu dingin.

Two populations of channels exist, mutant and wild-type; the impaired fast-inactivation results inprolonged depolarization of the mutant muscle fiber membranes and can explain the 2 cardinal

symptoms of these disorders, myotonia and weakness. Dua populasi saluran ada, mutan dan wild

type, cepat-inaktivasi hasil terganggu pada depolarisasi berkepanjangan dari membran serat otot

mutan dan dapat menjelaskan gejala kardinal 2 myotonia ini, gangguan dan kelemahan. Inhyperkalemic PP, a gain of function occurs in mutant channel gating, resulting in an increased

sodium current excessively depolarizing the affected muscle. Dalam PP hyperkalemic,

keuntungan fungsi terjadi pada saluran gating mutan, yang mengakibatkan natrium meningkat

saat ini berlebihan depolarizing otot terpengaruh. Mild depolarization (5-10 mV) of the myofibermembrane, which may be caused by increased extracellular potassium concentrations, results in

the mutant channels being maintained in the noninactivated mode. Depolarisasi ringan (5-10

mV) dari membran myofiber, yang mungkin disebabkan oleh meningkatnya konsentrasi kaliumekstraseluler, hasil dalam saluran mutan dipertahankan dalam modus noninactivated. The

persistent inward sodium current causes repetitive firing of the wild-type sodium channels,

which is perceived as stiffness (ie, myotonia). Natrium batin terus-menerus saat ini menyebabkan

pembakaran berulang-ulang dari saluran sodium wild type, yang dianggap sebagai kekakuan(yaitu, myotonia).

If a more severe depolarization (20-30 mV) is present, both normal and abnormal channels are

fixed in a state of inactivation, causing weakness or paralysis. Jika depolarisasi lebih parah (20-

30 mV) hadir, baik saluran normal dan abnormal adalah tetap dalam keadaan inaktivasi,

menyebabkan kelemahan atau kelumpuhan. Thus, subtle differences in severity of membranedepolarization may make the difference between myotonia and paralysis. Dengan demikian,

perbedaan yang halus dalam keparahan depolarisasi membran dapat membuat perbedaan antara


6/35

myotonia dan kelumpuhan. Temperature sensitivity is a hallmark of PC. sensitivitas Suhu adalah

tanda dari PC. Cold exacerbates myotonia and induces weakness. Dingin memperparah myotonia

dan menginduksi kelemahan. A number of mutations are associated with this condition, 3 ofthem at the same site (1448) in the S4 segment. Sejumlah mutasi yang terkait dengan kondisi ini,

3 dari mereka di tempat yang sama (1448) di segmen S4. These mutations replace arginine with

other amino acids and neutralize this highly conserved S4 positive charge. Mutasi ini menggantiarginine dengan asam amino lain dan menetralisir muatan positif yang sangat lestari S4.Mutations of these residues are the most common cause of PC. Mutasi residu ini merupakan

penyebab paling umum PC. Some of the possible mechanisms responsible for temperature

sensitivity include the following: Beberapa mekanisme yang mungkin bertanggung jawab untuksensitivitas temperatur antara lain meliputi:

Temperature may differentially affect the conformational change in the mutant channel.Diferensial suhu dapat mempengaruhi perubahan konformasi dalam saluran mutan.

Lower temperatures may stabilize the mutant channels in an abnormal state. Turunkansuhu dapat menstabilkan saluran mutan dalam keadaan normal.

Mutations may alter the sensitivity of the channel to other cellular processes, such asphosphorylation or second messengers. Mutasi dapat mengubah sensitivitas saluran ke

proses seluler lain, seperti fosforilasi atau utusan kedua.

Most cases of hyperkalemic PP are due to 2 mutations in SCN4A, T704M, andM1592V.

Mutations in the sodium channel, especially at residues 1448 and 1313, are responsible for

paramyotonia congenita. Kebanyakan kasus PP hyperkalemic disebabkan oleh 2 mutasi dalamSCN4A, T704M, danM1592V. Mutasi di saluran natrium, terutama pada residu 1448 dan 1313,

bertanggung jawab untuk congenita paramyotonia. A small proportion of hypokalemic periodic

paralysis cases are associated with mutations at codons 669 and 672 (HypoPP2). Sebagian kecildari hipokalemik kasus paralisis periodik berhubungan dengan mutasi pada kodon 669 dan 672

(HypoPP2). In HypoPP2, sodium channel mutations enhance inactivation to produce a net loss of

function defect. Dalam HypoPP2, natrium meningkatkan saluran inaktivasi mutasi untuk

menghasilkan rugi bersih sebesar cacat fungsi.

