DR. PRIYA KUBENDIRAN DR. PRIYA KUBENDIRAN M 1 UNIT M 1 UNIT PROF. DR. MAGESH PROF. DR. MAGESH KUMAR KUMAR CASE PRESENTATION CASE PRESENTATION
Nov 02, 2014
DR. PRIYA KUBENDIRANDR. PRIYA KUBENDIRANM 1 UNITM 1 UNIT
PROF. DR. MAGESH KUMAR PROF. DR. MAGESH KUMAR
CASE PRESENTATIONCASE PRESENTATION
A 45 year old female was admitted on 08.11.10 with
C/O : Difficulty in walking - 6 months Difficulty in lifting her arms – 5 months Difficulty in holding her head erect – 4
months
H/O PRESENT ILLNESS :
6 months ago, the patient noticed that she had difficulty climbing stairs
difficulty in getting up from squatting position difficulty in walkingAround a month later, she was unable to lift her
arm above her headAfter 2 months she was unable to hold her head
erectGradually progressive weakness over the past 6
monthsNo history s/o distal weakness of upper & lower
limbs
h/o difficulty in turning from side to side in bed
h/o difficulty in getting up from bedNo h/o noticing any difference in limb sizeNo history s/o cranial n involvementNo h/o sensory disturbancesNo h/o bowel/bladder disturbancesNo h/o cramps/ pain in the limbsNo h/o diurnal variation h/o facial puffiness +h/o retrosternal chest pain burning, aggravated by food
h/o abdominal pain upper abdomen, burning typeh/o constipation +h/o loss of appetiteNo h/o loss of weightNo h/o feverNo h/o skin rash / joint pain
PAST HISTORY : No h/o similar illness Not a known case of DM/SHT/PT/BA/IHD No h/o any drug intake / surgeries
PERSONAL HISTORY : Mixed diet Unmarried Attained menopause before 4 years Not a tobacco/betel nut chewer
FAMILY HISTORY : No h/o similar illness
SUMMARIZING….
A 45 year old female has presented with gradually progressive weakness of proximal muscles over the past 6 months
POSSIBILITIES….???
Endocrine – thyroid parathyroid adrenal Drug induced myopathy Inflammatory – Polymyositis /
dermatomyositis Infectious Connective tissue disorders - SLE Myasthenia gravis Inherited - Limb girdle muscular dystrophy
GPE : Conscious, oriented VITALS : Afebrile Voice was hoarse PR – 76/ min Hydration fair BP – 110/70 mm
hg Facial puffiness RR – 16 cycles /
min Pale Macroglossia Anicteric No neck swelling No cyanosis/clubbing Icthyosis in lower limbs No pedal edema No lymphadenopathy
SYSTEMIC EXAMINATION :
RS - NVBS heard no added soundsCVS - S1 S2 heard no added soundsP/A - distended divarication of recti + soft, epigastric tenderness + no organomegaly
CNSHMF - normalCRANIAL NERVES - normalMOTOR SYSTEM – 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
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
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
REFLEXES
Coordination – impaired due to weaknessGait – walks with support
SENSORY - touch, pain, temperature position, vibration sense
CEREBELLUM – normalSPINE & CRANIUM - normal
normal
PROBABLE DIAGNOSIS…??
Endocrine – thyroid parathyroid adrenal Drug induced myopathy Inflammatory – Polymyositis /
dermatomyositis Infectious SLE Myasthenia gravis
IMPRESSION : PROXIMAL MUSCLE WEAKNESS + DELAYED RELAXATION OF DTR NO SENSORY / CRANIAL NERVES
INVOLVEMENT F/S/O HYPOTHYROIDISM
HYPOTHYROID MYOPATHY
INVESTIGATIONS :
CBC Hb – 10.7 gm% TC – 6600 DC – P70 L30 ESR – 5/12 PCV – 30 PLT – 1 lakh
RBS – 96 mg%B. Urea – 22S. Creatinine – 0.8
S. Electrolytes :
Na – 134 meq/L K - 4.4 Cl – 86 Hco3 – 21
ECG – NSR, complete RBBBCXR – Lt side eventration of diaphragmELISA for HIV1,2 – non reactiveS. lipid profile total chol – 224 mg/dl TGL – 141
HDL – 46 LDL – 150 VLDL – 28P smear- normocytic normochromic anemia WBCs & platelets normal
USG ABDOMEN – normal study
ECHO - no RWMA normal LV systolic function normal valves mild PE no e/o tamponade
ANA - negativeRF – negativeS. calcium - 9 mg/dl
Neurological consult :
Proximal myopathy + Slow relaxation of ankle jerk +Hoarseness of voice
Suggested : S. CPK TFT EMG
? HYPOTHYROID MYOPATHY
TFT : T3 < 10 ng/dl (60-200) T4 < 0.30 μg/dl (4.5 – 12) TSH > 150 μIU/ml (0.3-5.5)
S. CPK – 1014 u/l
Anti TPO - 13.80 IU/ml (0-34)
NCS
Motor (CMAPs) & sensory (SNAPs) NORMAL
EMG EMG done in Rt quadriceps, Lt quadriceps, Rt
biceps,
Rt EDB In the above muscles, Insertional activity normal Spontaneous activity – no abnormal waves seen MUP’s were of low amplitude about 37 μV Interference pattern – recruitment complete with
low amplitude
S/O MYOPATHIC PATTERN
SPONTANEOUS ACTIVITY
MOTOR UNIT POTENTIALS
INTERFERENCE PATTERN
FINAL DIAGNOSIS
HYPOTHYROIDISM HYPOTHYROID MYOPATHY EVENTRATION OF LEFT DIAPHRAGM
MANAGEMENT
T. Eltroxin 100 μg od
250 μg od
T. Ranitidine 150 mg 1 bd
As suggested by endocrinologist
COURSE IN THE COURSE IN THE HOSPITALHOSPITAL
Neuromuscular symptoms present in 30 – 80 % of patients with hypothyroidism
Weakness is observed in 1/3rd of patientsIncidence – 5.6% in a study of 53 patients
with acquired muscle disease
Muscle cramping Proximal symmetrical muscle weakness Muscle stiffness Muscle enlargement – Kocher Debre
Semilaigne Hoffman’s Delayed relaxation of DTR (85%)
PATHOPHYSIOLOGY Muscle involvement may be caused by
changes in muscle fibres from fast twitching type II to slow twitching type I fibres
deposition of glycosaminoglycanspoor contractility of actin–myosin units low myosin ATPase activitylow ATP turnover in skeletal muscleinvolvement of the muscle membrane
PSEUDOMYOTONIC REFLEX
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.
Calcium ATPase activity of fast twitch variety of muscle fibre is decreased in hypothyroidism producing delayed relaxation, the pseudomyotonic reflex.
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)
EMG – normal or myopathic The MUAPs are generally polyphasic
with reduced amplitude and duration. Early recruitment may also be observed
NCS – normal may show f/s/o mild neuropathy
Biopsy – non specific changes. Fiber atrophy is noted, and increased
numbers of internal nuclei, glycogen aggregates, and deposition of mucopolysaccharides in the connective tissue
MANAGEMENT
Levothyroxine replacement Myopathy improves within 2-3 weeks, but
may take months to resolve completelyEMG findings revert with treatment
References :-Harrison 17/e-Emedicine sources-DeJong