DR. PRIYA KUBENDIRAN M 1 UNIT PROF. DR. MAGESH KUMAR  CASE PRESENTATION
A 45 year old female was admitted on 08.11.10 with <ul><li>C/O :  Difficulty in walking  -  6 months </li></ul><ul><li>Dif...
H/O PRESENT ILLNESS : <ul><li>6 months ago, the patient noticed that she had difficulty climbing stairs </li></ul><ul><li>...
<ul><li>h/o difficulty in turning from side to side in bed </li></ul><ul><li>h/o difficulty in getting up from bed </li></...
<ul><li>h/o abdominal pain </li></ul><ul><li>upper abdomen, burning type </li></ul><ul><li>h/o constipation + </li></ul><u...
<ul><li>PAST HISTORY : </li></ul><ul><li>No h/o similar illness </li></ul><ul><li>Not a known case of DM/SHT/PT/BA/IHD </l...
SUMMARIZING…. <ul><li>A  45 year old female has presented with gradually progressive weakness of proximal muscles over the...
 
<ul><li>Endocrine – thyroid </li></ul><ul><li>parathyroid </li></ul><ul><li>adrenal </li></ul><ul><li>Drug induced myopath...
GPE : <ul><li>Conscious, oriented  VITALS : </li></ul><ul><li>Afebrile  </li></ul><ul><li>Voice was hoarse  PR – 76/ min <...
 
 
 
SYSTEMIC EXAMINATION : <ul><li>RS  -  NVBS  heard </li></ul><ul><li>no added sounds </li></ul><ul><li>CVS  -  S1 S2 heard ...
CNS <ul><li>HMF -  normal </li></ul><ul><li>CRANIAL NERVES  -  normal </li></ul><ul><li>MOTOR SYSTEM – </li></ul>RIGHT LEF...
POWER RIGHT LEFT Neck 2 UL Shoulder Flexors 3 3 Extensors 3 3 Abduction 3 3 Adduction 3 3 Elbow 4- 4- Wrist 5 5 Hand grip ...
REFLEXES RIGHT LEFT LL Knee 3 3 Ankle 5 5 EHL 5 5 RIGHT LEFT SUPERFICIAL Corneal + + Conjuctival + + Abdominal - - Plantar...
<ul><li>Coordination – impaired due to weakness </li></ul><ul><li>Gait – walks with support </li></ul><ul><li>SENSORY  -  ...
PROBABLE  DIAGNOSIS…??
<ul><li>Endocrine – thyroid </li></ul><ul><li>parathyroid </li></ul><ul><li>adrenal </li></ul><ul><li>Drug induced myopath...
IMPRESSION : <ul><li>PROXIMAL MUSCLE WEAKNESS + </li></ul><ul><li>DELAYED RELAXATION OF DTR </li></ul><ul><li>NO SENSORY /...
INVESTIGATIONS : <ul><li>CBC </li></ul><ul><li>Hb –  10.7  gm% </li></ul><ul><li>TC – 6600 </li></ul><ul><li>DC – P70 L30 ...
<ul><li>ECG  – NSR, complete RBBB </li></ul><ul><li>CXR  – Lt side eventration of diaphragm </li></ul><ul><li>ELISA for HI...
 