Calcium channel geneKalsium channel gen

The calcium channel gene ( CACNL1A3 ) is a complex of 5 subunits (alpha-1, alpha-2, beta,

gamma, and delta). Saluran kalsium gen (CACNL1A3) adalah kompleks dari 5 subunit (alpha-1,alfa-2, beta, gamma, dan delta). The skeletal muscle dihydropyridine (DHP) receptor is located

primarily in the transverse tubular membrane. The dihydropyridine otot rangka (DHP) reseptor

terletak terutama di membran tubular melintang. The alpha-1 subunit has binding sites for DHP

drugs and conducts the slow L-type calcium current. The alfa-1 subunit memiliki situs mengikatbagi obat DHP dan melakukan kalsium L-tipe lambat saat ini. It also participates in excitation-

contraction (EC) coupling and acts as a voltage sensor through its linkage with the ryanodine

receptor of sarcoplasmic reticulum (ie, calcium release channel). Hal ini juga berpartisipasi

dalam eksitasi-kontraksi (EC) kopling dan bertindak sebagai sensor tegangan melalui keterkaitandengan reseptor ryanodine dari retikulum sarkoplasma (yaitu, kalsium saluran pelepasan). Any

changes in the membrane potential are linked to intracellular calcium release, enabling EC

coupling. Setiap perubahan dalam potensial membran terkait untuk melepaskan kalsium


7/35

intraseluler, sehingga kopling EC. Point mutations in DHP receptor/calcium channel alpha-1

subunit cause hypokalemic PP (HypoPP1). Point mutasi pada reseptor DHP / kalsium alpha

channel-1 menyebabkan subunit hipokalemik PP (HypoPP1). Two mutations ofCACNA1Sgene,R528H and R1239H, are responsible for most cases of hypokalemic PP. Dua mutasi gen

CACNA1S, R528H dan R1239H, bertanggung jawab atas sebagian besar kasus PP hipokalemik.

The physiological basis of disease is still not understood, but is more likely due to a failure of

excitation rather than a failure of EC coupling. Dasar fisiologis dari penyakit ini masih belum

dimengerti, tapi lebih cenderung karena kegagalan eksitasi daripada kegagalan kopling EC.However, hypokalemia-induced depolarization may reduce calcium release, affecting the voltage

control of the channel directly or indirectly through inactivation of the sodium channel. Namun,

hipokalemia-depolarisasi induced dapat mengurangi pelepasan kalsium, mempengaruhi kontrol

tegangan pada saluran secara langsung atau tidak langsung melalui inaktivasi saluran natrium.Insulin and adrenaline may act in a similar manner. Insulin dan adrenalin bisa bertindak dengan

cara yang sama. Mutations of the calcium channel gene have some similarities to SCN4A

mutations. Mutasi gen calcium channel memiliki beberapa kesamaan dengan mutasi SCN4A.

Mutations modify channel inactivation but not voltage-dependent activation. Mutasimemodifikasi inaktivasi channel tetapi tidak tergantung pada tegangan aktivasi. Recordings from

myotube cultures from affected patients revealed a 30% reduction in the DHP-sensitive L-typecalcium current. Rekaman dari budaya myotube dari pasien yang terkena menunjukkanpenurunan 30% dalam kalsium tipe L-DHP-sensitif saat ini. Channels are inactivated at low

membrane potentials. Saluran yang tidak aktif pada potensial membran rendah.

Calcium channel mutations cause a loss of function manifested as a reduced current density and

slower inactivation. mutasi saluran Kalsium menyebabkan hilangnya fungsi diwujudkan sebagai

densitas arus berkurang dan inaktivasi lambat. How this inactivation is related to hypokalemia-induced attacks is not understood. Bagaimana inaktivasi hal ini berkaitan dengan hipokalemia-

serangan induksi tidak dipahami. At least inR528Hmutation, a possible secondary

channelopathy occurs, tied to a reduction in the ATP-sensitive potassium current from altered

calcium homeostasis. Setidaknya dalam mutasiR528H, sebuah channelopathy sekunder yangmungkin terjadi, terkait dengan penurunan sensitif kalium ATP arus dari homeostasis kalsium

diubah. The lower currents associated with CACNL1A3 mutations could slightly alter

intracellular calcium homeostasis, which could affect the properties and expression of K+

channels, particularly KATP (ATP-sensitive potassium channel) belonging to inward rectifier

class of channels. Arus bawah yang terkait dengan mutasi CACNL1A3 sedikit bisa mengubah

homeostasis kalsium intraselular, yang dapat mempengaruhi sifat dan ekspresi K+

channel,khususnya KATP (sensitif saluran-kalium ATP) milik kelas penyearah ke dalam saluran. Insulin

also acts in HypoPP by reducing this inward rectifier K+

current. Insulin juga bertindak dalam

HypoPP dengan mengurangi penyearah ini ke dalam K+

saat ini.

Voltage sensor charge loss accounts for most cases of HypoPP. Tegangan sensor account rugi

biaya untuk sebagian besar kasus HypoPP. Sodium and calcium channels have homologous

pore-forming alfa subunits. Natrium dan saluran kalsium telah subunit alfa homolog pori-

membentuk. Point mutations in CACNL1A3 and SCN4A affect argentine residues in the S4voltage sensors of these channels. mutasi Point di CACNL1A3 dan SCN4A mempengaruhi residu

Argentina di sensor tegangan S4 dari saluran ini. Arginine mutations in S4 segments are


8/35

responsible for 90% of HypoPP cases.3mutasi Arginine di segmen S4 bertanggung jawab untuk

90% kasus HypoPP.3

Glucocorticosteroids cause HypoPP by stimulating Na+

K+

ATPase mediated by insulin and

amylin.4Glukokortikosteroid menyebabkan HypoPP dengan merangsang Na

+K

+ATPase

dimediasi oleh insulin dan amylin.

