3. Introduction
Acute flaccid paralysis
Prevalence 1:100.000
Onset in 1st-2nd
decade of life
Man > female
Decrease QOL, life-
threatening proper
management
Primary and
secondary
hypokalemia
resolve basic etiology
Periodic
Paralysis
Hypokalemia (HPP)
4. Objective
To present the description, diagnosis, and
management of hypokalemia periodic paralysis
Identification etiologies and risk factors to prevent
recurrence of this disease.
6. Patient’s Identity
• Name : GFA
• Sex : Female
• Age : 11 yo
• Date of birth : January 4rd, 2008
sudden onset of weakness in both arms
and legs since one day before admission
Chief
Complaint
7. Weakness of legs
and arms, can’t
walk, but can raised
hands, symmetrical
no fever, no
lateralization, fully
alert, normal
swallowing and
respiratory function.
polyuria, normal
defecation, no
tingling or
numbness
One day before admission…
Sudden onset,
3rd attack which required
hospitalization
History of Present Illness
8. History of Past Illness
• Tumor
suprasellar
• Brain CT scan &
MRI
• Steroid
February
2019
• Hypothyroidism
• Levothyroxine
February
2019
• Polyuria
• Diabetes
insipidus
• Desmopressin
March 2019
12. PHYSICAL EXAMINATION
THE DAY OF ADMISSION
Blood
Pressure
96/63
mmHg
Heart
Rate
100
beat/min
Respiratory
Rate
20 breath/min
Temp
36.9o C
CRT
< 2 sec
VITAL SIGNS
(P50-90)
13. Head Normocephaly, no deformity
Eye
Conjunctiva was not pale, sclera was not icteric,
normal movement, pupil round 3mm/3mm, normal
pupillary response, hemianopsia
Neck
Normal lymph node, thyroid glands were not
palpable
Lung Normal lung sound
Heart Normal heart sound
Abdominal
Not distended, normal abdominal sound, no
hepatosplenomegaly, no tenderness, normal turgor
Extremities Warm, CRT <2 seconds, no edema,
Physical examination
14. Neurological examination
• No cranial nerve palsy
• Motoric
• Physiological reflex
• No pathological reflex
• Sensory : no numbness or tinngling
3333 3333
1111 1111
+ +
+ +
21. Definition
• HPP is characterized by muscle weakness secondary to
low serum potassium levels (<3.5 mmol/L)
• The episodes develop over minutes to hours and last
several minutes to several days with spontaneous
recovery in 3-36 hours.
22. Epidemiology
• Prevalence 1:100.000
• Familial hypokalemic paralysis is often found among
Caucasians
• Thyrotoxic periodic paralysis is often in the Asian
population with male to female ratio 70:1.
• The age of onset of hypokalemic periodic paralysis is
mostly in the first to second decade.
23. • Two types: Primary HPP and secondary HPP.
• Primary etiology can be sporadic or familial in autosomal
dominant inheritance
• Mutation of calcium channel gene (CACNA1S) and
sodium channel (SCN4A) related to primary HPP
• Secondary etiologies : renal tubular acidosis, Gitelman
syndrome, Bartter syndrome, hyperaldosteronism,
hyperthyroidism, medication use, and gastrointestinal loss.
Etiology
24. Risk factor
• Attacks may be triggered by rest after vigorous exercise,
stress, or a high-carbohydrate meal, often after a delay
of several hours.
• These events are often associated with an increased
release of epinephrine or insulin, both of which cause
movement of potassium into cells and low potassium
blood levels
25. Pathogenesis
Maintain the K+ cell membrane gradient (K+ inside > K +
outside) to maintain potential intermembrane differences.
Hyperkalemia will decrease the potential action of the membrane,
whereas hypokalemia causes hyperpolarization and membrane
unresponsiveness, so the muscles cannot be excitated.7
26. Clinical manifestation
• Decreased muscle tone (flaccidity)
• Bilateral, symmetric, proximal muscle > distal muscles
• Deep tendon reflex normal or decrease
• Plantar reflex normal
• The typical evolution of symptoms is as follows:
– Rapid installation (over minutes or over hours)
– Duration of several minutes to several days
– Spontaneous recovery
– Symptoms tend to occur : At rest after strong physical exertion,
fever, mental stress, on awakening after a carbohydrate-rich meal
the previous evening, after prolonged immobility.
27. Diagnosis
Consensus diagnostic criteria
Two or more attacks of muscle weakness with serum potassium <3.5 mEq/L
OR
One attack of muscle weakness in the proband and one attack of weakness
in one relative with documented serum potassium <3.5 mEq/L OR
Three or more of the following six clinical/laboratory features:
1. Onset in the first or second decade
2. Duration of attack > two hours, involving ≥1 limbs
3. The presence of triggers
4. Improvement in symptoms with potassium intake
5. A family history of the condition or genetically confirmed skeletal
calcium or sodium channel mutation
6. Positive long exercise test
28. Treatment
3.0-3.5
mEq/L
• KCl 0,25
mEq/kg
• 1 hour
2.5-3.0
mEq/L
• KCl 0,5
mEq/kg
• 2 hours
< 2.5 mEq/L
• KCl 0,75
mEq/kg
• 3 hours
• The goal is the prevention of potentially life-threatening cardiac
conduction disturbances and neuromuscular dysfunction by raising serum
potassium to a safe level.
• Safety approach 0,5 mEq/kg, with maximal dose 10 mEq/hour
• Nonurgent hypokalemia is treated with 40 to 100 mmol of oral potassium
per day over days to weeks
29. Tumor suprasellar
• One of brain tumor, frequently
found in children
• Located in sella tursica near
pituitary gland5
• Lession can affect chiasma
opticum, infundibulum,
hypothalamus, and ventricle.
• Cause visual impairment,
endocrine dysfunction,
hydrocephalus.22
32. Fully alert
No convulsion
No lateralization of
brain function
Reflex <<
•
sudden onset of
weakness in both arms
and legs, symmetrical,
no trauma
Bowel and urinary
movement normal
No tingling
No numbness
No pain
No ptosis
Normal swallowing
No diplopia
11 yo
Central
origin
Peripheral
nerve
Spinal cord
Neuromuscular
junction disorder
Muscular
problem Toxic myositis
Post viral myositis
Periodic paralysis hypokalemia
33. 11 yo
sudden onset of
weakness in both arms
and legs, symmetrical,
no trauma
3rd attack need
hospitalization
Potassium
level 2.0 mEq/L
Periodic paralysis hypokalemia
1st – 2nd decade of life
0.5 mEq/kg 20.5 meq KCl iv
3 hours
KCl oral
maintenance
35. Prevention
PREVENT
• Unusually strenuous effort
• Excess of carbohydrate-rich meals
or sweets
• Stress/excitement/fear
• High salt intake
• Prolonged immobility
• Oral or intravenous glucosteroids
• Use of glucose infusion, excessive
sodium-containing fluids
• Use of alcohol
• Proper medication
Sejak satu hari SMRS kaki sama sekali tidak dapat digerakkan, terasa lemas, sehingga pasien tidak dapat berjalan. Bersamaan dengan itu terdapat pula kelemahan lengan, namun lengan masih dapat diangkat. Kelemahan dirasakan sama pada sisi kanan dan kiri. Tidak ada demam, wajah mencong, penurunan kesadaran, sesak napas, muntah maupun riwayat trauma sebelumnya. Buang air kecil (BAK) banyak dan buang air besar (BAB) tidak ada keluhan. Pasien juga masih dapat menelan makanan dengan baik. Tidak ada rasa baal atau kesemutan di tangan dan kaki.