A case of rapidly progressive generalised weakness
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A CASE OF RAPIDLY PROGRESSIVE GENERALISED
WEAKNESS
BY:Dr. Tikal Kansara
R2 Medicine D Unit
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BIO-DATA
• Sufiaben Abdulhamid Vohra• 42 y / F• Muslim• 7th standard schooling• Housewife• Married• Rs. 5000 / month• Hathikhana, suburban Vadodara
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CHIEF COMPLAIN
• Difficulty in using left upper limb for 2 hours
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O. D. P.
• Alright before going to sleep the night before, and on waking up in the morning she noticed
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Difficulty in using her left upper limb
Numbness of her left upper limb
On waking up in morning
Around 1.5 hours after waking upDifficulty in sitting without support
Following the next 2 to 3 hoursDifficulty in using her right upper limb and both lower
limbs
Difficulty in moving her neck sideways and lifting head
That late night
Point of Contact with the patient in ER
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• No C/O – Altered Sensorium / Convulsions / Headache
• No C/O – Difficulty in vision and opening eyes (double vision / diminished vision)
• No C/O – Tingling and numbness of face and other parts of body
• No C/O – difficulty in chewing• No C/O – Deviation of mouth to one side /
dribbling of saliva• No C/O – Difficulty in swallowing or speaking• No C/O – Neck pain / pain in any limb• No C/O – Bladder disturbances
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• No C/O – Diarrhoea / Vomitting / Cough / Fever
• No C/O – Abdominal Pain / Muscle Pain• No C/O – Rash marks all over body/ Multiple
Joint Pains• No H/O Recent Vaccinations / head trauma /
Medication ingestion / Recent Travel • No H/O – Insect/animal bites
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• Past History : – N/K/C/O – DM, HTN– N/P/H/S/O – TB, Jaundice
• Family History : DM in mother x 5 years
• Personal History : NAD
• Vaccination & Immunization History: Patient is immunized in childhood according to IAP schedule
• Menstrual History:– Menopausal since 8 years
• Obstretic History:– G4P5A0L4
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HISTORY CONCLUSION• So, at the end of history we have a 42 y/o F, with rapidly
progressive, near-symmetrical type of paralysis involving all four limbs as well as truncal muscles without the involvement of cranial nerves, higher functions or overt sensory symptoms and intact bladder, most likely we are dealing with a case of lower motor neuron type (LMN) of palsy. I would like to label the patient at this stage as a case of Acute Flaccid Paralysis (AFP).
– Guillian-Barre Syndrome– Occult Bites:
• Envenomous Snake Bites• Tick Paralysis
– Inceptive episode of periodic paralysis
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GENERAL EXAMINATION– GC – Guarded– TPR – N / 60 / Regular– Bp – 110 / 70 mmHg
• Oral Cavity – Normal• No pallor/ cyanosis / clubbing / icterus / pedal
edema/ lymphadenopathy• Back & Spine – Normal• No Rash / Bite Marks / Tick stuck to the skin
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CENTRAL NERVOUS SYSTEM
• Patient is conscious, well cooperative, well oriented to time, place and person
• Her recent as well as remote memory is intact• Speech is normal
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CRANIAL NERVES• Olfactory: Normal• Optic:
– Acuity of vision: Normal– Field of vision: Normal– Color vision: Normal
• Oculomotor, Trochlear and abducens:– No Ptosis– No Squint– No enophthalmos or exophthalmos– Normal movement of eyeballs in all directions– No Nystagmus– Pupils:
• PERRLA
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• Trigeminal:– Motor Function: No hindrance in movement of
muscles of mastication– Sensations on face: Intact– Corneal Reflex:
• Right: Present• Left: Present
– Jaw Jerk: Present• Facial:– Intact frowning, bilaterally equal nasolabial folds,
no deviation of mouth to one side, – Bell’s Sign: Negative
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• Vestibulocochlear:– Watch Test: Patient perceives the sound– Rinne’s Test: AC > BC– Weber’s Test: Bilaterally equal
• Glossopharyngeal and vagus:– Soft palate movement: Intact– Gag Reflex: Present
• Spinal Accessory:– Power of sternocleidomastoid and trapezius: 5/5
• Hypoglossal:– Centralized on protrusion– No fasciculation noted