4

Potassium channel geneKalium saluran gen

Potassium channel mutations are seen in Andersen-Tawil syndrome. mutasi saluran Kalium

terlihat pada sindrom Andersen-Tawil. The triad of dysmorphic features, periodic paralysis, and

cardiac arrhythmias characterizes Andersen-Tawil syndrome. Tiga serangkai fitur dismorfik,

paralisis periodik, dan aritmia jantung ciri sindrom Andersen-Tawil. This syndrome is associatedwith mutations in theKCNJ2 gene.

5TheKCNJ2 gene encodes the inward-rectifying potassium

channel Kir2.1. Sindrom ini dikaitkan dengan mutasi pada genKCNJ2.5GenKCNJ2

mengkodekan kalium perbaikan saluran-ke dalam Kir2.1. Potassium channel mutations in

KCNE3 are reported to cause hypokalemic PP, but this has not been substantiated. mutasi saluranKalium dalamKCNE3 dilaporkan menyebabkan PP hipokalemik, tetapi hal ini belum

dibuktikan.

Frequency Frekuensi

United States Amerika Serikat

The frequencies of hyperkalemic periodic paralysis, paramyotonia congenita (PC), and

potassium-aggravated myotonias (PAM) are not known. Frekuensi kelumpuhan periodikhyperkalemic, congenita paramyotonia (PC), dan-kalium myotonias diperburuk (PAM) tidak

diketahui. Hypokalemic periodic paralysis has a prevalence of 1 case per 100,000 population.hipokalemik paralisis periodik memiliki prevalensi 1 kasus per 100.000 penduduk.

International Internasional

Not known Tidak diketahui

Race Ras

Thyrotoxic PP is most common in males (85%) of Asian descent with a frequency of

approximately 2%. Thyrotoxic PP adalah paling umum pada laki-laki (85%) dari keturunan Asia

dengan frekuensi sekitar 2%.

Clinical Klinis

History Sejarah


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All periodic paralyses (PPs) are characterized by episodic weakness. Semua melumpuhkan

periodik (PP) yang ditandai dengan kelemahan episodik. Strength is normal between attacks.

Kekuatan normal diantara serangan. Fixed weakness may develop later in some forms.kelemahan tetap dapat berkembang kemudian dalam beberapa bentuk. Most patients with

primary PP develop symptoms before the third decade. Kebanyakan pasien dengan PP primer

mengalami gejala sebelum dasawarsa ketiga.

Hyperkalemic periodic paralyses Hyperkalemic periodik melumpuhkano Age at onset is younger than 10 years. Umur saat onset lebih muda dari 10 tahun.

Patients usually describe a sense of heaviness or stiffness in the muscles. Pasien

biasanya menggambarkan rasa berat atau kekakuan pada otot. Weakness starts in

the thighs and calves, which then spreads to arms and neck. Kelemahan dimulai

pada paha dan betis, yang kemudian menyebar ke lengan dan leher. Proximalweakness predominates; distal muscles may become involved after vigorous

exercise. kelemahan proksimal mendominasi; otot distal dapat menjadi terlibat

setelah olahraga berat.

oIn children, a myotonic lid lag (lagging of upper eyelid on downward gaze) maybe the earliest symptom. Pada anak-anak, sebuah lag tutup myotonic (tertinggal

dari kelopak mata atas pada pandangannya ke bawah) mungkin gejala awal.Complete paralysis is rare and some residual mobility remains. kelumpuhanLengkap jarang dan beberapa mobilitas sisa tetap. Respiratory muscle

involvement is rare. Keterlibatan otot pernapasan jarang. The attacks last less than

4 hours and in the majority of cases, less than 1 hour. Serangan terakhir kurangdari 4 jam dan dalam sebagian besar kasus, kurang dari 1 jam. Sphincters are not

involved; any bowel and bladder dysfunction is due to abdominal muscle

weakness. Sphincters tidak terlibat, setiap disfungsi usus dan kandung kemih

disebabkan kelemahan otot perut.

o Weakness occurs during rest after a period of strenuous exercise or during fasting.Kelemahan terjadi selama beristirahat setelah periode latihan berat atau selama

puasa. It also may be provoked by potassium, cold, ethanol, or stress. Mungkin

juga terprovokasi oleh kalium, dingin, ethanol, atau stres. It may be relieved bymild prolonged exercise or carbohydrate intake. Ini mungkin lega dengan latihan

ringan atau berkepanjangan asupan karbohidrat. Patients also may report muscle

pains and paresthesias. Pasien juga dapat melaporkan nyeri otot dan parestesia.Between attacks, clinical and electrical myotonia is present in the majority of

patients. Antara serangan, myotonia klinis dan listrik hadir pada sebagian besar

pasien. Some families have no myotonia. Beberapa keluarga memiliki myotonia

tidak. Clinically apparent myotonia is seen less than 20% of patients, butelectrical myotonia may be found in 50-75%. Myotonia klinis jelas terlihat kurang

dari 20% pasien, tetapi myotonia listrik dapat ditemukan pada 50-75%. Interictal

weakness, if present, is not as severe as in hypokalemic PP. Interictal kelemahan,jika ada, tidak begitu parah seperti di PP hipokalemik.

