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A Case of Quadriparesis
1. M2Prof. Dr. S Sundarโs UnitCase Presentation A case of Quadriparesis Presented by Dr.DeepuSebin
2. Shabana , 28 year old female. Housewife from Washermanpet Patient being discharged from Govt. RSRM hospital after Rx for 20 daysandreceived in the ward as case of
3. Clinical History in brief She was an apparently normal ,except for her thin built . First complaint started 1.5 years back as gradually progressing weakness of all four limbs . For which she was treated in a local Hospital with Drugs and IV injections. The weakness lasted for 2 weeks and she gradually recovered with treatment. She was not on any long term drugs.
4. No records were available with the patient except for a nerve conduction study report which says : Nerve conduction study suggestive of demyelinatingradiculopathy (Motor neuronopathy Sensory conduction velocity normal.
5. She was symptom free since this first onset of weakness and conceived for the second time. Antenatal period was uneventful However on day 2 pospartumshe started experiencing some weakness of her limbs and body
6. At presentation Complains of weakness of both upper and lower limbs, progressing over 2 days In upper limbs She has difficulty in mixing food, holding objects, also difficulty is raising arm overhead, combing etc In lower limb Difficulty in gripping chappals, Climbing stairs and sitting up from squatting positions ( All simultaneously) She was confined to bed because of the weakness. History of difficulty in turning side to side in bed, history of difficulty in lifting the neck + No higher mental function defects in history No history suggestive of cranial nerve involvement No sensory, bowel or bladder involvement Patient complained of breathing difficulty. Present even at rest , no orthopnea or PND No h/o of fever or fever preceding the illness
7. Past H/o Similar illness 1.5 years back, probably treated as Demyelinating disease No h/o PTB,CAD,DM,SHT Personal history In Lactation amenorrhea Mixed diet Family History Nil Specific
8. Clinical Examination Patient is thinly built and poorly nourished Conscious, Oriented, Mod dehydrated, Afebrile Dyspneic , Tachypneic PR = 110/min BP = 110/70 Resp Rate: 36/min
9. General Examination No pallor, Icterus, cynosis, cubbing, LNE, pedal odema, Thyroid - Normal CVS Normal RS โ Normal P/A Normal
10. CNS HMF โ Normal CrN โ Normal Spinomotor Bulk โ Tone- decrease b/l Power UL B/L Proximal 4-/5 Distal 4-/5 LL B/L Proximal 3/5 Distal 3/5
15. LFT โ Normal Urine Routine - Normal Diagnosis : Hypokalemic Paralysis (? Periodic Paralysis ) Chest Xray - normal
16. Inj. KCL 40 meq TID was initiaited , since patient was not tolerating oral KCL (Which was later changed to oral KCL) From next day onwards patient started showing improvement in power. Not dyspneic or tachypneic anymore
21. Repeat ABG pH 7.31 Bicarbonate - 14 mmol/1, PCO2 โ 24mmHG Na+ - 133.2 mmol/l K+ - 2.9 mmol/l Cl- - 109 mmol/l Anion gap 14 mmol/1 Non anion gap metabolic acidosis, with respiratory compensation
22. 24 hour Urine K+ - 119 meq/24 hr ***less than 20 mEq/24-hour urine specimen suggests appropriate renal conservation of potassium, while values above that indicate some degree of renal wasting.
23. Urine pH 7.00 ๏ 7.2๏ 7.0 Urine anion gap โ positive Urine [ Na+K โ Cl ] = [40+34-30] = +ve Hypokalemia, Renal K+ loss, Non anionic metabolic acidosis, Normal GFR, Persistently high Urine pH, Positive Urine anion gap. Diagnosis โ Type 1 RTA
24. S.Ca++ - 10.4 mg/dl S.Mg++ - 2.0 mg/dl 24 hour Urine Ca++ -3.6 meq/24hr Thyroid antibodies โ Not done Peripheral smear - Normal Chest, Xray KUB โ Normal USG Abdomen โ Normal. Kidneys normal in size and echotexture. No evidence of calculi MRI Brain with spine screening - normal
25. ANA โ positive 1:100 speckled pattern ds DNA โ Negative Anti SSaโ positive Anti SSb โ negative RA factor โ negative Schrimmers test at opthalmology dept - 4 mm +ve
26. On further enquiry History of dry mouth > 3months. History using water to swallow food + History of dryness of eyes. No history sand / gritty feeling in the eyes No arthralgia, no rash, no photosensitivity, no oral ulcers. No history of palpitations, anxiety, heat intolerance or wt loss.