<ul><li>USG ABDOMEN  – normal study </li></ul><ul><li>ECHO  -  </li></ul><ul><li>no RWMA </li></ul><ul><li>normal LV systo...
Neurological consult : <ul><li>Proximal myopathy +  </li></ul><ul><li>Slow relaxation of ankle jerk + </li></ul><ul><li>Ho...
<ul><li>TFT : </li></ul><ul><li>T3  <  10  ng/dl  (60-200) </li></ul><ul><li>T4  <  0.30  μ g/dl  (4.5 – 12) </li></ul><ul...
NCS <ul><li>Motor (CMAPs)  &  sensory  (SNAPs) </li></ul><ul><li>NORMAL </li></ul>
EMG <ul><li>EMG done in Rt quadriceps, Lt quadriceps, Rt biceps, </li></ul><ul><li>Rt EDB </li></ul><ul><li>In the above m...
SPONTANEOUS  ACTIVITY
MOTOR  UNIT  POTENTIALS
INTERFERENCE  PATTERN
FINAL DIAGNOSIS <ul><li>HYPOTHYROIDISM </li></ul><ul><li>HYPOTHYROID  MYOPATHY </li></ul><ul><li>EVENTRATION OF LEFT DIAPH...
MANAGEMENT <ul><li>T. Eltroxin 100  μ g  od  </li></ul><ul><li>250  μ g od </li></ul><ul><li>T. Ranitidine 150 mg 1 bd </l...
COURSE IN THE HOSPITAL
 
 
 
<ul><li>Neuromuscular symptoms present in 30 – 80 % of patients with hypothyroidism </li></ul><ul><li>Weakness is observed...
PATHOPHYSIOLOGY <ul><li>Muscle involvement may be caused by  </li></ul><ul><li>changes in muscle fibres from fast twitchin...
PSEUDOMYOTONIC  REFLEX <ul><li>The rate-limiting step in muscle relaxation is reuptake of calcium by the sarcoplasmic reti...
<ul><li>serum CK  levels -  2 to 6 fold though marked elevation up to the range of 20,000- 25,000 U/L may be seen (thyroid...
 
<ul><li>NCS  – normal </li></ul><ul><li>may show f/s/o mild neuropathy </li></ul><ul><li>Biopsy  – non specific changes. <...
MANAGEMENT <ul><li>Levothyroxine replacement </li></ul><ul><li>Myopathy improves within 2-3 weeks, but may take months to ...
 
<ul><li>References : </li></ul><ul><li>Harrison 17/e </li></ul><ul><li>Emedicine sources </li></ul><ul><li>DeJong </li></ul>
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A Case of Hypothyroid Myopathy

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A Case of Hypothyroid Myopathy