Hypokalemic periodic paralyses Hipokalemik periodik melumpuhkano This can be divided into HypoPP1 (calcium channel mutation) and HypoPP2

(sodium channel mutation). Hal ini dapat dibagi menjadi HypoPP1 (mutasi

saluran kalsium) dan HypoPP2 (mutasi natrium channel).


10/35

o Severe cases present in early childhood and mild cases may present as late as thethird decade. kasus berat hadir dalam anak usia dini dan kasus-kasus ringan dapat

hadir hingga akhir dekade ketiga. A majority of cases present before age 16 years.Sebagian besar kasus ini sebelum usia 16 tahun. Weakness may range from slight

transient weakness of an isolated muscle group to severe generalized weakness.

Kelemahan bisa berkisar dari kelemahan transient sedikit kelompok otot terisolasiuntuk kelemahan umum yang parah. Severe attacks begin in the morning, oftenwith strenuous exercise or a high carbohydrate meal on the preceding day.

serangan berat mulai di pagi hari, seringkali dengan latihan berat atau makan

karbohidrat yang tinggi pada hari sebelumnya. Sometimes, the time betweenpremonitory symptoms to full-blown attack is in order of minutes. Kadang-

kadang, waktu antara gejala pertanda untuk menyerang besar-besaran adalah

dalam rangka menit. Attacks may also be provoked by stress, including infections,

menstruation, lack of sleep, and certain medications (eg, beta-agonists, insulin,corticosteroids). Serangan juga dapat dipicu oleh stres, termasuk infeksi,

menstruasi, kurang tidur, dan obat-obatan tertentu (misalnya, beta-agonis, insulin,

kortikosteroid). Patients wake up with severe symmetrical weakness, often withtruncal involvement. Pasien bangun dengan kelemahan simetris parah, seringkali

dengan keterlibatan truncal.

o Mild attacks are frequent and involve only a particular group of muscles, and maybe unilateral, partial, or monomelic. serangan ringan sering terjadi dan hanyamelibatkan kelompok tertentu otot, dan mungkin sepihak, parsial, atau

monomelic. This may affect predominantly legs; sometimes, extensor muscles are

affected more than flexors. Hal ini dapat mempengaruhi terutama kaki, kadang-kadang, otot ekstensor dipengaruhi lebih dari fleksor. Duration varies from a few

hours to almost 8 days but seldom exceeds 72 hours. Jangka waktu bervariasi dari

beberapa jam sampai hampir 8 hari tapi jarang melebihi 72 jam. The attacks are

intermittent and infrequent in the beginning but may increase in frequency untilattacks occur almost daily. Serangan yang intermiten dan jarang pada awalnya

tetapi mungkin peningkatan frekuensi sampai serangan terjadi hampir setiap hari.

The frequency starts diminishing by age 30 years; it rarely occurs after age 50years. Frekuensi mulai berkurang pada usia 30 tahun, itu jarang terjadi setelah

usia 50 tahun.

o Urinary output is decreased during the attack because water accumulatesintracellularly in muscles. keluaran urin menurun selama serangan karena air

terakumulasi intrasel pada otot. In HypoPP1 patients, the age of onset is earlier

(10 y), the symptoms lasts longer (20 h), and the fixed proximal weakness is more

frequent (about 70%), compared with HypoPP2 patients (16 y, 1 h, none). Padapasien HypoPP1, usia onset yang lebih awal (10 y), gejala-gejala berlangsung

lama (20 jam), dan kelemahan proksimal tetap lebih sering (sekitar 70%),

dibandingkan dengan pasien HypoPP2 (16 y, 1 jam, tidak ada ).

o Permanent muscle weakness may be seen later in the course of the disease andmay become severe. Tetap kelemahan otot dapat dilihat kemudian dalam

perjalanan penyakit dan bisa menjadi berat. Hypertrophy of the calves has been

observed. Hipertrofi dari betis telah diamati. Proximal muscle wasting, rather thanhypertrophy, may be seen in patients with permanent weakness. Wasting


11/35

proksimal otot, daripada hipertrofi, dapat dilihat pada pasien dengan kelemahan

permanen.

o HypoPP2 differs from HypoPP1 by (1) late onset, (2) tubular aggregates inmuscle biopsy (vacuolar myopathy in HypoPP1), (3) aggravation by

acetazolamide in HypoPP2. HypoPP2 berbeda dari HypoPP1 oleh (1) terlambat

onset, (2) agregat tubular di biopsi otot (miopati vacuolar di HypoPP1), (3)kejengkelan oleh acetazolamide di HypoPP2.

