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Hypokalemic periodic paralysis: A case series,

review of the literature and update of
management
Article in European Journal of Emergency Medicine February 2010
DOI: 10.1097/MEJ.0b013e32832d6436 Source: PubMed

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Short report 45

Hypokalemic periodic paralysis: a case series, review

of the literature and update of management
Juma M. Alkaabia, Ahmed Mushtaqc, Fatma N. Al-Maskarib, Nagi A. Moussac
and Salah Gariballaa
The objective of this study was to present a case series of
patients with hypokalemic periodic paralysis. We described
all patients with diagnosis of hypokalemic periodic
paralysis admitted to the Al Ain Hospital (UAE) during
the year 2006. Seventeen patients, all males and mostly
Asians, were presented to the Al Ain Hospital over
a 12-month period. The majority were admitted during
the summer months. Four were thyrotoxic. All of the
17 patients received oral potassium supplements and
recovered well without any major complications. In
conclusion, clinicians should have a high index of
suspicion, especially among Asians presenting with flaccid
paralysis and hypokalemia. The main steps in the
management include exclusion of other causes of
hypokalemia, potassium replacement, hydration and close
monitoring of the cardiac rhythm and serum potassium
levels. When possible, the underlying cause must be

adequately addressed to prevent the persistence or

recurrence of paralysis. European Journal of Emergency
c 2010 Wolters Kluwer Health |
Medicine 17:4547
Lippincott Williams & Wilkins.

Introduction

diagnosis of HypoPP for the year 2006 were reviewed.

Alternative causes of hypokalemia or muscular weakness
were excluded.

Hypokalemic periodic paralysis (HypoPP) is a rare disorder

of uncertain cause characterized by potentially fatal
episodes of muscle weakness through the involvement
of the respiratory muscles, and life-threatening cardiac
arrhythmias [1]. It is the most common form of periodic
paralysis with a prevalence estimated to be one per 100 000
[2]. It results from genetic disorders of ion channels called
channelopathies [2]. Acute attacks are often precipitated
by rest after exercise, stress, or a high carbohydrate meal
[1]. HypoPP may be familial with autosomal dominant
inheritance or may be acquired in patients with thyrotoxicosis; it has been estimated that the risk of developing the
disease is 8% in hyperthyroid Japanese men [3].
The treatment of acute attacks is aimed at restoring serum
potassium levels into the normal range. This is best achieved
by the oral administration of potassium. In addition, administration of a nonselective b-blocker is sometimes given in
either familial or thyrotoxic periodic paralysis.
The aim of this study was to describe the presentation,
diagnosis and clinical course of patients presenting to
the Al Ain Hospital with HypoPP over a 12-month period.

Methods
The study was carried out at the Al Ain Hospital
(UAE). All medical records of patients with a discharge
c 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins
0969-9546

European Journal of Emergency Medicine 2010, 17:4547

Keywords: channelopathies, hypokalemic periodic paralysis, neuromuscular
disorders, periodic weakness
Departments of aInternal Medicine, bCommunity Medicine, Faculty of Medicine
and Health Sciences, UAE University and cInternal Medicine Department, Alain
Hospital, Al Ain, UAE
Correspondence to Juma M. Alkaabi, BSc, MBcHB, MRCP, FRCP (London and
Edinburgh), FACP, FACE, Assistant Professor and Consultant Endocrinologist,
Department of Internal Medicine, Faculty of Medicine and Health Sciences,
UAE University, PO Box 17666, Al Ain, UAE
Tel: + 971 3 7137472; fax: + 971 3 7672995; e-mail: j.kaabi@uaeu.ac.ae
Received 16 January 2009 Accepted 26 April 2009

Data were analysed using SPSS (SPSS Inc., Chicago,

USA). The study was approved by the Al-Ain Medical
District Human Research Ethics Committee.

Results
Over a period of 12 months, 17 men were identified
with HypoPP; eight reported previous similar attacks.
Table 1 shows some sociodemographics of the patients.
The majority were hospitalized during the summer
months. Fourteen awoke on the morning of hospitalization with muscle weakness after physically demanding
outdoor work the day before. One patient reported viral
upper respiratory infection and another had a positive
family history of HypoPP.
All reported symmetrical weakness of both upper
and lower limbs, the lower limbs being more
affected (Table 2). On examination, none had sensory
or cranial nerves abnormalities, ocular problems or
evidence of respiratory muscle involvement. Deep
tendon reflexes were decreased in all cases. Four
patients had thyrotoxic periodic paralysis (one was
known case). Subsequent investigations confirmed Graves
disease in three patients and a multinodular toxic goiter
DOI: 10.1097/MEJ.0b013e32832d6436

Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.

