A Case of Quadriparesis


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A Case of Quadriparesis

  1. 1. M2Prof. Dr. S Sundar’s UnitCase Presentation<br />A case of Quadriparesis<br />Presented by Dr.DeepuSebin<br />
  2. 2. Shabana , 28 year old female.<br />Housewife from Washermanpet<br />Patient being discharged from Govt. RSRM hospital after Rx for 20 daysandreceived in the ward as case of<br />
  3. 3. Clinical History in brief<br />She was an apparently normal ,except for her thin built .<br />First complaint started 1.5 years back as gradually progressing weakness of all four limbs .<br />For which she was treated in a local Hospital with Drugs and IV injections.<br />The weakness lasted for 2 weeks and she gradually recovered with treatment.<br />She was not on any long term drugs.<br />
  4. 4. No records were available with the patient except for a nerve conduction study report which says :<br />Nerve conduction study suggestive of demyelinatingradiculopathy (Motor neuronopathy<br />Sensory conduction velocity normal.<br />
  5. 5. She was symptom free since this first onset of weakness and conceived for the second time.<br />Antenatal period was uneventful<br />However on day 2 pospartumshe started experiencing some weakness of her limbs and body<br />
  6. 6. At presentation<br />Complains of weakness of both upper and lower limbs, progressing over 2 days<br />In upper limbs<br />She has difficulty in mixing food, holding objects, also difficulty is raising arm overhead, combing etc<br />In lower limb<br />Difficulty in gripping chappals, Climbing stairs and sitting up from squatting positions ( All simultaneously)<br />She was confined to bed because of the weakness.<br /> History of difficulty in turning side to side in bed, history of difficulty in lifting the neck +<br />No higher mental function defects in history<br />No history suggestive of cranial nerve involvement<br />No sensory, bowel or bladder involvement<br />Patient complained of breathing difficulty. Present even at rest , no orthopnea or PND<br />No h/o of fever or fever preceding the illness <br />
  7. 7. Past H/o<br />Similar illness 1.5 years back, probably treated as Demyelinating disease<br />No h/o PTB,CAD,DM,SHT<br />Personal history<br />In Lactation amenorrhea<br />Mixed diet<br />Family History<br />Nil Specific<br />
  8. 8. Clinical Examination<br />Patient is thinly built and poorly nourished<br />Conscious, Oriented, Mod dehydrated, Afebrile<br />Dyspneic , Tachypneic<br />PR = 110/min <br />BP = 110/70<br />Resp Rate: 36/min<br />
  9. 9. General Examination<br />No pallor, Icterus, cynosis, cubbing, LNE, pedal odema, <br />Thyroid - Normal<br />CVS <br />Normal<br />RS <br />– Normal<br />P/A<br /> Normal<br />
  10. 10. CNS<br />HMF – Normal<br />CrN – Normal<br />Spinomotor<br />Bulk – <br />Tone- decrease b/l<br />Power<br />UL B/L<br />Proximal 4-/5<br />Distal 4-/5<br />LL B/L<br />Proximal 3/5<br />Distal 3/5<br />
  11. 11. Reflexes<br />BJ + +<br />TJ + +<br />SJ + -<br />KJ ++ ++<br />AJ + +<br />Superficial refexes present B/L<br />Plantar flexor B/L<br />
  12. 12. Sensory examination – Normal<br />Cerebellum – Normal<br />Skull & Spine -Normal <br />No signs of meningeal irritation<br />
  13. 13. ECG<br />
  14. 14. CBC<br />Hb-10.6 g/dl<br />TC – 8600<br />DC – P64L39<br />ESR -16mm/hr<br />Platelet – 1.4 lakhs<br />RFT<br />B.Urea – 34 mg/dl<br />S. Creatinine – 0.8 mg/dl<br />RBS – 110mg/dl<br />S. electrolytes<br />S.Na+ – 136 mEq<br />S.K+ - 1.8 mEq<br />
  15. 15. LFT – Normal<br />Urine Routine - Normal<br />Diagnosis : Hypokalemic Paralysis (? Periodic Paralysis )<br />Chest Xray - normal<br />
  16. 16. Inj. KCL 40 meq TID was initiaited , since patient was not tolerating oral KCL (Which was later changed to oral KCL)<br />From next day onwards patient started showing improvement in power.<br />Not dyspneic or tachypneic anymore<br />
  17. 17.