Paramyotonia congenita Paramyotonia congenitao In this autosomal dominant inherited disorder, myotonia worsens with activity

(paradoxical myotonia) or cold temperatures. Dalam gangguan ini diwariskanautosomal dominan, myotonia memburuk dengan aktivitas (myotonia paradoks)

atau suhu dingin.

o Symptoms are most pronounced in the face, tongue, and hand muscles with lesserinvolvement of lower limb. Gejala yang paling diucapkan dalam lidah, wajah, danotot tangan dengan keterlibatan yang lebih rendah dari tungkai bawah.

o Muscle hypertrophy may be seen in 30% of patients. Hipertrofi otot dapat dilihatpada 30% pasien.

o Myotonia lasts for seconds to minutes, but weakness may persist for hours andsometimes days. Myotonia berlangsung selama detik untuk menit, namun

kelemahan dapat bertahan selama berjam-jam dan kadang-kadang hari. Frequency

of paralytic attacks declines with age. Frekuensi menurun serangan lumpuhdengan usia.

o Permanent and severe myopathy is more frequent in patients with periodicparalysis. Permanen dan parah miopati lebih sering pada pasien dengankelumpuhan periodik.

o Episodic weakness also may develop after exercise or cold temperatures andusually lasts only a few minutes, but may last as long as days. kelemahan

Episodic juga dapat berkembang setelah suhu latihan atau dingin dan biasanyaberlangsung hanya beberapa menit, namun dapat berlangsung selama hari.

o Potassium loading usually worsens the symptoms, but in some cases, lowering theserum potassium level precipitates the attacks. loading Kalium biasanyamemperburuk gejala, tetapi dalam beberapa kasus, menurunkan tingkat serum

kalium presipitat serangan.

Thyrotoxic periodic paralyses Thyrotoxic periodik melumpuhkano Thyrotoxicosis periodic paralyses (TPP) are the most common secondary

hypokalemic PP. melumpuhkan Thyrotoxicosis periodik (TPP) adalah PP yang

paling umum hipokalemik sekunder. TPP is most common in adults aged 20-40

years. TPP yang paling umum pada orang dewasa berusia 20-40 tahun.Hyperinsulinemia, a carbohydrate load, and exercise are important in precipitating

paralytic attacks. Hyperinsulinemia, beban karbohidrat, dan latihan yang penting

dalam mempercepat serangan paralitik. Weakness is proximal and, if severe, may

involve respiratory or bulbar muscles. Kelemahan proksimal dan, jika parah,mungkin melibatkan otot pernafasan atau yg berhubungan dgn bengkak. Attacks

last hours to days. Serangan terakhir jam untuk hari.

o The prevalence of TPP in patients with thyrotoxicosis is estimated to be 0.1-0.2%in Caucasians and 13-14% in Chinese. Prevalensi TPP pada pasien dengan


12/35

tirotoksikosis diperkirakan 0,1-0,2% di Kaukasia dan 13-14% di Cina. Ninety-five

percent of TPP cases are sporadic. Sembilan puluh lima persen kasus TPP yang

sporadis. As TPP is more common in Asians, a genetic predisposition is stronglysuspected. Seperti TPP lebih umum di Asia, kecenderungan genetik diduga kuat.

Familial clustering of TPP indicates unmasking of an inherited disease (which is

sporadic) by thyrotoxicosis. clustering Familial dari TPP menunjukkanunmasking dari penyakit warisan (yang sporadis) dengan tirotoksikosis. Amutation inKCNE3 potassium channel gene was identified in one series.

6Sebuah

mutasi dalam gen saluran kaliumKCNE3 diidentifikasi dalam satu seri.6

Andersen-Tawil syndrome Andersen-Tawil syndromeo Andersen-Tawil syndrome is characterized by variable expression of the triad of

dysmorphic features, periodic paralysis, and cardiac arrhythmias. Andersen-Tawil

syndrome ditandai oleh variabel ekspresi tiga serangkai fitur dismorfik, paralisis

periodik, dan aritmia jantung. Patients may have short stature, hypertelorism, low-set ears, micrognathia, fifth finger clinodactyly, and scoliosis. Pasien mungkin

memiliki perawakan pendek, hypertelorism, telinga rendah-set, micrognathia, jari

kelima clinodactyly, dan scoliosis. Episodic weakness lasting a few hours toseveral days may arise spontaneously but usually follows physical activity.

kelemahan Episodic berlangsung beberapa jam sampai beberapa hari mungkin

timbul secara spontan tetapi biasanya mengikuti aktivitas fisik. The periodic

paralysis is not associated with myotonia. Kelumpuhan periodik tidak terkaitdengan myotonia.

o Prolonged QT interval and ventricular arrhythmias are the most common cardiacmanifestations. Berkepanjangan QT interval dan ventrikel aritmia adalahmanifestasi jantung yang paling umum. Other ECG abnormalities include PVCs,

ventricular bigeminy, supraventricular and ventricular tachycardias, prominent U

waves, and torsades de pointes. kelainan EKG lainnya termasuk PVC, bigeminy

ventrikel, tachycardias supraventrikuler dan ventrikel, menonjol U gelombang,dan de torsades pointes. Bidirectional ventricular tachycardia, which is

characterized by beat-to-beat alternating QRS axis polarity, is unique to a subset

of patients. takikardia ventrikel dua arah, yang ditandai dengan memukul-to-beatbolak sumbu QRS polaritas, adalah unik untuk subset dari pasien. Patients may be

completely asymptomatic. Pasien mungkin sama sekali tanpa gejala. Patients may

experience palpitations, syncopal episodes, and cardiac arrest. Pasien mungkinmengalami palpitasi, episode syncopal, dan serangan jantung. Sudden cardiac

death is less frequent in ATS when compared with the other long QT syndromes.