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MOTOR FUNCTIONS
• Nutrition:– No muscle wasting
• Tone:– Upper limb:• Right: Reduced• Left: Reduced
– Lower limb:• Right: Reduced• Left: Reduced
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• RIGHT – UPPER LIMB:
• Shoulders: » Flexors: 5 / 5» Extensors: 5 / 5» Abductors: 5 / 5» Adductors: 5 / 5
• Elbow: » Flexors: 5 / 5» Extensors: 5 / 5
• Small Muscles of hand: 5 / 5– LOWER LIMB:
• Hip: » Flexors: 4 / 5» Extensors: 4 / 5» Abductors: 4 / 5» Adductors: 4 / 5
POWER
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• LOWER LIMB:– Knee:
» Flexors: 4 / 5» Extensors: 4 / 5
– Ankle:» Dorsiflexors: 4 / 5» Plantar Flexion: 4 / 5
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• LEFT– UPPER LIMB:
• Shoulder:» Flexors: 4 / 5» Extensors: 3 / 5» Abduction: 3 / 5» Adduction: 4 / 5
• Elbow:» Flexors: 3 / 5» Extensors: 3 / 5
• Small Muscles of hand: 4 / 5– LOWER LIMB:
• Hip:» Flexors: 2 / 5» Extensors: 2 / 5» Abduction: 2 / 5» Adduction: 2 / 5
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– LOWER LIMB• Knee:
» Flexors: 3 / 5» Extensors: 3 / 5
• Ankle:» Dorsiflexion: 3 / 5» Plantar Flexion: 3 / 5
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• Co-ordination:– Not elicitable
• Involuntary Movements:– Absent
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SENSORY FUNCTIONS
• Superficial – Pain : Intact– Touch : Intact– Temperature : Grossly intact
• Deep– Vibration : Intact– Pressure : Intact– Joint Sense : Intact– Position Sense : Intact
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• Cortical Sensation:– One point discrimination : Intact– Two point discrimination : Intact– Stereognosis : Intact– Graphasthesia : Intact– Sensory extinction: Intact
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REFLEXES
• Superficial:
RELFEX RIGHT LEFT
Abdominal
Upper ++ ++
Middle ++ ++
Lower ++ ++
Plantar - -
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REFLEX RIGHT LEFT
Biceps Jerk + +
Triceps Jerk + +
Supinator Jerk + +
Knee Jerk + +
Ankle Jerk - -
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PERIPHERAL NERVES
• Peripheral Nerves are not thickened.
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CEREBELLAR SIGNS
• Finger-Nose Test : Not elicitable• Dysdiadokokinesia : Not elicitable• Intention Tremor : Not elicitable
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OTHER SYSTEM EXAMINATION
• CARDIOVASCULAR EXAMINATION:– S1, S2 Normal– No murmurs
• RESPIRATORY EXAMINATION:– AEBE– No crepitations / rhochi
• PER ABDOMEN EXAMINATION:– Soft, non tender– Liver, spleen – Not palpable– Bowel sounds - Present
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HISTORY & PE CONCLUSION• So, at the end of history & PE, we have a 42 y/o F, with rapidly
progressive, near-symmetrical type of paralysis involving all four limbs as well as truncal muscles without the involvement of cranial nerves, higher functions or overt sensory symptoms and intact bladder, with generalised hypotonia, reduced motor power and diminished reflexes; without evidence of any rash, muscle tenderness, visible bite marks or ticks, nor any subtle sensory examination findings. This could most likely be a case of lower motor neuron type (LMN) of palsy due to:
– Periodic Paralysis– Atypical Presentation of Gullian-Barre Syndrome
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INVESTIGATIONS
INVESTIGATION VALUE NORMAL VALUE
Hemoglobin 11.20 gm% 12.0 – 16.0 gm%
Total WBCs 7,800 / cu. mm 4,000 – 11,000 / cu/ mm
Platelets Adequate
ESR 48 mm / hr 2 – 20 mm / hr
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BIOCHEMICAL INVESTIGATION VALUE NORMAL VALUE
UREA 45 mg/dl 13 – 45 mg/dl
Bilirubin
Total 0.9 mg/dl 0.1 – 1.2 mg/dl
Direct 0.4 mg/dl 0 – 0.4 mg/dl
Indirect 0.5 mg/dl 0.1 – 0.8 mg/dl
SGPT (ALT) 20 U/L <40 U/L
SGOT (AST) 26 U/L < 37 U/L
Alkaline Phosphatase 182 IU/L 28 – 111 IU/L
Total Protein 7.3 gm/dl 6.0 – 8.0 gm/dl
Albumin 3.8 gm/dl 3.2 – 5.0 gm/dl
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BIOCHEMISTRYINVESTIGATION VALUE NORMAL VALUE
S. Creatinine 1.1 mg/dl 0.5 – 1.4 mg/dl
S. Sodium 136 mEq/L 135 – 150 mEq/L
S. Potassium 3.0 mEq/L 3.5 – 5.0 mEq/L
S. Calcium 8.6 mg/dl 8.6 – 10.6 mg/dl
S. Magnesium 2.0 mg/dl 1.7 – 2.5 mg/dl
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URINE EXAMINATIONCHEMICAL EXAMINATION
Reaction 6.5
Specific Gravity 1.005
Protein Present, +1 (30 mg/dl)