46

European Journal of Emergency Medicine 2010, Vol 17 No 1

Table 1

Sociodemographic characteristics of the patients (N = 17)

Variable
Age, median (range) (years)
Male sex
Occupation
Farmer
Skilled laborers
Unskilled laborers
Unknown
Nationality
Bangladeshi
Pakistani
Indian
Philippino
Iranian
Syrian

34 (2050)
17

100

6
6
3
2

35.3
35.3
17.6
11.8

10
3
1
1
1
1

58.8
17.6
5.9
5.9
5.9
5.9

Table 2 Symptoms, physical findings and clinical investigations

at presentation (N = 17)
Variable
Mode of arrival to the emergency room
Walking
In wheelchair
On stretcher
Goiter present
Mean pulse rate
Mean Systolic blood pressure
Mean Diastolic blood pressure
Mean respiratory rate
Mean power in upper limbs
The mean power in lower limbs
Serum potassium (mmol/l)
Electrocardiogram changes
Sinus tachycardia
U waves
Prolonged QT interval
Normal

N or mean SD

5
10
2
4
85 15
127 17.6
77 16.3
20 1.1
3.7 1.36a
2.5 1.17a
1.9 0.43

29.4
58.8
11.8
23.5

1
7
4
9

5.9
41.1
23.5
52.9

MRC, Medical Research Council.

a
MRC rating (normal = 5, no movement = 0).

in one. Two were known to have diabetes mellitus and

were on oral hypoglycemic agents.
All patients were hypokalemic upon admission to the
hospital with a mean serum potassium level being
1.9 mmol/l (normal range: 3.55.5 mmol/l). Other causes
of hypokalemia were excluded. All had electrocardiograms, nine being normal, seven showing U waves, four a
prolonged QT interval and one sinus tachycardia
(Table 2). All patients were admitted to the general
medical ward, none required admission or transfer to the
intensive care unit care. All received an oral potassium
preparation as per the hospitals protocol in the form of
preprepared kay-cee-L (KCL) Syrup 7.5% w/v (Geistlich,
Geistlich Sons Ltd., Long Lane, Chester, UK), 1 mmol
of potassium/1 ml. The initial dose was 3050 ml
(3050 mmol of potassium) with repeat measurements
of serum potassium levels after 60 min and then every
2 h. Additional doses were given depending on serial
measurements of serum potassium. Bed rest and oral
water hydration were encouraged. The thyrotoxic periodic paralysis patients were treated with propanolol and

antithyroid medications and also with oral potassium. The

majority of patients achieved a normal serum potassium
level within 6 h of initiation of the treatment. Once the
serum potassium level was above 3 mmol/l, treatment was
switched to oral KCL tablet supplements. The mean
duration of hospitalization was 2.5 days (range: 18 days).
Recovery was uneventful with no major complications.
Patients were educated with regard to the possibility of
recurrence and were discharged with potassium supplements being prescribed. A diet that is low in sodium and
carbohydrates, but rich in potassium, was recommended
and patients were informed of possible precipitating
factors.

Discussion
On account of population mobility, HypoPP is encountered frequently in clinical practice in the UAE. The gold
standard for a firm diagnosis of HypoPP is a positive
genetic test. Unfortunately, this was not possible in our
setting. All the patients in our study were male, and
hence this may reflect the fact that most of the working
immigrants in the UAE are males. Furthermore, male
dominance in this disorder has been reported because of
decreased penetrance in women [2]. Most admissions
were during the hot summer months and many of our
patients were outdoor workers. Interestingly, there was
no correlation between serum potassium and the severity
of weakness [4].
Early recognition and initiation of appropriate treat
ment are of vital importance. The danger of overdosing
during the treatment of HypoPP with oral potassium
is low compared with intravenous administration, and oral
potassium is well absorbed [5]. For a man with a total
body weight of 60120 kg, 60 mmol is a reasonable initial
dose. Typically, the total dose of potassium should not
exceed 200 mmol in 24 h [6]. Regarding fluids, ample
volume of water is the best option to be taken with the
oral potassium supplementation.
Intravenous potassium use should be spared for potentially life-threatening cases of HypoPP. Where intravenous administration of potassium is deemed essential,
mannitol should be used as the carrier rather than saline
or dextrose, both of which are potential triggers of attacks
[7]. Although intravenous potassium of 10 mmol/h is the
favored rate [8], urgent situations may warrant higher
initial rates of administration [4]. Excess potassium
administration during an acute episode may lead to
posttreatment hyperkalemia [9].
Four of our patients had hypokalemic thyrotoxic periodic
paralysis (TPP). TPP is a rare complication of hyperthyroidism. The exact mechanism by which hyperthyroidism predisposes to HypoPP is not yet well understood.
Although it is known that without treatment, TPP

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Update on hypokalemic periodic paralysis Alkaabi et al. 47

victims may spontaneously recover muscle strength over

a period of 36 h as potassium shifts out of cells into
the extacellular space [10], treatment as outlined above
is advisable.
Although HypoPP is a rare condition, clinicians should
have a high index of suspicion especially among Asians
presenting with flaccid paralysis and hypokalemia.
Thyroid function tests should be carried out routinely.
The treatment of HypoPP includes potassium replacement, hydration and close monitoring of the cardiac
rhythm and serum potassium levels. When possible, the
underlying cause must be adequately addressed to
prevent the persistence or recurrence of paralysis.
Patients need to be educated with regard to the
possibility of recurrence and should be prescribed
potassium supplements on discharge.

Acknowledgement
Disclosure: the authors have no conflicts of interest to
disclose.

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