Electrolyte Conference

Proximal Muscle Weakness

. 1, . 2, .. 3
1

, 2, 3

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Vol. 10 No.2

60
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17 ..
2554

:
2

: 3 .

2 .




.

: 5
3


.
40

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. 2-3


:

10

:

:

4-5

General appearance: A man looked healthy with

good consciousness
Vital signs: BT 36.8 C, PR 84 /min, RR 14 /min,
BP 150/90 mmHg
HEENT: Pink conjunctivae, anicteric sclerae, no
enlarged thyroid gland, no cervical lymphadenopathy, no lid lag or lid retraction
Heart: Normal S1 S2, no murmur
Lungs: Normal breath sound, no adventitious
sound

Abdomen: Soft, not tender, impalpable liver and

spleen
Extremities: No edema, no tremor
Neurological examination: Alert, normal orientation to time place and person

Normal speech, no cranial nerve palsy

Motor power; Upper extremities, proximal
grade III both, lower grade V both

Lower extremities,proximal
grade III both, lower grade V both

Babinski sign; plantar flexion both,
Clonus; negative both

Sensation; all intact

18 ..54
140/87

144
3.9
24.2
110

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Vol. 10 No.2

3.9

20 ..54
135/85
11.3
0.8
137
4.5
21.9
104
9
4.2
4.1
2.5

IJM

Blood pressure
BUN (mg/dL)
Cr (mg/dL)
Na (mEq/L)
K (mEq/L)
HCO3 (mEq/L)
Cl (mEq/L)
Ca (mg/dL)
Albumin (g/dL)
Phosphorus (mg/dL)
Mg (mg/dL)
Plasma osmole (mOsm/Kg H2O)
Urine osmole (mOsm/Kg H2O)
Urine Na (mEq/L)
Urine K (mEq/L)
Urine Cl (mEq/L)
Urine Cr (mg/dL)

17 ..54
150/90
10.8
0.8
142
2.2
22.1
107
9.5
4.3
1.1
2.3
291
569
45
8
51
264.7

41

IJM

Vol. 10 No.2

CBC : Hb 15 g/dL, Hct 44.9%, WBC 14,900

cells/mm3 ( PMN 72%, Lym 15.9%,
Mo 6.2%, Eo 0.4% Baso 0.5%) Platelet
252,000 cells/mm3
LFT: Chol 144 mg/dL, TP 7.1 g/dL, Alb 4.3 g/
dL, Glob 2.8 g/dL, TB 0.5 mg/dL, DB 0.2
mg/dL, ALT 26 U/L, AST 33 U/L , ALP
75 U/L
PT: 10.4 min, INR 1.02, PTT 28.02 min
TFT: FT3 2.76 (2.3-6.9 pg/mL), FT4 0.835
(0.78-2.11 ng/dL), TSH 0.982 (0.2-3.2
mIU/L)
UA: yellow, clear, sp. gr. 1.012 , pH 7.0, albumin: negative, glucose: negative, ketone:
negative, blood: negative, RBC 0/hpf,
WBC 0-1 /hpf
CK: 251 U/L
EKG: Normal sinus rhythm, normal ST segment
Film chest & LS spine : Normal
:17 ..2554

- oral elixir KCl 30 ml ( KCl 20 mEq)
2 3 doses

- oral PO4 mixture 3 3 doses

- 0.9% NSS 1000 ml + K2PO4 40 mEq iv
60 ml/hr x 1 L

42

1. Proximal muscle weakness

2. Low back pain (
muscle strain)

10 2 - 2554

proximal muscle weakness

myopathy, neuromuscular disorder, neuropathy anterior horn
cell disease myopathy
motor sensory deep
tendon reflex

progressive
muscle weakness long tract sign
cranial nerves
myopathy
periodic paralysis

(sudden onset)


periodic
paralysis 2

1. Primary periodic paralysis

(gene) ion
channels ( sodium channel, potassium channel
calcium channel)

(resting and
action potential)

primary periodic
paralysis hyperkalemic periodic paralysis,
hypokalemic periodic paralysis, paramyotonia
congenital von Eulenburg, Andersens syndrome,
thyrotoxic periodic paralysis, distal renal


10 2 - 2554

43

Vol. 10 No.2

urine potassium > 20 mEq/L 20 mEq/day (

> 15 mEq/day) TTKG > 2)

