A CASE OF RAPIDLY PROGRESSIVE GENERALISED

WEAKNESS

BY:Dr. Tikal Kansara

R2 Medicine D Unit

BIO-DATA

• Sufiaben Abdulhamid Vohra• 42 y / F• Muslim• 7th standard schooling• Housewife• Married• Rs. 5000 / month• Hathikhana, suburban Vadodara

CHIEF COMPLAIN

• Difficulty in using left upper limb for 2 hours

O. D. P.

• Alright before going to sleep the night before, and on waking up in the morning she noticed

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

• 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

• 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

• 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

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

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

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

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

• 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

• 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

MOTOR FUNCTIONS

• Nutrition:– No muscle wasting

• Tone:– Upper limb:• Right: Reduced• Left: Reduced

– Lower limb:• Right: Reduced• Left: Reduced

• 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

• LOWER LIMB:– Knee:

» Flexors: 4 / 5» Extensors: 4 / 5

– Ankle:» Dorsiflexors: 4 / 5» Plantar Flexion: 4 / 5

• 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

– LOWER LIMB• Knee:

» Flexors: 3 / 5» Extensors: 3 / 5

• Ankle:» Dorsiflexion: 3 / 5» Plantar Flexion: 3 / 5

• Co-ordination:– Not elicitable

• Involuntary Movements:– Absent

SENSORY FUNCTIONS

• Superficial – Pain : Intact– Touch : Intact– Temperature : Grossly intact

• Deep– Vibration : Intact– Pressure : Intact– Joint Sense : Intact– Position Sense : Intact

• Cortical Sensation:– One point discrimination : Intact– Two point discrimination : Intact– Stereognosis : Intact– Graphasthesia : Intact– Sensory extinction: Intact

REFLEXES

• Superficial:

RELFEX RIGHT LEFT

Abdominal

Upper ++ ++

Middle ++ ++

Lower ++ ++

Plantar - -

REFLEX RIGHT LEFT

Biceps Jerk + +

Triceps Jerk + +

Supinator Jerk + +

Knee Jerk + +

Ankle Jerk - -

PERIPHERAL NERVES

• Peripheral Nerves are not thickened.

CEREBELLAR SIGNS

• Finger-Nose Test : Not elicitable• Dysdiadokokinesia : Not elicitable• Intention Tremor : Not elicitable

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

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

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

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

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

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

URINE EXAMINATION

MICROSCOPIC EXAMINATION

Pus Cells Occassional / hpf

Red Cells 4 – 6 / hpf

Epithelial Cells Few / hpf

Casts Absent

Crystals Absent

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

ON ADMISSION

QTc = 624 msec

QTc = 624 msec

DAY 2 IN HOSPITAL

SUBSEQUENT ECG DURING CORRECTIONS

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

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

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

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

USG ABDOMEN

• NAD

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

• X – Ray DL Spine – AP, Lateral• X – Ray Cervical Spine – AP, Lateral

– NAD

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

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

STATUS AS OF NOW !!!

INVESTIGATION RESULT NORMAL RANGE

Urine K 16.08

Urine K / Cr (Ratio) 4.92

Urine Creatinine 37.0 30 - 125

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

OTHER INVESTIGATIONS

• D - FUNDUS:– Not suggestive of hypertensive retinopathy at

present• Urine Ketones:– Negative

So … …

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

Reproduced From: Department of Neurology, JIPMER 2014

By:Dr. Tikal KansaraR2 Medicine D Unit