Glucose Absent
Ketone Absent
Blood Present, +1
Urobilinogens Absent
Bile Salts Absent
Bile Pigments Absent
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URINE EXAMINATION
MICROSCOPIC EXAMINATION
Pus Cells Occassional / hpf
Red Cells 4 – 6 / hpf
Epithelial Cells Few / hpf
Casts Absent
Crystals Absent
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SERIAL POTASSIUM READINGS
DATE SERUM POTASSIUM
CUMMULATIVE POTASSIUM
CORRECTION (EXCLUDING ORAL)
22/10/2015 3.0
24/10/2015 1.7 80 mEq
24/10/2015 2.1 160 mEq
26/10/2015 2.6 320 mEq
29/10/2015 3.0
30/10/2015 2.9
31/10/2015 2.5
01/11/2015 2.8
03/11/2015 2.7
04/11/2015 2.8
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ON ADMISSION
QTc = 624 msec
QTc = 624 msec
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DAY 2 IN HOSPITAL
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SUBSEQUENT ECG DURING CORRECTIONS
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BLOOD GAS ANALYSIS (ABG)
TEST RESULT REFERENCE RANGE (Arterial)
pH 7.38 7.35 – 7.45
PCO2 15.7 mmHg 35.0 – 45.0
PO2 150 mmHg >80
O2 Sat 99.4 % 95.0 – 98.0
Base Excess (Be) -14.7 mmol/L (-2) – (+3)
cHCO3 (P) 9.1 mmol/L 22.0 – 26.00
ctCO2 (B) 8.7 mmol/L 23.00 – 27.00
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INVESTIGATION RESULT NORMAL VALUE
Urinary Potassium (Spot)
19.5 mEq/L 22 – 164 (For Female)
Urinary Sodium (Spot)
25 mEq/L 15 – 237 (For Female)
INVESTIGATION RESULT NORMAL VALUE
24 hour URINE POTASSIUM
11.64 mmol/L < 20 mmol/L
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INVESTIGATION RESULT NORMAL VALUE
Free T3 1.94 pg/ml 2.0 – 4.43
Free T4 1.16 ng/ml 0.93 – 1.70
TSH 2.22 μIU/ml 0.27 – 4.20
THYROID FUNCTION TESTS
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What we could not do !!!
• Urine Osmolality• Trans Tubular Potassium Gradient (TTKG)• Early and prompt Arterial Blood Gas Analysis
(ABG)• Nerve Conduction Studies, including exercise
testing
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USG ABDOMEN
• NAD
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USG – B/L ADRENAL & THRYOID GLAND
• B/L suprarenal region appears clear• Thyroid gland appears normal in size,
homogenous echo pattern and normal vasularity… no e/o focal lesion
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• X – Ray DL Spine – AP, Lateral• X – Ray Cervical Spine – AP, Lateral
– NAD
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What we did !!!• Gave Basic & Supportive treatment to patient– ICU Care– BiPAP Support (Day 2 to ½ of Day 3)
• Corrected Serum Potassium– Intravenous– Oral
• When symptoms reduced sufficiently not to debilitate the patient, discharged her with very close follow up with– Oral Potassium Supplements– Tab. Acetazolamide (250 mg) QiD
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STATUS AS OF NOW !!!
INVESTIGATION RESULT NORMAL RANGE
pH 7.46 7.35 – 7.45
pCO2 14.4 35.0 – 45.0
HCO3 16.7 22.0 – 26.0
sO2 98.7 %
ABG
INVESTIGATION RESULT NORMAL RANGE
Serum Potassium 1.45 mEq/L 3.5 – 5.5 mEq/L
VENOUS SAMPLE ANALYSIS
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STATUS AS OF NOW !!!
INVESTIGATION RESULT NORMAL RANGE
Urine K 16.08
Urine K / Cr (Ratio) 4.92
Urine Creatinine 37.0 30 - 125
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URINE K / Cr (RATIO) ACID – BASE STATUS INTERPRETATION
< 1.5 Metabolic Acidosis Diarrhoea, Laxatives
< 1.5 Metabolic Alkalosis Vomitting (Severe)
> 1.5 Metabolic Acidosis DKA, RTA 1 & 2
> 1.5 Metabolic Alkalosis
(Normal or low Blood Pressure)
Diuretics, Bartter Syndrome, Gitelmann Syndrome
> 1.5 Metabolic Alkalosis (High Blood Pressure)
Primary Hyperaldosteronism, Cushing Syndrome, Liddle Syndrome
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OTHER INVESTIGATIONS
• D - FUNDUS:– Not suggestive of hypertensive retinopathy at
present• Urine Ketones:– Negative
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So … …
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Our Plan now … …
• Supplement IV and oral potassium to bring it as close to normal as possible
• Start with Thiazide diuretics.• Investigate for the case of probable Renal
Tubular Acidosis as below:– Serum Chloride levels– Serum and Urinary Ammonium ions– Urinary pCO2– Urinary Bicarbonate ion
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Reproduced From: Department of Neurology, JIPMER 2014
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By:Dr. Tikal KansaraR2 Medicine D Unit