  1. 1. DR. PRIYA KUBENDIRAN M 1 UNIT PROF. DR. MAGESH KUMAR CASE PRESENTATION
  2. 2. A 45 year old female was admitted on 08.11.10 with <ul><li>C/O : Difficulty in walking - 6 months </li></ul><ul><li>Difficulty in lifting her arms – 5 months </li></ul><ul><li>Difficulty in holding her head erect – 4 months </li></ul>
  3. 3. H/O PRESENT ILLNESS : <ul><li>6 months ago, the patient noticed that she had difficulty climbing stairs </li></ul><ul><li>difficulty in getting up from squatting position </li></ul><ul><li>difficulty in walking </li></ul><ul><li>Around a month later, she was unable to lift her arm above her head </li></ul><ul><li>After 2 months she was unable to hold her head erect </li></ul><ul><li>Gradually progressive weakness over the past 6 months </li></ul><ul><li>No history s/o distal weakness of upper & lower limbs </li></ul>
  4. 4. <ul><li>h/o difficulty in turning from side to side in bed </li></ul><ul><li>h/o difficulty in getting up from bed </li></ul><ul><li>No h/o noticing any difference in limb size </li></ul><ul><li>No history s/o cranial n involvement </li></ul><ul><li>No h/o sensory disturbances </li></ul><ul><li>No h/o bowel/bladder disturbances </li></ul><ul><li>No h/o cramps/ pain in the limbs </li></ul><ul><li>No h/o diurnal variation </li></ul><ul><li>h/o facial puffiness + </li></ul><ul><li>h/o retrosternal chest pain </li></ul><ul><li>burning, aggravated by food </li></ul>
  5. 5. <ul><li>h/o abdominal pain </li></ul><ul><li>upper abdomen, burning type </li></ul><ul><li>h/o constipation + </li></ul><ul><li>h/o loss of appetite </li></ul><ul><li>No h/o loss of weight </li></ul><ul><li>No h/o fever </li></ul><ul><li>No h/o skin rash / joint pain </li></ul>
  6. 6. <ul><li>PAST HISTORY : </li></ul><ul><li>No h/o similar illness </li></ul><ul><li>Not a known case of DM/SHT/PT/BA/IHD </li></ul><ul><li>No h/o any drug intake / surgeries </li></ul><ul><li>PERSONAL HISTORY : </li></ul><ul><li>Mixed diet </li></ul><ul><li>Unmarried </li></ul><ul><li>Attained menopause before 4 years </li></ul><ul><li>Not a tobacco/betel nut chewer </li></ul><ul><li>FAMILY HISTORY : </li></ul><ul><li>No h/o similar illness </li></ul>
  7. 7. SUMMARIZING…. <ul><li>A 45 year old female has presented with gradually progressive weakness of proximal muscles over the past 6 months </li></ul><ul><li>POSSIBILITIES….??? </li></ul>
  8. 9. <ul><li>Endocrine – thyroid </li></ul><ul><li>parathyroid </li></ul><ul><li>adrenal </li></ul><ul><li>Drug induced myopathy </li></ul><ul><li>Inflammatory – Polymyositis / dermatomyositis </li></ul><ul><li>Infectious </li></ul><ul><li>Connective tissue disorders - SLE </li></ul><ul><li>Myasthenia gravis </li></ul><ul><li>Inherited - Limb girdle muscular dystrophy </li></ul>
  9. 10. GPE : <ul><li>Conscious, oriented VITALS : </li></ul><ul><li>Afebrile </li></ul><ul><li>Voice was hoarse PR – 76/ min </li></ul><ul><li>Hydration fair BP – 110/70 mm hg </li></ul><ul><li>Facial puffiness RR – 16 cycles / min </li></ul><ul><li>Pale </li></ul><ul><li>Macroglossia </li></ul><ul><li>Anicteric </li></ul><ul><li>No neck swelling </li></ul><ul><li>No cyanosis/clubbing </li></ul><ul><li>Icthyosis in lower limbs </li></ul><ul><li>No pedal edema </li></ul><ul><li>No lymphadenopathy </li></ul>
  10. 14. SYSTEMIC EXAMINATION : <ul><li>RS - NVBS heard </li></ul><ul><li>no added sounds </li></ul><ul><li>CVS - S1 S2 heard </li></ul><ul><li>no added sounds </li></ul><ul><li>P/A - distended </li></ul><ul><li>divarication of recti + </li></ul><ul><li>soft, epigastric tenderness + </li></ul><ul><li>no organomegaly </li></ul>