( 100 mEq)

hypokalemic periodic paralysis (HypoPP), thyrotoxic
periodic paralysis, (alkalemia),
,
-adrenergic agonist, theophyline a-adrenergic antagonist barium
intoxication


hypoPP

IJM

tubular acidosis, X-linked episodic muscle weakness syndrome congenital myasthenic syndrome
1

2. Secondary periodic paralysis

, ,
digitalis, beta-2 agonist, barium
,
,





(hypokalemia)
1

-
periodic paralysis

proximal muscle
weakness

( 2.5
mEq/L)
(22-24 meq/l), thyroid
function test
urine K 8 mEq/L, transtubular potassium
gradient =1.86 [TTKG = (Urine K/Plasma K) /
(urine osmole/plasma osmole)]

IJM

Vol. 10 No.2

1: primary periodic paralysis

: PMC, paramyotonia congenita; ATS, AndersenTawil syndrome; TPP, thyrotoxic periodic

paralysis; DRTA, distal renal tubular acidosis; XLEPMWS, X-linked episodic, prolonged
muscle weakness syndrome; CMS, ongenital myasthenic syndrome; ad, autosomal dominant;
Xl, X-linked; AchE, acetylcholine esterase; uk, unknown; Nm, neuromuscular.

44

10 2 - 2554

Increased Anion Gap

Diabetic Ketoacidosis
Ethylene Glycol
Methanol

Acid-base alterations
Alkalemia
Increased plasma HCO3
Insulin excess
Endogenous
Exogenous
-Adrenergic agonists
Endogenoun
Exogenous
Drugs/Toxins
Barium intoxication
Hypokalemic periodic paralysis
Familial/sporadic
Thyrotoxic

Redistribution

Low Renin
Primary Hyperaldosteronism
Adenoma
Hyperplasia
Glucocorticoid-suppressible

High Aldosterone

Normal Cortisol
High Renin
Exogenous Mineralocorticoid Low Cortisol
Secondary Hyperaldosteronism
Licorice
17 - Hyeroxylase
Renin-Secreting Tumor
Carbenoxolone
Deficiency
Renovascular Disease
Liddles Syndrome
11 - Hydroxylase
Main Renal Arteries
Apparent Mineralocorticoid Deficiency
Small Vessels
Excess Syndrome
Hereditary
Dexamethasone-suppressible
1 : (hypokalemia)

Normal Aldosterone
High Glucocorticoid
Cushings Syndrome
Endogenous
Exogenous
Ectopic ACTH

High BP
Steroid-Excess
Conditions

Normal BP
Diuretics+
Severe K Depletion
Congenital K+ Wasting
Bartters Syndrome
Gittlemans Syndrome
Hereditary K+ Wasting
With Interstitial Disease

Normal Anion Gap

Renal Tubular Acidosis
(Proximal or Distal)
Carbonic Anhydrase Inhibitors
Ureterosigmoidostomy

Normal Acid-Base Status

Acute Tubular Necrosis (Recovery)
Postobstructive Diuresis
Drugs (e.g., Aminoglycosides,
Cisplatin, High-dose Penicillin)
Leukemia
Metabolic Acidosis
Magnesium Depletion

Renal K+ Losses

High Urine CL-(> 10 mEq/L)

Metabolic Alkalosis

Metabolic Alkalosis
Laxative Abuse
Congenital
Chloride losing Diarrhea

Low Urine CL (< 10 mEq/L)

Chroide Depletion Syndrome
Vomiting&Gastric Drainage
Diuretics
Prosthypercapneic
Cl-Losing Diarrhea
Inadequate Cl- Intake
Cystic Fibrosis

Normal Acid-Base
Status
Decreased Intake
Cutaneous Losses
Gastrointestinal Losses
(Geophagia)
Laxative Abuse

Extra renal K Losses

Low Aldosterone (Low Renin)

Metabolic Acidosis
Diarrhea
GI Fistulas
Laxative Abuse

Spurious
High WBC count

Hypokalemia

Vol. 10 No.2

45

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Vol. 10 No.2

IJM

hypoPP
primary periodic paralysis

3.2 mEq/L
1 mEq/L
1-20
15-35
35


proximal myopathy
,
(night rest),
, ,

, ,
b-adrenergic agonist,


,


(bulbar muscles)

(intercellular water accumulation)