  18. 18. T3- 179 (60-200)<br />T4- 15 (4.5-12.0)<br />TSH – 0.02 (0.30 – 5.5)<br />Thyrotoxic Periodic Paralysis<br />
  19. 19. Spot Urine K+ - 48mEq/L<br /> **Low urine potassium (<20 mEq/L) suggests poor intake, a shift into the intracellular space, or gastrointestinal loss. High urine potassium (>40 mEq/L) suggests renal loss.<br />ABG – Compensated Metabolic Acidosis + Respiratory alkalosis. AG normal<br />pH- 7.32<br />Pco2 – 22 <br />HCO3- 14.0<br />Cl - 108<br />Na+ - 136<br />K+ - 1.4<br />
  20. 20. Repeat investigations after stopping K+ supplimentation for 48 hours<br />S. K+ - 2.8<br />S.Na+ - 133<br />S.Ca++ -10.8<br />S.Mg++ - 2.0<br />Spot Urine K+ - 40<br />Spot Urine Na+ - 34<br />Spot Urine Cl- - 30<br />Spot Urine Creatinine – 15.5 <br />
  21. 21. Repeat ABG<br />pH 7.31<br />Bicarbonate - 14 mmol/1, <br />PCO2 – 24mmHG<br />Na+ - 133.2 mmol/l<br />K+ - 2.9 mmol/l<br />Cl- - 109 mmol/l<br />Anion gap 14 mmol/1<br />Non anion gap metabolic acidosis, with respiratory compensation<br />
  22. 22. 24 hour Urine K+ - 119 meq/24 hr<br />***less than 20 mEq/24-hour urine specimen suggests appropriate renal conservation of potassium, while values above that indicate some degree of renal wasting.<br />
  23. 23. Urine pH 7.00  7.2 7.0<br />Urine anion gap – positive <br />Urine [ Na+K – Cl ] = [40+34-30] = +ve<br />Hypokalemia, Renal K+ loss, Non anionic metabolic acidosis, Normal GFR, Persistently high Urine pH, Positive Urine anion gap.<br />Diagnosis – Type 1 RTA<br />
  24. 24. S.Ca++ - 10.4 mg/dl<br />S.Mg++ - 2.0 mg/dl<br />24 hour Urine Ca++ -3.6 meq/24hr<br />Thyroid antibodies – Not done<br />Peripheral smear - Normal<br />Chest, Xray KUB – Normal<br />USG Abdomen – Normal. Kidneys normal in size and echotexture. No evidence of calculi<br />MRI Brain with spine screening - normal<br />
  25. 25. ANA – positive 1:100 speckled pattern<br />ds DNA – Negative<br />Anti SSa– positive<br />Anti SSb – negative<br />RA factor – negative<br />Schrimmers test at opthalmology dept - 4 mm +ve<br />
  26. 26. On further enquiry<br />History of dry mouth > 3months. History using water to swallow food +<br /> History of dryness of eyes. No history sand / gritty feeling in the eyes<br /> No arthralgia, no rash, no photosensitivity, no oral ulcers.<br />No history of palpitations, anxiety, heat intolerance or wt loss.<br />
  27. 27. Final Diagnosis<br />Hypokalemic Periodic Paralysis<br />Renal Tubular Acidosis Type 1<br />Probable Sjogren Syndrome<br />Hyperthyroidism<br />On treatment with<br />T. Carbimazole 5mg 2-2-2<br />SypKCl 15 ml TID<br />Bicarb Powder 3 sachets/day<br />To review for Rpt electrolytes, ABG and further evaluation for Sjogren Syndrome (Lip Biopsy).<br />
  28. 28. THYROTOXICOSIS AND RENAL TUBULAR ACIDOSIS PRESENTING AS<br />HYPOKALAEMIC PARALYSIS<br />C. C. SZETO, C. C. CHOW, K. Y. LI, T. C. KO, V. T. F. YEUNG and C. S. COCKRAM<br />Department of Medicine, Prince of Wales Hospital, The Chinese University of Hong<br />Hypokalemic Paralysis : which is which <br />Atals of Rhuematology<br />
  29. 29. Q<br />What is causing the periodic paralysis, Hyperthyroidism or Renal Tubular Acidosis or both ?<br />Why there were no symptoms of hyperthyroidism in this patient ?<br />Why the initial nerve conduction study showed Motor Demyelination ?<br />What awaits the patient ?<br />
  30. 30. RTA<br />The normal renal response to acidemia (Acid Load) is toreabsorb all of the filtered bicarbonate in PCTand to increase hydrogen excretion (DT) primarily by enhancing the excretion of ammonium ions in the urine in Distal Tubules.<br />