kematian mendadak jantung kurang sering di ATS bila dibandingkan dengan

sindrom QT panjang lainnya.

o Andersen-Tawil syndrome should always be considered in any patient withperiodic paralysis as facial dysmorphism may be subtle and cardiac symptoms are

not always present in spite of an abnormal ECG. Andersen-Tawil syndrome harus

selalu dipertimbangkan dalam setiap pasien dengan kelumpuhan periodik sebagaidysmorphism wajah mungkin gejala halus dan jantung tidak selalu hadir

meskipun EKG abnormal.

Physical Fisik


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Most of the patients with a periodic paralysis (PP) have similar clinical features, which are as

follows: Sebagian besar pasien dengan kelumpuhan periodik (PP) memiliki fitur klinis yang

serupa, yang adalah sebagai berikut:

Interictal lid lag and eyelid myotonia - May be the only clinical signs in hyperkalemic PPInterictal tutup lag dan myotonia kelopak mata - Mei menjadi satu-satunya tanda-tandaklinis pada PP hyperkalemic

Normal sensation Normal sensasi Fixed proximal weakness - May develop in patients with either hyperkalemic or

hypokalemic PP kelemahan proksimal Tetap - Mei mengembangkan pada pasien dengan

baik hyperkalemic atau hipokalemik PP

Diminished stretch reflexes during attacks Berkurang refleks peregangan selamaserangan

Table 2. Tabel 2. Distinguishing Features Among the Common Forms of Periodic Paralyses Fitur

Membedakan antara Bentuk umum dari Paralyses Berkala



Table Tabel

Syndrome

SindromaAge of

OnsetAgeof Onset

Duration of

AttackDurasiAttack

Precipitating

Pengendapan

FactorsFaktor-

faktor

Severity of

Attacks

Keparahan

Serangan

Associated

Asosiasi

FeaturesFitur

Hyper-kalemicperiodic

paralyses

Hyper-kalemicperiodik

melumpuhkan

Firstdecade of

life

Pertamadekade

kehidupan

Few minutes toless than 2 h

(mostly less

than 1 h)Beberapa

menit sampai

kurang dari 2jam

(kebanyakan

kurang dari 1

jam)

Lowcarbohydrate

intake (fasting)

Rendah asupankarbohidrat

(puasa)

