A 28year old normotensive euthyroid male presented with recurrent lower motor neuron type of weakness without any sensory or autonomic involvement, with preserved reflexes. Systemic examination was significant for a mild hepatosplenomegaly. Investigations revealed persistent hypokalemic, hyperchloremic, normal anion gap metabolic acidosis with deranged liver functions. Urine pH was 6.0 even after an oral ammonium-chloride loading test. A case of Type I Renal Tubular Acidosis (RTA) was diagnosed. A search for the etiology revealed bilateral Kayser-Fleischer (KF) ring, with low serum ceruloplasmin levels and high urinary copper , confirming it to be Wilson's Disease (WD). A 28 year-old male farmer was admitted with weakness of all the four limbs. To start with, the weakness involved the lower limbs and then progressed rapidly to involve the upper limbs making the patient bed–bound within the next day. The weakness involved both proximal and distal group of muscles and was associated with significant flaccidity but without fasciculations, sensory abnormalities or diurnal variation. There was no history suggestive of alteration of higher functions, cranial nerve or sphincter involvement and of radicular pain or girdle sensation. Weakness was not preceded by vaccination, respiratory tract infection or diarrhoea and was not associated with food intake, exercise or change of temperature. He denied unexplained sweating, tremors, heat intolerance, prolonged vomiting, diabetes, hypertension or any drug intake over a prolonged period of time. The patient had 2 such similar attacks in the past, but those were milder, subsided spontaneously and didn't require hospitalization. His personal and family history was noncontributory. The patient had a normal higher function, and normal vitals with blood pressure of 110/80mm Hg. The neurological examination revealed marked hypotonia and weakness in all four limbs, the power being 1/5 in the lower and 2/5 in the upper limbs. There was no cranial nerve involvement, sensory impairment and no abnormality in any of the superficial or deep reflexes. Plantar reflexes were bilaterally flexor and coordination could not be tested. Both the lobes of liver were palpable 3c.m below the costal margin with firm consistency. He had a mild splenomegaly without any venous prominence and clinical evidence of free fluid in the abdomen. The complete blood count, serum urea and creatinine were normal but the fasting plasma glucose was 182mg/dl and the post-challenge plasma glucose was 214mg/dl. The liver function test revealed the following: Bilirubin-0.9mg/dl, AST-168U/L, ALT-58U/L, Albumin-2.2gm/dl and Globulin-4.1gm/dl. The serum sodium, potassium, chloride and bicarbonate were 140mEq/L, 2.8mEq/L,108.1mEq/L and 20.4mEq/L respectively. The arterial blood gas analysis suggested mild metabolic acidosis with normal anion gap. A provisional diagnosis of hypokalemic paralysis was considered and the electrolyte deficit was corrected with oral and intravenous potassium supplementation. The patient improved completely but a repeat estimation after 4 days showed a persistent hypokalemia (K=2.7meq/L) and metabolic acidosis. Measured calcium (9.1mg/dl) and magnesium (1.9mg/dl) were within normal limits. He had a normal thyroid profile. Serum HIV serology, Serum electrophoresis , Creatine Kinase, Aldolase, Porphobilinogen, Cerebrospinal fluid study and Electrophysiological studies were all normal. 24 hours urinary potassium excretion was 242.76mEq. Acute Flaccid Quadruparesis In A Young Male: A Case Report 2 of 4 Plasma and urinary osmolality were 296.76mosm/kg and 482.2mosm/kg respectively. Estimated transtubular potassium gradient(TTKG) came out to be 15.25. To proceed with the diagnosis of a case of hypokalemia, we followed the algorithm given in Figure 1(1). A potassium excretion >15mmol/day and a TTKG of more than 4 narrowed our possibilities further. A diagnosis of renal tubular acidosis (RTA) was made and oral NH4Cl test (0.1gm/kg) revealed worsening of acidosis(7.377 to 7.289) with a urinary pH of 6.
Read full abstract