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Year : 2017  |  Volume : 65  |  Issue : 1  |  Page : 184--185

Bartter's syndrome: A rare cause of seizures and quadriparesis

Rajesh Verma, Abdul Qavi, Sudhakar Pandey, Amrit A Bansod 
 Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh, India

Correspondence Address:
Prof. Rajesh Verma
Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh
India




How to cite this article:
Verma R, Qavi A, Pandey S, Bansod AA. Bartter's syndrome: A rare cause of seizures and quadriparesis.Neurol India 2017;65:184-185


How to cite this URL:
Verma R, Qavi A, Pandey S, Bansod AA. Bartter's syndrome: A rare cause of seizures and quadriparesis. Neurol India [serial online] 2017 [cited 2020 Apr 4 ];65:184-185
Available from: http://www.neurologyindia.com/text.asp?2017/65/1/184/198190


Full Text

Sir,

Bartter's syndrome is an autosomal recessive renal tubular disorder with a specific set of abnormal metabolic profile including hypokalemic metabolic alkalosis, hyperreninemia, hyperaldosteronism, and hypomagnesaemia with hypocalciuria.[1] Hypokalemia is a well-known entity associated with neuromuscular dysfunction causing episodic weakness.[2] Secondary hypokalemia due to other systemic disorders such as gastrointestinal, renal, and thyroid disorders can cause episodic weakness, which is four times more common than the primary inherited form of hypokalemic paralysis.[3]

Here, we report a rare presentation of Bartter's syndrome as quadriparesis and seizures along with certain signal intensity changes on magnetic resonance imaging (MRI) of the brain.

A 10-year-old short-statured boy presented with polyuria and polydipsia followed by generalized tonic clonic seizures and quadriparesis. On examination, the patient was having pure motor hyporeflexic quadriparesis without bladder and bowel involvement with normal cognition. General examination was normal except that he had a short stature (height below the third percentile of corresponding age) and low blood pressure (82/50mmHg).

He had profound hypokalemia varying from 1.1 to 1.9 mEq/L with a normal serum sodium. Arterial blood gas analysis showed metabolic alkalosis with a blood pH of 7.49 and bicarbonate level of 29mEq/Lwith a normal anion gap. Serum magnesium level was low (1.1 mEq/L) with raised urinary potassium (29.3mEq/L). There was elevated direct serum renin (50.6 ng/ml) and aldosterone level (39 ng/dl) with hypercalciuria. MRI brain showed T2 hyperintense lesions in the right frontal and parietal areas [Figure 1].{Figure 1}

Potassium replacement was done using intravenous potassium chloride infusion in mannitol initially followed by oral replacement. This treatment resulted in clinical improvement.

The clinical features and laboratory parameters in our patient were in accordance with the diagnosis of Bartter's syndrome; however, we were unable to perform a genetic analysis to confirm the diagnosis.[4] Unusual features in our case were the history of seizures and T2 signal intensity changes on MRI of the brain, which have been earlier reported by Beltagi et al., in Gitelman syndrome.[5] In our case, the brain lesions seen on MRI, were suggestive of cytotoxic edema probably because of metabolic derangement and neuronal cellular edema, which resolved at follow-up in 3 months time.

We intend to create awareness through this case that secondary hypokalemia due to a renal cause is an under-recognized etiology of hypokalemic paralysis and should be suspected in children with quadriparesis and profound hypokalemia with polyuria and polydipsia [Table 1]. This case also highlights the role of arterial blood gas and electrolyte analysis in diagnosing secondary causes of hypokalemic paralysis, which may be associated with other neurological features such as seizures and subtle MRI changes in the brain.[6],[7],[8]{Table 1}

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Kurtz I. Molecular pathogenesis of Bartter's and Gitelman's syndromes. Kidney Int 1998;54:1396.
2Ahlawat SK, Sachdev A. Hypokalemic paralysis. Postgrad Med J 1999;75:193.
3Kumar V, Armstrong L, Seshadri MS, Finny P. Hypokalemic periodic paralysis in rural northern India – Most have secondary causes. Trop Doct 2014;44:33-5.
4Duman O, Koyun M, Akman S, Guven AG, Haspolat S. Case of Bartter syndrome presenting with hypokalemic periodic paralysis. J Child Neurol 2006;21:255-6.
5El Beltagi A, Norbash A, Vattoth S. Novel brain MRI abnormalities in Gitelman syndrome. Neuroradiol J 2015;28:523-8.
6Chianq WF, Lin SH, Chan JS, Lin SH. Hypokalemic paralysis in a middle aged female with classic Bartter syndrome. Clin Nephrol 2014;81:146-50.
7Yin FM, Zhenq FQ, Zhanq X, Wu MJ, Wei HY, Ma ZS, et al. Clinical analysis of 6 cases of Bartter syndrome. Zhonqua Yi Xue Za Zhi 2011;91:528-31.
8Virdi VS, Poddar B, Parmar VR. Hypokalemic respiratory paralysis in Bartter's syndrome. Indian J Pediatr 2002;69:527-8.