Cold Dingin

Rest following

exercise Istirahat

Latihan berikut

Alcohol Alkohol

Infection Infeksi

Emotional stress

Rarely severeJarang parah

Perioral and limbparesthesias

Perioral dan

parestesia anggotatubuh

Myotonia frequentMyotonia sering

Occasional

pseudo-

hypertrophy ofmuscles Sesekalipseudo-hipertrofi

otot


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Stres emosional

Trauma Trauma

Menstrual period

Periodemenstruasi

Hypo-kalemicperiodic

paralyses

Hypo-kalemicperiodik

melumpuhkan

Variable -Childhood

to third

decadeVariabel-

Anak untuk

dekade

ketiga

Majority ofcasesbefore 16

years

Mayoritaskasus

sebelum 16

tahun

Few hours toalmost a week

Beberapa jam

untuk hampirseminggu

Typically nolonger than 72

h Biasanya

tidak lebih dari72 jam

Early morningattacks after

previous day

physical activityDini hari

serangan setelah

melakukan

aktivitas fisikhari sebelumnya

High-carbohydrate

meal, Chinese

food, alcoholTinggi-

karbohidrat

makan, makananCina, alkohol

Cold, change in

barometricpressure or

humidity Dingin,

perubahantekanan udara

atau kelembaban

Fever, upper

respiratory tractinfections

Demam, infeksi

saluranpernapasan atas

Lack of sleep,

Kurang tidur,fatigue kelelahan

Menstrual cycle

Severe Parah

Completeparalysis

Lengkap

kelumpuhan

Occasionalmyotonic lid lag

Sesekali lag

myotonic tutup

Myotonia between

attacks rareMyotonia antara

serangan langka

Unilateral, partial,

monomelic

Sepihak, parsial,monomelic

Fixed muscleweakness late in

disease kelemahan

otot tetap

terlambat dalam

penyakit


15/35

Siklusmenstruasi

Potassium-

associated

myotonia

Kalium terkaitmyotonia

First

decade

Pertama

dekade

No weakness

Tidak ada

kelemahan

Cold Dingin

Rest after

exercise Istirahatsetelah latihan

Attacks of

stiffness can

be mild to

severeSerangan

kekakuan

dapat ringansampai berat

Muscle

hypertrophy Otot

hipertrofi

Para-myotonia

congenita Para-

myotoniacongenita

First

decade

Pertamadekade

2-24 h 2-24 h Cold Dingin Rarely severe

Jarang parah

Pseudo-

hypertrophy of

muscles Pseudo-hipertrofi otot

Paradoxical

myotoniaParadoks

myotonia

Fixed weakness

rare Tetapkelemahan langka

Thyrotoxic

periodic

paralysesThyrotoxic

periodikmelumpuhkan

Third and

fourth

decadesKetiga dan

dekadekeempat

Few hours to 7

d Beberapa

jam untuk 7 d

Same as

hypokalemic PP

Sama seperti PPhipokalemik

Hyper-insulinemia

Hyper-

insulinemia

Same as

hypokalemic

PP Samaseperti PP

hipokalemik

Fixed muscle

weakness may

developkelemahan otot

tetap dapatmengembangkan

Hypokalemia

during attacksHipokalemia saat

serangan

Syndrome

SindromaAge of

OnsetAge

of Onset

Duration of

AttackDurasi

Attack

Precipitating

Pengendapan

FactorsFaktor-faktor

Severity of

Attacks

Keparahan

Serangan

Associated

Asosiasi

FeaturesFitur

Hyper-kalemic

periodicparalyses

Hyper-kalemic

periodikmelumpuhkan

First

decade oflife

Pertama

dekadekehidupan

Few minutes to

less than 2 h(mostly less

than 1 h)

Beberapamenit sampai

kurang dari 2

Low

carbohydrateintake (fasting)

Rendah asupan

karbohidrat(puasa)

Rarely severe

Jarang parah

Perioral and limb

paresthesiasPerioral dan

parestesia anggota

tubuh

Myotonia frequent


16/35

jam(kebanyakankurang dari 1

jam)

Cold Dingin

Rest following

exercise Istirahat

Latihan berikut

Alcohol Alkohol

Infection Infeksi

Emotional stress

Stres emosional

Trauma Trauma

Menstrual period

Periodemenstruasi

Myotonia sering

Occasional

pseudo-

hypertrophy of

muscles Sesekalipseudo-hipertrofi

otot

Hypo-kalemicperiodic

paralyses

Hypo-kalemicperiodik

melumpuhkan

Variable -Childhood

to third

decadeVariabel-

Anak untuk

dekade

ketiga

Majority ofcasesbefore 16

years

Mayoritaskasus

sebelum 16

tahun

Few hours toalmost a week

Beberapa jam

untuk hampirseminggu

Typically nolonger than 72

h Biasanya

tidak lebih dari72 jam

Early morningattacks after

previous day

physical activityDini hari

serangan setelah

melakukan

aktivitas fisikhari sebelumnya

High-carbohydrate

meal, Chinese

food, alcoholTinggi-

karbohidrat

makan, makananCina, alkohol

Cold, change in

barometricpressure or

humidity Dingin,

perubahantekanan udaraatau kelembaban

Fever, upper

Severe Parah

Complete

paralysisLengkap

kelumpuhan

Occasionalmyotonic lid lag

Sesekali lag

myotonic tutup

Myotonia between

attacks rareMyotonia antara

serangan langka

Unilateral, partial,

monomelic

Sepihak, parsial,monomelic

Fixed muscleweakness late in

disease kelemahan

otot tetap

terlambat dalam

penyakit


17/35

respiratory tractinfectionsDemam, infeksi

saluran

pernapasan atas

Lack of sleep,

Kurang tidur,fatigue kelelahan

Menstrual cycle

Siklus

menstruasi

Potassium-

associatedmyotonia

Kalium terkaitmyotonia

First

decadePertama

dekade

No weakness

Tidak adakelemahan

Cold Dingin

Rest after

exercise Istirahatsetelah latihan

Attacks of

stiffness canbe mild to

severeSerangankekakuan

dapat ringan

sampai berat

Muscle

hypertrophy Otothipertrofi

Para-myotoniacongenita Para-

myotonia

congenita

Firstdecade

Pertama

dekade

2-24 h 2-24 h Cold Dingin Rarely severeJarang parah

Pseudo-hypertrophy of

muscles Pseudo-

hipertrofi otot

Paradoxical

myotoniaParadoks

myotonia

Fixed weakness

rare Tetap

kelemahan langka

Thyrotoxicperiodic

paralyses

Thyrotoxic

periodikmelumpuhkan

Third andfourth

decades

Ketiga dan

dekadekeempat

Few hours to 7d Beberapa

jam untuk 7 d

Same ashypokalemic PP

Sama seperti PP

hipokalemik

Hyper-insulinemia

Hyper-insulinemia

Same ashypokalemic

PP Sama

seperti PP

hipokalemik

Fixed muscleweakness may

develop

kelemahan otot

tetap dapatmengembangkan

Hypokalemiaduring attacks

Hipokalemia saat

serangan


18/35

Causes Penyebab

Refer toPathophysiologyandTable 2andTable 3. LihatPatofisiologidanTabel 2danTabel 3.

