BRIEF REPORTS Primary Hypokalemic Periodic 3-4 days every time. Most of the episodes Paralysis started in the early morning hours without any particular precipitating factor. The patient used to have 3-4 such episodes every year. One of the attacks also involved res- piratory muscles. The general examination V.P. Dandge was normal except for the presence of W.B. Pagarkar bilateral phlyctenular conjunctivitis. There M. Agarwal was hypotonia and proximal muscle weak- V.R. Dharnidharka ness in the limbs except right upper limb S.P. Rathi which was normal. Deep tendon jerks were depressed with bilateral flexor planters. No other neurological signs were present. On investigations, the tuberuclin test Primary hypokalemic periodic paralysis showed an induration of 20 mm. As shown (PHPP) is a rare entity first described by in Table I, the patient had a consistent Shakanowitch in 1882. Only a few cases of hypokalemia in the first week of hospitali- PHPP have been reported in Indian litera- zation along with ECG showing prominent ture in adults(l). In children hypokalemic 'U' waves till the fifth day. Initial arterial paralysis secondary to gastroenteritis and blood pH was normal. Other related inves- chronic renal disease is much more com- tigations were normal. mon than primary disease(2). We hereby report a case of PHPP in a child, success- Considering the clinical pattern and fully managed with acetazolamide and oral biochemical abnormalities, a diagnosis of potassium supplementation. primary hypokalemic periodic paralysis was made and the patient was put on oral Case Report potassium supplements and acetazolamide An 11-year-old boy weighing 15 kg (30 mg/kg/day) from the second day of presented with complaints of recurrent hospitalization. Clinical recovery started attacks of quadriparesis since 3 years of from fourth day onwards gradually with age. Each episode used to start with sym- return of normal power in all the limbs by metrical lower limb weakness progressing the 7th day. Alternate day investigations till to the upper limbs over a period of 3-4 recovery revealed corresponding rise in hours. Spontaneous recovery occurred over serum potassium values to near normal levels by the end of the first week (Table I). On therapy the patient also developed From the Department of Pediatrics, T.N. Medical metabolic acidosis (Table I) which resolved College and B.Y.L. Nair Ch. Hospital, after omitting acetazolamide after two days Bombay 400 008. of recovery. In addition, the patient was Reprint requests: Dr. V.P. Dandge, Professor given antitubercular treatment for phlyctenu- of Pediatrics, T.N. Medical College and lar conjunctivitis and a strongly positive B.Y.L. Nair Ch. Hospital, Dr. A.L. Nair Mantoux test. Road, Bombay 400 008. Received for publication: September 3, 1993; After recovery, electromyogram and Accepted: July 8, 1993 nerve conduction studies were done which 326 were normal as also was the muscle biopsy. and normal potassium values in between Both parents were asymptomatic with nor- attacks points to a primary cause in the mal serum potassium values. During a one present case. Though the child also had tu- year long follow up on oral potassium berculosis, the association appears inciden- supplementation, the patient had no relapse, tal. No case of HPP in association with tu- in contrast to a frequency of 3-4 attacks/ berculosis has been reported in literature. year before treatment. Periodic serum Though the exact etiology of hypokale- potassium estimations were also within mia in PHPP is not known, a possibility of normal limits. abnormally reduced surface membrane Discussion permpability to potassium in muscles has been proposed(4). Thus, low serum potas- Familial or PHPP is an autosomal sium coupled with high muscle potassium dominant disorder with incomplete pene- levels produce hyperpolarisation of the trance in females or rarely sporadic in 20% muscle membrane, making it inexcitable(4). of the cases. In the present case no family Accordingly, acetazolamide has been used member was affected. The first attack oc- in the treatment as it causes mild metabolic curs before 16 years of age in 60%(3). acidosis driving the potassium out of the Involvement of respiratory muscles is usu- cell(5,6). In one case clinical recovery and ally uncommon during an attack(3). Our rise in serum potassium level was associat- child however, did have a history suggestive ed with progressively developing acidosis of one such self limiting attack in the past. after starting acetazolamide therapy. Once Though attacks can be induced by cold, the recovery is complete, acetazolamide can emotional excitement, rest after exercise and be omitted with continuation of potassium a high carbohydrate meal—our case had supplementation without a risk of recur- no precipitating factor. rence, as the present case has been asyp- Diagnosis of PHPP rests upon exclud- tomatic on follow up with only one potas- ing secondary causes of hypokalemia, e.g., sium supplementation. Returning of blood gastroenteritis, hyperaldostereronism, renal pH to normal after stopping acetazolamide tubular acidosis, etc.(3). Intermittent attacks was not associated with clinical recurrence. 327 BRIEF REPORTS Low carbohydrate low sodium diet, spirono- 2. Subba Rao SD, Rekha S, Chandrashek- lactone and diclofenamid have also been har MK. Hypokalemic Paralysis. Indian tried in the management of HPP(7). None Pediatr 1991, 28: 425-426. of these measures prevent progressive 3. Fenichel GM. Flaccid limb weakness in myopathic changes. childhood. In: Clinical Pediatric Neurolo- gy, 2nd edn. Philadelphia, WB Saunders To conclude, in addition to rarity, the Co, 1993, pp 192-193. present case also emphasizes the usefulness of acetazolamide in the treatment and the 4. Grob D, Johns RJ, Liljestrand A. Potas- case with which recurrent attacks can be sium movement in patients with hypokale- mic periodic paralysis. Am J Med 1957, prevented by potassium supplementation. 23: 356-357. Acknowledgements 5. Griggs RC, Engel WK, Resnick JS. Ace- The authors acknowledge the help of tazolamide treatment of hypokalemic their Dean, Dr. K.D. Nihalani, who gave period paralysis. Ann Intern Med 1970, 73: 39-40. them her kind permission to publish this case report. 6. Vroom FFQ, Jarrell MA, Maren TH. Acetazolamide treatment of hypokalemic REFERENCES period paralysis: Probable mechanism of 1. Muralikrishna GS, Yasoda T, Subhash S, action. Arch Neurol 1975, 32: 385-386. Venkatesan S, Sayeed ZA, Rajagopalan 7. Sander C. Therapy of paroxysmal hy- RS. Familial hypokalemic periodic paral- pokalemic paralysis: Experience with di- ysis. J Assoc Physicians India 1983,5: 316- clofenamid. Monatsschr Kinderheilkd 318. 1988, 136: 149-150. Hyperimmunoglobulin E presents with repeated pulmonary infec- Syndrome tions, pyoderma, otitis media and subcuta- neous abscesses, in the child who has coarse features, short stature, eczema and ungal candidiasis. Serum IgE levels are usually greatly raised. Although 130 cases have been A.V. Pherwani reported world-over, to the best of our C. Rodrigues A. Dasgupta From the Department of Pediatrics, National Health M.A. Bavdekar and Education Society, P.D. Hinduja Hospital N.D. Rao and Medical Research Centre, Bombay. Reprint requests: Dr. Asha V. Pherwani, 303, Samudra Mahal, Shivsagar Estate, Worli, Bombay 400 018. Hyperimmunoglobulin E syndrome (HIE) Received for publication: December 14, 1992; is a rare immunodeficiency disorder which Accepted: July 9, 1993 328 .