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ABSTRACT:

            Hypokalemic periodic palsy a common hereditary disorder encountered in clinical practice. Classical history physical findings, laboratory diagnosis and response to treatment establish the disease. Association of another hereditary cardiac disease (Situs in versus with Dextrocardia) is recorded for the first time.

Key words:  Hypokalemic, Dextrocardia.

INTRODUCTION

            Dextrocardia in situs inverses is one of the earliest known congenital alterations of the heart described in 1943 by the anatomist surgeon. Marco Aurelio Severino. There are three clinically important cardiac malpositions2 i) visceroatrial situs inverses with Dextrocardia (complete situs inverses) ii) Vesceroatrial situs solitorious with right –side heart and apex iii) visceroatrial situs inverses with left sided heart and apex. These cardiac malpositions are associated with various cardiac and non cardiac anatomical detected by chest radiography and surface ECG. Metabolic diseases, epically hereditary diseases like hypokalemic periodic palsy, which is due to abnormality in K+ channel, can alter the presentation of dextrocardia.

CASE REPORT

            A 37 yr Hindu male presented with weakness of both upper and lower limbs for 5 days slurring of speech for 2 days and mild unable to walk from the first day. This was marked at noon when he was taking rest, after lunch. There was no history of trauma convulsion, headache, or root pain. One yr before he surfaces from same type of episodic illness. He was married and had two children.  No family members suffered from this type of illness. On the day of presentation, he was apyrexial. Para nasal sinuses were tender. His higher functions were normal. Power in lower limbs and upper limbs were decreased (2/5). Planter was B/L flexor. Reflexes of both UL and LL were bilaterally diminished. His cranial nerves were normal except mild slurring of speech. Chest examination revealed respiratory rate 24/min and chest expansion <4cm and crepitations over bilateral infrascapular area.

On C.V.S examination, apex was present at right 4^th I.C.S on MCL instead of left side.  All the cardiac sounds were prominent on right side. No murmur of extra heart sounds detected. No abnormally of pulse or B.P. was noted. Fundic gas on percussion was present in the right side and liver dullness on left side.  A structure was felt at Rt. Hypochondrium probably spleen.

On that day, Na+ and K+ were detected as 127 mmol/L respectively Sputum for AFB was sent for 3 consecutive days, which was negative. Other routine investigations were within limits. Chest X- ray showed right-sided cardiac shadow with left sided hepatic shadow.

ECG was done on the day of admission of which P were was inverted in lead I, aVR, aVL; QRS axis was+150⁰ and –ve in lead I, aVL and all chest leads . QT interval normal TU wave was seen in lead I, III, & aVL & U wave in lead II. Nerve conduction study was normal.

On the basis of ECG and laboratory finding the patients was diagnosed as hypokalemic palsy and treated with oral as well as parenteral Potassium Chloride (KCL) and muscle power improved within 24 hr with no residual neurological deficits. Same day, abdominal USG and 2D ECHO, were done, which showed situs inversus with dextrocardia. After KCL treatment, k-level became normal and patient was discharged after recovery.

DISCUSSION

            From 1937 hypokalmeic periodic palsy is a well-known disease3. Usually pattern of inheritance is autosomal dominant with reduced penetrance in women4. Above mentioned male patient who presented with acute onset LMN type of quadriparesis with mild respitory and bulbar muscle involvement after an episode of fever give suspicion about AIDP, through repeated attack of same type of illnmess give suspicion about hypokalemic periodic palsy. Low K+ level normal nerve conduction study and no sensory involvement established the diagnosis and justified by recovery with oral and i.v KCL supplementation . Through hypokalamic periodic palsy may present with ventricular dysarrhythmia and dysmorphic features (Anderson’s syndrome)5 reported earlier dextrocardia association is not described till now. Clinical examination, ECG and X- ray chest PA. view of the patient reveals Dextrocardia with situs inversus, crepitations over the chest and sinus tenderness may be the sign of another two of three cardinal features of Katagener’s trid6, which is autosomal recessive disorder. Through situs inversus is usually associated wit male infertility, this patient is dertile with two children7.

CONCLUSION

            Hypokalemic periodic palsy with dextrocardia with situs inversus may be a coincidence or rare  association reported first time.

REFERENCES:

1. Brown, J.W Congential Heat Disease. London St. Apless Press Ltd, 1950.
2. Van Pragh. R. Winburgs, et al Malposition of the heart in Moss. A.J. Alams et al (Eds) Heart disease in infants, children and adolescents. 4^th edition Baltimore. The Willams & Wikins Company, 1989.
3. Talboot JH Periodic Paralysis : A clinical Syndrome Medicine 20:85, 1994.
4. Fortain B et al: Mapping of hypokalemic periodic paralysis to chromosime 1q3t, q32, Euofan and family nature genet 6:267, 1994.
5. S. Ansone V. Griggs RC, Meolag et al: Anderson’s Syndrome; a distinct periodic paralysis. Ann Neural ; 7: 110 1984.
6. Barnstom W.H. et al; Situs inersus Bronchaactesis and sinusitis; reported of family with 2 cases of Kartagener’s triad pediatrics 6:573, 1950.
7. Afzelius B.A General and Ulterstructural aspects of the immobile cilia syndrome Am of human generic 33:852, 1981.