(EKG)

flattened T-wave ST-segment
depression

46

10 2 - 2554

hypoPP
hypoPP type-1 mutation
1-subunit calcium channel
hypoPP type-2 mutation
-subunit sodium channel
arginine
(voltage
sensor)

channels
(excitationcontraction coupling)
potassium channel
hypoPP fiber depolarization

depolarization [depolarizing gating pore currents (Igp)]
channel mutation

repolarization

potassium channel [inwardly rectifying potassium channel (Kir) ATP-sensitive

subtype potassium channel (KATP)]
(

3Na+/2K+-ATPase hypokalemia
hypokalemia
Kir6.2 and SUR2A subunits ) fiber
depolarization
(resting membrane potential) -90
mV -60 mV sodium

 (resting membrane potential) -90 mV -60 mV

sodium channel calcium channel ( 2)
calcium
channel

channel
hypoPP

2.

50-100

(provocative
test)

(
2) hypokalemia
1
(
7
)

hy-

poPP

1.

3.
1.5
g/kg
(100
)
test )g/kg

hypokalemia

epinephrine

brachial
(provocative
1.5-3

60

0.1

action
u/kg

30
60
artery
hypokalemia

2-3

potential

1. action potential
2. 50-100 1 ( 7 )
1.5 g/kg (100 ) 30

1.5-3 g/kg

3.

epinephrine

brachial
60

artery

0.1

action

u/kg

30 potential
60

30
action
potential
hypokalemia
, epinephrine

2-3

epinephrine

IJM
Vol. 10 No.2

2:

periodic paralysis
2:
hypokalemic
hypokalemic
periodic paralysis

KCl 60-120 mEq

15-20 KCl 60 mEq

10 2 - 2554

47

IJM

Vol. 10 No.2

KCl
60-120 mEq
15-20 KCl 60 mEq

conductance


(

(intracellular Na+ depletion)
Na+ channel ), beta
adrenergic blocker (non selective beta
adrenergic blocker) ,
minerocorticoid ( spironolactone)
carbonic anhydrase inhibitor acetazolamide dichlorphenamide [ calciumactivated potassium channel (BK channel)
repolarization

mutation
arginine histidine
glycine
]

48

10 2 - 2554

1. ,
Hypokalemia and Hyperkalemia :
Nephrology ,

, 2547: 137-67.
2. . . :
, ,
. . :
, 2543: 164-83.
3. Advanced challenges in resuscitation : Life
threatening electrolyte abnormalities . Circulation. 2000 ; 102 (suppl I) : I-217 22.
4. Brady HR, Wilcox CS. Therapy in nephrology and hypertension. Philadelphia: W.B.
Saunders, 1999: 270-91.
5. Comprehensive clinical nephrology,Fourth
edition , jurgen Floege,Richard J. Johnson,
John Feehally:Disorder of potassium metabolism, page 118-129.
6. Finsterer J. Primary periodic paralyses. Acta
Neurol Scand 2008 ;117:145-58.
7. Halparin ML, Goldstein MB. Fluid, electrolyte, and acid-base physiology. Philadelphia:
W.B.Saunders, 1999: 73-481.
8. Jacobson HR, Striker GE, Klahr S. The principles and practice of nephrology. Missouri:
Mosby-Year Book, 1995: 911-41.
9. Massry SG, Glassock RJ. Textbook of nephrology. Baltimore: Williams & Wilkins, 1995:
276-476.

IJM

10. Narins RG. Clinical disorders of fluid and

eletrolyte metabulism. New York: McGrawHill, 1994: 583-825.
11. Okuda T, Kurokawa K, Papadakis MA.
Fluid & electrolyte disorders. In : Tierney
LM, McPhee SJ, Papadakis MA, eds. Current medical diagnosis & treatment. 37th ed.
London:Prentice Hall, 1998: 824-49.
12. Rose BD. Clinical physiology of acid-base and
electrolyte disorders. New York: McGrawHill, 1994: 515-862.
13. Singer GG, Brenner BM. Fluid and electrolyte disturbances. In: Fauci AS, Braunwald
E, Isselbacher KJ, eds. Harrisons principles
of internal medicine. 14th ed. New York:
McGraw-Hill, 1998: 265-86.
14. Tricarico D, Camerino DC. Recent advances
in the pathogenesis and drug action in periodic
paralyses and related channelopathies. Front
Pharmacol 2011;2:8.

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