  31. 31. Normal Renal Function<br />Proximal Tubule<br />Reabsorption: <br /><ul><li> HCO3- (90%) – carbonic anhydrase
  32. 32. calcium
  33. 33. glucose
  34. 34. Amino acids
  35. 35. NaCl, water</li></ul>Distal Tubule<br /><ul><li> Na+ reabsorbed
  36. 36. H+ (NH4+ or phosphate salts) excreted
  37. 37. molar competition between H+ and K+
  38. 38. Aldosterone</li></li></ul><li>Normal Renal Function<br />Proximal Tubule<br />Reabsorption: <br /><ul><li> HCO3- (90%) – carbonic anhydrase
  39. 39. calcium
  40. 40. glucose
  41. 41. Amino acids
  42. 42. NaCl, water</li></ul>Distal Tubule<br /><ul><li> Na+ reabsorbed
  43. 43. H+ (NH4+ or phosphate salts) excreted
  44. 44. molar competition between H+ and K+
  45. 45. Aldosterone</li></ul>Type 1 RTA<br />Type 4 RTA<br />Type 2 RTA<br />
  46. 46. 1 IDHS<br />[Type 1 impared distal hydrogen secretion]<br />2 IPBR<br />[Type 2 Impared proximal bicarbreabsorption]<br />
  47. 47. When to suspect ?<br />Hypo / Hyperkalemia<br /> Non Anion Gap<br />Hyperchloremic<br /> Metabolic Acidosis<br /> with Normal <br />GFR<br />
  48. 48. Types of RTA<br />Distal or type 1 RTA<br />Proximal or type 2 RTA<br />Hypoaldosteronism or type 4 RTA<br />What happened to Type3 RTA ?<br />Although initially used to describe a transiently severe form of type 1 RTA in infants, the term type 3 RTA is now most often applied to a rare autosomal recessive syndrome (resulting from carbonic anhydrase II deficiency)<br />
  49. 49. Type 1 RTA - DISTAL<br />Distal (type 1) RTA is <br />Reduction in net secretion of H+ therefore ammonium secretion in the collecting tubules. <br />The impairment in hydrogen ion secretion is manifested as an abnormally high (greater than 5.5) urine pH during systemic acidosis.<br />Decreased proton pump (H-ATPase) activity <br />Increased luminal membrane permeability with backleak of hydrogen ions <br />Diminished distal tubular sodium reabsorption which reduces the electrical gradient for proton secretion<br />
  50. 50. Cl-<br />Distal RTA or RTA type 1<br />Acidification defect<br />H+<br />HCO3-<br />K+<br />1 IDHS<br /> excreted<br />
  51. 51. Type 1 RTA - DISTAL<br />Distal RTA <br />It is often associated with hypercalciuria due to the effects of chronic acidosis on both bone resorption and the renal tubular reabsorption of calcium. <br />Hypercalciuria contributes to the development of nephrolithiasis and nephrocalcinosis.<br />
  52. 52. Major causes of type I (distal) renal tubular acidosis <br />Primary <br />Idiopathic, sporadic ,Familial<br />Secondary <br />Sjögren's syndrome <br />Hypercalciuria<br />Rheumatoid arthritis <br />Hyperglobulinemia<br />Ifosfamide , Amphotericin B<br />Cirrhosis <br />Systemic lupus erythematosus (may be hyperkalemic) <br />Sickle cell anemia (may be hyperkalemic) <br />Obstructive uropathy (may be hyperkalemic) <br />Lithium carbonate <br />Renal transplantation <br />
  53. 53. Type 2 RTA - PROXIMAL<br />Proximal (type 2) RTA may occasionally present as an isolated defect, but is more commonly associated with generalized proximal tubular dysfunction called the Fanconi syndrome. <br />In addition to bicarbonaturia, generalized proximal dysfunction may be associated with one or more of the following: glucosuria, phosphaturia, uricosuria, aminoaciduria, and tubular proteinuria.<br />
  54. 54. HCO3<br />100%<br />HCO3<br />HCO3<br />80% reabsorbed<br />15% reabsorbed<br />HCO3<br />5% excreted<br />Normal renal tubular function<br />
  55. 55. HCO3<br />100%<br />HCO3<br />HCO3<br />30% reabsorbed<br />15% reabsorbed<br />Cl-<br />Decreased proximal tubule efficiency<br />25% HCO3-<br />K+<br />2 IPBR<br />Proximal RTA or RTA type 2<br />