Diferensial Diagnosa

Acute Inflammatory Demyelinating

PolyradiculoneuropathyPolyradiculoneuropathyinflamasi akut demielinasi

Spinal Cord HemorrhageSpinal Cord

Perdarahan

Cauda Equina and Conus Medullaris Syndromes

Cauda equina dan Conus medullaris Syndromes

Spinal Cord InfarctionSpinal Cord

Infarction

Chronic Inflammatory DemyelinatingPolyradiculoneuropathyPolyradiculoneuropathy

kronis inflamasi demielinasi

Spinal Cord, Topographical andFunctional AnatomySpinal Cord, dan

Fungsional Anatomi Topografi

Guillain-Barre Syndrome in ChildhoodGuillain-Barre Syndrome in Childhood

Spinal Epidural AbscessSpinal EpiduralAbses

Lambert-Eaton Myasthenic SyndromeLambert-Eaton

Sindrom miasthenikMultiple SclerosisMultiple Sclerosis

Myasthenia GravisGravis gravis

Other Problems to Be Considered Masalah lain untuk Be Dianggap

Table 3. Tabel 3. Differential Diagnosis of Secondary Periodic Paralyses Diferensial Diagnosis

Paralyses Berkala Sekunder



Table Tabel

HypokalemicHipokalemik HyperkalemicHyperkalemic

Urinary potassium-wasting syndromes Kencing

kalium-buang sindrom

Hyperaldosteronism Hyperaldosteronism Conn syndrome Conn sindrom Bartter syndrome Bartter sindrom Licorice intoxication Licorice keracunan

Alcohol Alkohol Addison disease Penyakit Addison

Chronic renal failure Gagal ginjal kronis
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Hyporeninemic HyporeninemicHypoaldosteronism Hypoaldosteronism

Drugs - Amphotericin B, barium Obat - Amfoterisin

B, barium

Ileostomy with tight stoma Ileostomy

dengan stoma ketat

Renal tubular acidosis Renal tubular asidosis Potassium load Kalium beban

GI potassium-wasting syndromes GI kalium-buangsindrom

Laxative abuse Penyalahgunaan pencahar Severe diarrhea Diare berat

Potassium-sparing diuretics Potassium-sparing diuretic

HypokalemicHipokalemik HyperkalemicHyperkalemic

Urinary potassium-wasting syndromes Kencingkalium-buang sindrom

Hyperaldosteronism Hyperaldosteronism Conn syndrome Conn sindrom Bartter syndrome Bartter sindrom Licorice intoxication Licorice keracunan

Alcohol Alkohol Addison disease Penyakit Addison

Chronic renal failure Gagal ginjal kronisHyporeninemic Hyporeninemic

Hypoaldosteronism Hypoaldosteronism

Drugs - Amphotericin B, barium Obat - Amfoterisin

B, barium

Ileostomy with tight stoma Ileostomy

dengan stoma ketat

Renal tubular acidosis Renal tubular asidosis Potassium load Kalium beban

GI potassium-wasting syndromes GI kalium-buang

sindrom

Laxative abuse Penyalahgunaan pencahar Severe diarrhea Diare berat

Potassium-sparing diuretics Potassium-

sparing diuretic

Table 4. Tabel 4. Differential Diagnosis of Other Entities Causing Acute Generalized Weakness

Diferensial Diagnosis Entitas Lain Menyebabkan Kelemahan Generalized Akut



Table Tabel

DisorderKekacauan Pattern andPola dan


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Distribution ofDistribusi

WeaknessKelemahan

Transient ischemic attacks Serangan iskemiktransien

Follow CNS distribution (ie, hemiparetic) Ikutidistribusi SSP (yaitu, hemiparetic)

May have sensory symptoms and signs

Mungkin memiliki gejala sensorik dan tanda-tanda

Sleep attacks Tidur serangan Occur at onset or termination of sleep Terjadi

pada awal atau pemutusan tidur

Last only minutes Terakhir hanya beberapamenit

Myelopathy Myelopathy

Traumatic Trauma Transverse myelitis Melintang myelitis Ischemic Iskemik

Sensory symptoms Sensory gejala

Presence of a sensory level Hadirnya tingkat

sensorikSphincter involvement Sphincter keterlibatan

Myasthenia gravis Myasthenia gravis

Lambert-Eaton myasthenic syndrome Sindrom

Lambert-Eaton miasthenik

Subacute in onset Subakut di awal

Associated autonomic symptoms in LEMS

Asosiasi gejala otonom memiliki kualifikasiHyporeflexia in LEMS Hyporeflexia di

ditempat anda

Abnormal repetitive nerve stimulationAbnormal stimulasi saraf berulang

Presence of distinct antibodies Kehadiran

antibodi yang berbeda

Peripheral neuropathy of acute onset Neuropatiperifer onset akut

Acute inflammatory Inflamasi akutdemyelinating poly-radiculoneuropathydemielinasi poli-radiculoneuropathy

Porphyria Porfiria

Pattern of weakness Pola kelemahanAbsent stretch reflexes Tidak ada stretchrefleks

Toxins Racun

Ciguatera Ciguatera Tetrodotoxin Tetrodotoxin

Clinical presentation Presentasi klinis

DisorderKekacauan Pattern andPola

Documents

Berkala Paralyses