A 58-year-old man presented with progressive weakness in his lower limbs over 6 days along with difficulty swallowing and speaking. His symptoms progressed to involve his upper limbs as well. On examination, he had decreased strength and reflexes in all limbs along with facial weakness and impaired swallowing. Nerve conduction studies showed axonal and demyelinating changes. He was diagnosed with acute motor axonal neuropathy, a subtype of Guillain-Barré syndrome. He was treated with IVIG and supportive care and showed significant improvement in strength and reflexes on follow up.
2. Chief Complaints
A 58 Y/o Muslim male from Godhra, Poultry farmer by profession,
belonging to lower socio-economic class, admitted on 28th of March
presented with a chief complain of progressive weakness in both
lower limbs for 6 days, difficulty in swallowing since 4 days, difficulty
in speaking since 3 days.
3. History of Present Illness
Patient was relatively asymptomatic before 10 days when he developed
diarrhoea for 1 day without fever or abdominal pain, without blood or
mucus, which was self limiting. After 4 days he developed bilateral
symmetrical lower limb weakness which he noticed in the form of difficulty
in walking (requiring assistance/support for standing and walking). It
gradually progressed in 2 days to an extent where he couldn’t stand up. The
weakness extended upto the upper limbs from onset of weakness in 2 days
in the form of decreased grasping strength and difficulty in moving upper
limbs.
He developed difficulty in swallowing since 4 days and difficulty in speaking
since 3 days.
4. Negative history
Patient had no complaint of
Bowel or bladder disturbance
Fever, cough, chest pain, difficulty in breathing
Abnormal movements
Visual disturbance (double vision or blurred vision)
Tingling, numbness
Gabhraman, palpitation, blackouts (syncope)
Muscle tenderness or joint pain
Recent Injury or bite
Compound ingestion
5. Past History
History of chikungunya fever diagnosed in December 2021 for which analgesics
were prescribed (pints and oral medications)
History of recent COVID vaccination present on 3rd February, 2022.
Vaccination was uneventful. (First Dose Taken)
No history of BT. No history suggestive of TB.
7. Differential Diagnosis
At the end of history we have 58 y/o male patient with a case of bilateral
ascending weakness of all 4 limbs with acute history which progressed over 4
days with bulbar involvement and no loss of sensations and no bladder
involvement without difficulty in breathing.
1. Guillain Barré Syndrome
2. Infectious Polyradiculoneuropathy (HIV, CMV)
3. Periodic Paralysis
8. Examination
Patient was examined after consent with proper light and exposure
General condition was guarded
Temperature was normal.
Pulse was 82 in right radial artery with normal force, volume and tension.
Blood pressure was 150/82 mmHg in right arm in supine position.
Respiration is regular at 16 breaths per minute with no accessory muscle
involvement. Respiratory excursion is normal (5cm)
9. General Examination
Well built and properly nourished
No pallor, icterus, cyanosis, clubbing, lymphadenopathy, oedema.
10. Systemic Examination
Since predominantly nervous system has been involved. I would like to do CNS
examination first.
Higher function except speech was normal.
Conciousness, behaviour, intelligence, memory, orientation was normal, no
delusion or hallucination. Speech (flaccid dysarthria) was affected in the form
of impaired labiodental and labial sounds. (b, bh, ph, ma etc.)
11. Cranial Nerve Examination
There was no abnormalities in Cranial nerves I, II, III, IV, V, VI, VIII, XI and XII.
Facial nerve
There is bilateral symmetrical weakness in the muscles of the face. Patient is
unable frown, close eyes completely, blow out his cheeks or show his teeth. Bell’s
Phenomenon present.
Absent Corneal and conjunctival reflex.
Taste Sensations are normal.
Lacrimation and salivation normal.
Glossopharyngeal and Vagus nerves
Uvula is in Midline. Diminished palatal arch movements.
Absent gag reflex. Nasal Twang present.
12. Motor system examination
Built normal, nutrition normal with normal muscle volume with loss of
contour.
Tone was decreased in all four limbs.
Power assessed was as follows. (At the time of Admission)
14. Hand Grip was decreased bilaterally.
Neck Flexion 2
Neck Extension 5
15. No Respiratory muscle weakness. Chest Excursion was normal ( 5cm )
SBC is more than 30.
16. Sensory system examination
Fine touch normal
Pain and Temperature normal
Joint position and proprioception normal
Pressure sensation normal
17. Refexes (superficial reflexes)
Corneal and conjunctival reflexes Absent.