27. Final Diagnosis Hypokalemic Periodic Paralysis Renal Tubular Acidosis Type 1 Probable Sjogren Syndrome Hyperthyroidism On treatment with T. Carbimazole 5mg 2-2-2 SypKCl 15 ml TID Bicarb Powder 3 sachets/day To review for Rpt electrolytes, ABG and further evaluation for Sjogren Syndrome (Lip Biopsy).
28. THYROTOXICOSIS AND RENAL TUBULAR ACIDOSIS PRESENTING AS HYPOKALAEMIC PARALYSIS C. C. SZETO, C. C. CHOW, K. Y. LI, T. C. KO, V. T. F. YEUNG and C. S. COCKRAM Department of Medicine, Prince of Wales Hospital, The Chinese University of Hong Hypokalemic Paralysis : which is which Atals of Rhuematology
29. Q What is causing the periodic paralysis, Hyperthyroidism or Renal Tubular Acidosis or both ? Why there were no symptoms of hyperthyroidism in this patient ? Why the initial nerve conduction study showed Motor Demyelination ? What awaits the patient ?
30. RTA 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.
47. When to suspect ? Hypo / Hyperkalemia Non Anion Gap Hyperchloremic Metabolic Acidosis with Normal GFR
48. Types of RTA Distal or type 1 RTA Proximal or type 2 RTA Hypoaldosteronism or type 4 RTA What happened to Type3 RTA ? 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)
49. Type 1 RTA - DISTAL Distal (type 1) RTA is Reduction in net secretion of H+ therefore ammonium secretion in the collecting tubules. The impairment in hydrogen ion secretion is manifested as an abnormally high (greater than 5.5) urine pH during systemic acidosis. Decreased proton pump (H-ATPase) activity Increased luminal membrane permeability with backleak of hydrogen ions Diminished distal tubular sodium reabsorption which reduces the electrical gradient for proton secretion
50. Cl- Distal RTA or RTA type 1 Acidification defect H+ HCO3- K+ 1 IDHS excreted
51. Type 1 RTA - DISTAL Distal RTA It is often associated with hypercalciuria due to the effects of chronic acidosis on both bone resorption and the renal tubular reabsorption of calcium. Hypercalciuria contributes to the development of nephrolithiasis and nephrocalcinosis.
52. Major causes of type I (distal) renal tubular acidosis Primary Idiopathic, sporadic ,Familial Secondary Sjรถgren's syndrome Hypercalciuria Rheumatoid arthritis Hyperglobulinemia Ifosfamide , Amphotericin B Cirrhosis Systemic lupus erythematosus (may be hyperkalemic) Sickle cell anemia (may be hyperkalemic) Obstructive uropathy (may be hyperkalemic) Lithium carbonate Renal transplantation
53. Type 2 RTA - PROXIMAL 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. In addition to bicarbonaturia, generalized proximal dysfunction may be associated with one or more of the following: glucosuria, phosphaturia, uricosuria, aminoaciduria, and tubular proteinuria.