  11. 15. CNS <ul><li>HMF - normal </li></ul><ul><li>CRANIAL NERVES - normal </li></ul><ul><li>MOTOR SYSTEM – </li></ul>RIGHT LEFT BULK UPPER LIMB AE 20 cm 20 cm BE 19 cm 18.5 cm LOWER LIMB AK 35 cm 35 cm BK 28 cm 27 cm TONE UPPER LIMB normal normal LOWER LIMB normal normal
  12. 16. POWER RIGHT LEFT Neck 2 UL Shoulder Flexors 3 3 Extensors 3 3 Abduction 3 3 Adduction 3 3 Elbow 4- 4- Wrist 5 5 Hand grip 100% 100% LL Hip Abduction 2 2 Adduction 2 2 Flexion 2 2 Extension 2 2
  13. 17. REFLEXES RIGHT LEFT LL Knee 3 3 Ankle 5 5 EHL 5 5 RIGHT LEFT SUPERFICIAL Corneal + + Conjuctival + + Abdominal - - Plantar flexor flexor DEEP Biceps + + Triceps + + Supinator + + Knee + + Ankle + delayed relaxation + delayed relaxation
  14. 18. <ul><li>Coordination – impaired due to weakness </li></ul><ul><li>Gait – walks with support </li></ul><ul><li>SENSORY - touch, pain, temperature </li></ul><ul><li>position, vibration sense </li></ul><ul><li>CEREBELLUM – normal </li></ul><ul><li>SPINE & CRANIUM - normal </li></ul>normal
  15. 19. PROBABLE DIAGNOSIS…??
  16. 20. <ul><li>Endocrine – thyroid </li></ul><ul><li>parathyroid </li></ul><ul><li>adrenal </li></ul><ul><li>Drug induced myopathy </li></ul><ul><li>Inflammatory – Polymyositis / dermatomyositis </li></ul><ul><li>Infectious </li></ul><ul><li>SLE </li></ul><ul><li>Myasthenia gravis </li></ul>
  17. 21. IMPRESSION : <ul><li>PROXIMAL MUSCLE WEAKNESS + </li></ul><ul><li>DELAYED RELAXATION OF DTR </li></ul><ul><li>NO SENSORY / CRANIAL NERVES INVOLVEMENT </li></ul><ul><li>F/S/O HYPOTHYROIDISM </li></ul><ul><li>HYPOTHYROID MYOPATHY </li></ul>
  18. 22. INVESTIGATIONS : <ul><li>CBC </li></ul><ul><li>Hb – 10.7 gm% </li></ul><ul><li>TC – 6600 </li></ul><ul><li>DC – P70 L30 </li></ul><ul><li>ESR – 5/12 </li></ul><ul><li>PCV – 30 </li></ul><ul><li>PLT – 1 lakh </li></ul><ul><li>RBS – 96 mg% </li></ul><ul><li>B. Urea – 22 </li></ul><ul><li>S. Creatinine – 0.8 </li></ul>S. Electrolytes : Na – 134 meq/L K - 4.4 Cl – 86 Hco3 – 21
  19. 23. <ul><li>ECG – NSR, complete RBBB </li></ul><ul><li>CXR – Lt side eventration of diaphragm </li></ul><ul><li>ELISA for HIV1,2 – non reactive </li></ul><ul><li>S. lipid profile </li></ul><ul><li>total chol – 224 mg/dl </li></ul><ul><li>TGL – 141 </li></ul><ul><li> HDL – 46 </li></ul><ul><li>LDL – 150 </li></ul><ul><li>VLDL – 28 </li></ul><ul><li>P smear- normocytic normochromic anemia </li></ul><ul><li>WBCs & platelets normal </li></ul>
  20. 25. <ul><li>USG ABDOMEN – normal study </li></ul><ul><li>ECHO - </li></ul><ul><li>no RWMA </li></ul><ul><li>normal LV systolic function </li></ul><ul><li>normal valves </li></ul><ul><li>mild PE </li></ul><ul><li>no e/o tamponade </li></ul><ul><li>ANA - negative </li></ul><ul><li>RF – negative </li></ul><ul><li>S. calcium - 9 mg/dl </li></ul>
  21. 26. Neurological consult : <ul><li>Proximal myopathy + </li></ul><ul><li>Slow relaxation of ankle jerk + </li></ul><ul><li>Hoarseness of voice </li></ul><ul><li>Suggested : S. CPK </li></ul><ul><li>TFT </li></ul><ul><li>EMG </li></ul>? HYPOTHYROID MYOPATHY
  22. 27. <ul><li>TFT : </li></ul><ul><li>T3 < 10 ng/dl (60-200) </li></ul><ul><li>T4 < 0.30 μ g/dl (4.5 – 12) </li></ul><ul><li>TSH > 150 μ IU/ml (0.3-5.5) </li></ul><ul><li>S. CPK – 1014 u/l </li></ul><ul><li>Anti TPO - 13.80 IU/ml (0-34) </li></ul>