Gag Reflex absent at the time of admission. Gag Reflex present today.
Plantars are flexors.
Abdominal Reflexes absent.
18. Deep tendon reflexes (at the time of
admission)
Muscle Right Left
Supinator 0 0
Biceps 0 0
Triceps 0 0
Knee 0 0
Ankle 0 0
19. Cerebellar Function
No evidence of cerebellar dysfunction.
Nose finger test, finger to finger test and nose finger nose test normal.
No dyssynergia, tremors, dysdiadochokinesia.
All cerebellar tests (including examination gait) couldn’t be performed due to
predominant weakness in the lower limbs.
20. Gait
Can’t be assessed as patient unable to walk.
Neck and Back
Normal
Nerves and Vessels
No nodularity, thickness or tenderness present over palpable nerves.
Palpable vessel wall thickening absent. Peripheral arterial pulsations
present in all 4 limbs.
21. Differential diagnosis at the end of
Examination
1. Gullain Barré Syndrome
2. Infectious polyradiculoneuropathy.
3. Periodic Paralysis
22. Provisional Diagnosis
Anatomical Abnormality :- Acute polyradiculoneuropathy.
Functional Abnormality:- Inability to stand up or swallow and difficulty in using
both the upper limbs
My provisional diagnosis is Ascending type of polyneuropathy with LMN type of
VII, IX and X nerve palsy most likely Guillain Barré Syndrome.
23. Investigation
Hb 15.70 gm/dL
TC 21100 /cmm
DC 88/10/1/1
Platelet 2.31 lacs/cmm
Urea 54 U/dL
Creat 1.14mg/dL
Bil 0.6/0.2/0.4
Na 134mEq/L
K 4.4mEq/L
Viral Markers Negative (HIV Negative)
24. NCV
Nerve Conduction Study of B/L Upper limbs and Lower limbs tested nerves
shows predominantly Motor Axonal + Demyelinating Affection.
Possibility of AMAN Variant of GBS likely.
25.
26. Final Diagnosis
Ascending type of Lower motor neuron type of weakness with VII, IX and X
nerve palsy. Motor Variant (AMAN subtype) of GBS.
27. Treatment
Patient was started with IVIg at 2gm/kg body weight over 5 days.
Supportive therapy including Antibiotics, Physiotherapy was also given.
28. MRC Sum Score Today
(Power Assessment)
Movement Right Left
Shoulder Abduction 4 4
Elbow Flexion 4 + 4 +
Wrist Extension 4 4
Hip Flexion 4 4
Knee Extension 4 3
Foot dorsiflexion 4 4
48
29. Deep Tendon Reflexes Today
Muscle Right Left
Supinator 2 2
Biceps 2 2
Triceps 2 2
Knee 1 1
Ankle 2 2
30. Facial weakness has improved and patient is able to swallow. Patient is able
to lift his eyebrows and close his eyes. There is still some weakness in the
form of him unable to blow out cheeks. Dysarthria has improved. Corneal and
conjunctival reflexes have reappeared.
31. Prognosis
There is significant improvement in power of and slight improvement in the
reflexes of the patient.
Approximately 85% of patients of GBS achieve full recovery within several
months to a year. Although minor findings on examination may persist and
symptoms of fatigue may persist.
Mortality rate is <5% in optimal settings; death usually results from secondary
pulmonary complications.
Editor's Notes
Difficulty in swallowing was associated with pain
Thiamine Deficiency Adults with thiamine deficiency from nutritional deficiencies or chronic alcohol use typically present with a symmetric, distal motor and sensory polyneuropathy
Lyme Disease
Tick Paralysis
HIV associated polyradiculoneuropathy (CMV Infection)
Sarcoidosis May present with peripheral neuropathy or cranial neuropathy, elevated ACE level in blood or meningeal enhancement in Brain or Spine Imaging supports the diagnosis
Leptomeningeal lymphoma may present with peripheral neuropathy but have other symptoms such as headache, Encephalopathy, or cranial nerve palsies.
Early stage of transverse myelitis, sensory level on examination, early bowel and bladder dysfunction, elevated CSF count.
Botulism
Poliomyelitis
Occult Snake Bite
OP poisoning
Modified Erasmus Global Outcome Score (mEGOS) can be used to estimate the risk of being unable to walk at 6 months. (Our patient’s Score 8 implying 22% risk of being unable to walk unaided at 6 months)
Risk factor for poor outcome
Age>60
Rapid onset of weakness (<7 days from