54. HCO3 100% HCO3 HCO3 80% reabsorbed 15% reabsorbed HCO3 5% excreted Normal renal tubular function
56. Major causes of type 2 (proximal) renal tubular acidosis with or without the Fanconi syndrome Primary disorders -Idiopathic, sporadic Familial disorders Cystinosis Tyrosinemia Hereditary fructose intolerance Galactosemia Glycogen storage disease (type I) Wilson's disease Acquired disorders Multiple myeloma Ifosfamide Carbonic anhydrase inhibitors Amyloidosis Heavy metals Vitamin D deficiency Renal transplantation Paroxysmal nocturnal hemoglobinuria
57. Type 4 RTA End organ target failure or low aldosterone: Lost of sodium โ hyponatremia Retention or decreased excretion of potassium - hyperkalemia Absorption of chloride โ hyperchloremia Decreased excretion of acids โ metabolic acidosis Loss of fluid - dehydration
58. Na H20 Adolsterone K+ RTA IV: Hypoaldosteronism or pseudohypoaldosteronism Cl- H+ Water Na+
59. Aldosterone deficiency Primary Primary adrenal insufficiency Congenital adrenal hyperplasia, particularly 21-hydroxylase deficiency Isolated aldosteronesynthase deficiency Heparin and low molecular weight heparin Hyporeninemichypoaldosteronism Renal disease, most often diabetic nephropathy Volume expansion, as in acute glomerulonephritis Angiotensin converting enzyme inhibitors , Nonsteroidalantiinflammatory drugs ,Cyclosporine H HIV infection Some cases of obstructive uropathy Aldosterone resistance Drugs which close the collecting tubule sodium channel Amiloride Spironolactone ,Triamterene ,Trimethoprim (usually in high doses) ,Pentamidine Tubulointerstitial disease Pseudohypoaldosteronism Distal chloride shunt
60. Lab Diagnosis of Type 1 & 2 RTA Hypokalemia ABG showing non anionic gap metabolic acidosis 24 hour Urine K+ > 40 showing kidney are losing K+ inspite of low serum K+ *Renal Loss*
61. Urine pH Patients without RTA and normal renal function, the urine pH should be below 5.0 to 5.3 when metabolic acidosis is present. Type : 1 In most cases of type 1 RTA, the urine pH is persistently above 5.3, reflecting the primary defect in distal acidification. Type : 2 Varies
62. Urine Ammonium Excretion Urine Anion Gap is Urine Na+K โ Cl Type 1 RTA โ IDHS โ positive Anion Gap
63. Cl- Distal RTA or RTA type 1 IDHS Acidification defect H+ As NH4 Along with Cl- HCO3- K+ Urine anion gap = [Na+] + [K+] โ [Cl-] excreted
64. Urine Anion Gap 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 . 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. 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)
65. Fractional Excretion of Bicarbonate 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 The urine pH, even if initially acid, will rise rapidly once the reabsorptive threshold for bicarbonate is exceeded. As a result, the urine pH will be above 7.5 and the fractional excretion of bicarbonate (FEHCO3) will exceed 15 to 20 percent.
66. Give more Bicarb HCO3 HCO3 HCO3 15% reabsorbed PCT Bicarb reabsorbing defect Only 30% reabsorbed More bicarb in urine (FE%) pH becomes high Cl- 25% HCO3- K+ 2 IPBR Proximal RTA or RTA type 2
69. Treatment โ Type 1 RTA Bicarbonate wasting is negligible in adults who can generally be treated with 1 to 2 meq/kg of sodium bicarbonate Bicarbonate therapy helps in allowing normal growth to resume, minimizing new stone formation and nephrocalcinosis, decreasing the risk of osteopenia;, lowering inappropriate urinary potassium losses. Potassium citrate, alone or with sodium citrate (Polycitraโข ), is indicated for persistent hypokalemia or for calcium stone disease
70. Treatment โ Type 1 RTA Phosphate and vitamin D supplementation may be necessary to normalize the plasma phosphate concentration. Treatment is more difficult Because bicarbonate give will be lost from PCT Thus, 10 to 15 meqof alkali/kg may be required per day to stay ahead of urinary losses .
75. In patients with type 1 RTA, the urine anion gap is positive, in low urine NH4+ levels. 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.
76. ย 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.
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.
78. Urine anion gap (UAG) Urine anion gap = [Na+] + [K+] โ [Cl-] Normal: zero or positive Metabolic acidosis: NH4+ excretion increases (which is excreted with Cl-) if renal acidification is intact GI causes: โneGUTiveโ UAG Impaired renal acid excretion (RTA): positive or zero Often not necessary b/c clinically obvious (diarrhea)