  23. 28. NCS <ul><li>Motor (CMAPs) & sensory (SNAPs) </li></ul><ul><li>NORMAL </li></ul>
  24. 29. EMG <ul><li>EMG done in Rt quadriceps, Lt quadriceps, Rt biceps, </li></ul><ul><li>Rt EDB </li></ul><ul><li>In the above muscles, </li></ul><ul><li>Insertional activity normal </li></ul><ul><li>Spontaneous activity – no abnormal waves seen </li></ul><ul><li>MUP’s were of low amplitude about 37 μ V </li></ul><ul><li>Interference pattern – recruitment complete with low amplitude </li></ul><ul><li>S/O MYOPATHIC PATTERN </li></ul>
  25. 30. SPONTANEOUS ACTIVITY
  26. 31. MOTOR UNIT POTENTIALS
  27. 32. INTERFERENCE PATTERN
  28. 33. FINAL DIAGNOSIS <ul><li>HYPOTHYROIDISM </li></ul><ul><li>HYPOTHYROID MYOPATHY </li></ul><ul><li>EVENTRATION OF LEFT DIAPHRAGM </li></ul>
  29. 34. MANAGEMENT <ul><li>T. Eltroxin 100 μ g od </li></ul><ul><li>250 μ g od </li></ul><ul><li>T. Ranitidine 150 mg 1 bd </li></ul>As suggested by endocrinologist
  30. 35. COURSE IN THE HOSPITAL
  31. 39. <ul><li>Neuromuscular symptoms present in 30 – 80 % of patients with hypothyroidism </li></ul><ul><li>Weakness is observed in 1/3 rd of patients </li></ul><ul><li>Incidence – 5.6% in a study of 53 patients with acquired muscle disease </li></ul><ul><li>Muscle cramping </li></ul><ul><li>Proximal symmetrical muscle weakness </li></ul><ul><li>Muscle stiffness </li></ul><ul><li>Muscle enlargement – Kocher Debre Semilaigne </li></ul><ul><li>Hoffman’s </li></ul><ul><li>Delayed relaxation of DTR (85%) </li></ul>
  32. 40. PATHOPHYSIOLOGY <ul><li>Muscle involvement may be caused by </li></ul><ul><li>changes in muscle fibres from fast twitching type II to slow twitching type I fibres </li></ul><ul><li>deposition of glycosaminoglycans </li></ul><ul><li>poor contractility of actin–myosin units </li></ul><ul><li>low myosin ATPase activity </li></ul><ul><li>low ATP turnover in skeletal muscle </li></ul><ul><li>involvement of the muscle membrane </li></ul>
  33. 41. PSEUDOMYOTONIC REFLEX <ul><li>The rate-limiting step in muscle relaxation is reuptake of calcium by the sarcoplasmic reticulum, which is dependent on calcium ATPase content (SERCA-1) of the muscle fibre. </li></ul><ul><li>Calcium ATPase activity of fast twitch variety of muscle fibre is decreased in hypothyroidism producing delayed relaxation, the pseudomyotonic reflex. </li></ul>
  34. 42. <ul><li>serum CK levels - 2 to 6 fold though marked elevation up to the range of 20,000- 25,000 U/L may be seen (thyroid deficiency permits leakage across muscle membranes and possibly due to actual muscle necrosis) </li></ul><ul><li>EMG – normal or myopathic </li></ul><ul><li>The MUAPs are generally polyphasic with reduced amplitude and duration. </li></ul><ul><li>Early recruitment may also be observed </li></ul>
  35. 44. <ul><li>NCS – normal </li></ul><ul><li>may show f/s/o mild neuropathy </li></ul><ul><li>Biopsy – non specific changes. </li></ul><ul><li>Fiber atrophy is noted, and increased numbers of internal nuclei, glycogen aggregates, and deposition of mucopolysaccharides in the connective tissue </li></ul>
  36. 45. MANAGEMENT <ul><li>Levothyroxine replacement </li></ul><ul><li>Myopathy improves within 2-3 weeks, but may take months to resolve completely </li></ul><ul><li>EMG findings revert with treatment </li></ul>
  37. 47. <ul><li>References : </li></ul><ul><li>Harrison 17/e </li></ul><ul><li>Emedicine sources </li></ul><ul><li>DeJong </li></ul>

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