  56. 56. Major causes of type 2 (proximal) renal tubular acidosis with or without the Fanconi syndrome<br />Primary disorders -Idiopathic, sporadic Familial disorders <br />Cystinosis<br />Tyrosinemia<br />Hereditary fructose intolerance <br />Galactosemia<br />Glycogen storage disease (type I) <br />Wilson's disease <br />Acquired disorders <br />Multiple myeloma <br />Ifosfamide<br />Carbonic anhydrase inhibitors <br />Amyloidosis<br />Heavy metals <br />Vitamin D deficiency <br />Renal transplantation <br />Paroxysmal nocturnal hemoglobinuria<br />
  57. 57. Type 4 RTA<br />End organ target failure or low aldosterone:<br />Lost of sodium – hyponatremia<br />Retention or decreased excretion of potassium - hyperkalemia<br />Absorption of chloride – hyperchloremia<br />Decreased excretion of acids – metabolic acidosis<br />Loss of fluid - dehydration<br />
  58. 58. Na<br />H20<br />Adolsterone<br />K+<br />RTA IV:<br />Hypoaldosteronism or pseudohypoaldosteronism<br />Cl-<br />H+<br />Water<br />Na+<br />
  59. 59. Aldosterone deficiency <br />Primary <br />Primary adrenal insufficiency <br />Congenital adrenal hyperplasia, particularly 21-hydroxylase deficiency <br />Isolated aldosteronesynthase deficiency <br />Heparin and low molecular weight heparin <br />Hyporeninemichypoaldosteronism<br />Renal disease, most often diabetic nephropathy <br />Volume expansion, as in acute glomerulonephritis<br />Angiotensin converting enzyme inhibitors , Nonsteroidalantiinflammatory drugs ,Cyclosporine H<br />HIV infection <br />Some cases of obstructive uropathy<br />Aldosterone resistance <br />Drugs which close the collecting tubule sodium channel <br />Amiloride<br />Spironolactone ,Triamterene ,Trimethoprim (usually in high doses) ,Pentamidine<br />Tubulointerstitial disease <br />Pseudohypoaldosteronism<br />Distal chloride shunt<br />
  60. 60. Lab Diagnosis of Type 1 & 2 RTA<br />Hypokalemia<br />ABG showing non anionic gap metabolic acidosis<br />24 hour Urine K+ > 40 showing kidney are losing K+ inspite of low serum K+ *Renal Loss*<br />
  61. 61. Urine pH<br /> Patients without RTA and normal renal function, the urine pH should be below 5.0 to 5.3 when metabolic acidosis is present. <br />Type : 1<br />In most cases of type 1 RTA, the urine pH is persistently above 5.3, reflecting the primary defect in distal acidification.<br />Type : 2<br />Varies<br />
  62. 62. Urine Ammonium Excretion<br />Urine Anion Gap is Urine Na+K – Cl<br />Type 1 RTA – IDHS – positive Anion Gap<br />
  63. 63. Cl-<br />Distal RTA or RTA type 1<br />IDHS<br />Acidification defect<br />H+<br />As NH4<br />Along with <br />Cl-<br />HCO3-<br />K+<br />Urine anion gap = [Na+] + [K+] – [Cl-]<br /> excreted<br />
  64. 64. Urine Anion Gap<br />The urine AG has a negative value in most patients with a normal AG metabolic acidosis due to the appropriate increase in urinary ammonium in an attempt to excrete the excess acid .<br />Ammonium is an unmeasured cation; as a result, an increase in its excretion as NH4Cl will lead to a rise in the urine Cl concentration and a negative urine AG, usually ranging from -20 to -50 meq/L.<br />In comparison, patients with renal failure, type 1 (distal) renal tubular acidosis (RTA), or hypoaldosteronism (type 4 RTA) are unable to excrete ammonium normally. As a result, the urine AG will have a positive value . ( due to decresedCl- excretion into urine)<br />
  65. 65. Fractional Excretion of Bicarbonate<br />The diagnosis of type 2 RTA can be established simply by raising the plasma bicarbonate concentration toward normal (18 to 20 meq/L) with an intravenous infusion of sodium bicarbonate at a rate of 0.5 to 1.0 meq/kg per hour<br />The urine pH, even if initially acid, will rise rapidly once the reabsorptive threshold for bicarbonate is exceeded.<br />As a result, the urine pH will be above 7.5 and the fractional excretion of bicarbonate (FEHCO3) will exceed 15 to 20 percent.<br />
  66. 66. Give more Bicarb<br />HCO3<br />HCO3<br />HCO3<br />15% reabsorbed<br />PCT Bicarb reabsorbing defect<br />Only 30% reabsorbed<br /> More bicarb in urine (FE%)<br /> pH becomes high<br />Cl-<br />25% HCO3-<br />K+<br />2 IPBR<br />Proximal RTA or RTA type 2<br />
  67. 67.                   UHCO3   x   PCr<br />     FEHCO3    =    — — — — — — — — — — —     x    100 <br />                               PHCO3   x   UCr<br /> In type 2 RTA<br />urine pH will be above 7.5 and the fractional excretion of bicarbonat(FEHCO3) will exceed 15 to 20 percent<br />
  68. 68. Urine pH 7.00  7.2 7.0<br />Urine anion gap – positive <br />Urine [ Na+K – Cl ] = [40+34-30] = +ve<br />24 hr urine K+ 119 (high)<br />Hypokalemia, Reanl K+ loss, Non anionic metabolic acidosis, Normal GFR, Persistently high Urine pH, Postive Urine anion gap.<br />Diagnosis – Type 1 RTA<br />
  69. 69. Treatment – Type 1 RTA<br />Bicarbonate wasting is negligible in adults who can generally be treated with 1 to 2 meq/kg of sodium bicarbonate<br />Bicarbonate therapy helps in<br />allowing normal growth to resume, <br />minimizing new stone formation and nephrocalcinosis,<br />decreasing the risk of osteopenia;,<br />lowering inappropriate urinary potassium losses.<br />Potassium citrate, alone or with sodium citrate (Polycitra™ ), is indicated for persistent hypokalemia or for calcium stone disease<br />
  70. 70. Treatment – Type 1 RTA<br />Phosphate and vitamin D supplementation may be necessary to normalize the plasma phosphate concentration.<br />Treatment is more difficult<br />Because bicarbonate give will be lost from PCT<br />Thus, 10 to 15 meqof alkali/kg may be required per day to stay ahead of urinary losses . <br />
  71. 71. Features of the RTA Syndromes<br />
  72. 72. Features of the RTA Syndromes<br />
  73. 73. Thank you<br />
  74. 74. 1 IDHS<br />2 IPBR<br />
  75. 75. In patients with <br />type 1 RTA, the urine anion gap is positive, in low urine NH4+ levels.<br /> In patients with a normal anion gap metabolic acidosis and hypokalemia due to diarrhea, the urine anion gap is negative because urine ammonium excretion rises appropriately in response to the acidosis.<br />
  76. 76.  A normal AG acidosis is characterized by a lowered bicarbonate concentration, which (in the presence of a normal sodium concentration) is counterbalanced by an equivalent increase in plasma chloride concentration. For this reason, it is also known as hyperchloremic metabolic acidosis.<br />
  77. 77. The diagnosis of proximal RTA is made by measurement of the urine pH and fractional bicarbonate excretion during a bicarbonate infusion. The hallmark is a urine pH above 7.5 and the appearance of more than 15 percent of the filtered bicarbonate in the urine when the serum bicarbonate concentration is raised to a normal level.<br />
  78. 78. Urine anion gap (UAG)<br />Urine anion gap = [Na+] + [K+] – [Cl-]<br />Normal: zero or positive<br />Metabolic acidosis: NH4+ excretion increases (which is excreted with Cl-) if renal acidification is intact<br />GI causes: “neGUTive” UAG<br />Impaired renal acid excretion (RTA): positive or zero<br />Often not necessary b/c clinically obvious (diarrhea)<br />