Mr. X, a 55-year-old businessman with a history of hypertension, diabetes, chronic kidney disease and hepatitis C, presented with weakness in both lower limbs and reduced sensation below the knees for 10 days and 3 days of breathing difficulty. Examination found distal more than proximal weakness in the lower limbs along with reduced sensation below the knees. Investigations including cerebrospinal fluid analysis and MRI brain were normal. He was initially diagnosed with Guillain-Barré syndrome and treated with IV immunoglobulin, but his symptoms worsened requiring intubation. His final diagnosis was considered to be hepatitis C-associated mixed cryoglobulinemia peripheral neuropathy.
2. History
Mr.X 55yrs old gentleman, Bussinessman , Rt.handed
person from Chennai - Known HTN-15yrs/T2DM-
25yrs/CKD-2yrs on MHD/CLD - HCV +ve on
Antivirals/ Rt. PE- on ATT ( H+E+Levo)-Nov 2017
Allergic to : Cipro, Augmentin, Sulphonamides
Presented with ℅ Weakness in both lower limbs -
10days
Breathing difficulty-3 days
3. HOPI
Patient started noticing weakness of both lower limbs in the
form of difficulty in getting up from the squatting position,
toilet chair, standing, walking and gripping the sandals
H/o Buckling while walking +
H/o Numbness in both the LL- 5-6 yrs - red. sensation below
knees
H/o walking on cotton wool/wash basin phenomen/ walking in
the dark - 3-4 yrs
No h/o band like sensation/ incontinence/ back pain / neck pain
No h/o muscle pains/ cramps
4. H/o Vesiculo-papular skin rash over the anterior chest
- week
No h/o fever/ loose stools/ throat pain/ joint pains
No h/o cough with expectoration/ chest pain/
palpitations
No h/o significant weight loss/ loss of appetite
No h/o fall
No h/o Ayurvedic or Native medicines/ toxin
exposure
Vaccinated against - Hepatitis B & Influenza
5. Past History: Known HTN-15yrs/T2DM-25yrs/CKD-
2yrs on MHD weekly twice /CLD - HCV +ve -2014
on Antivirals (Sofosbuvin & Ribavarin) , INF-
sustained remission/ Hypothyroid
Rt. Pleural Effusion (Exudative) - on Empirical ATT (
INH+Ethambutol+Levofloxacin)-Nov 2017
Family History : Nil significant
Personal History : Takes mixed diet, Non
Smoker/Non alcoholic , Sleep disturbed, Appetite - ok
6. On Examination
GPE: Moderately built and nourished
No P I C C L E
Vitals : RR- 32/min, HR- 96/min, BP- 140/60
Vesiculo-papular rash over the anterior chest +
CNS: Conscious, alert, well oriented to T/P/P
Speech - N
Pupils- 3mm B/L reactive, Fundus - normal, EOM - Full
No Facial lag, Tongue & Palate - N
7. Motor System: No wasting/ Fasciculations
Tone :UL - N, LL - Distal hypotonia +
Power : UL - 4/5 4/5
LL - Prox- 3/5 3/5
Dist- 4-/5 4-/5
Neck Power : Fle&Ext - 5/5
DTR’s- UL 1+, LL- Absent
Plantar - B/L flexors
8. Sensory System: Touch, pain, temp, vibration - sense
reduced 40-50% below knee
No cerebellar / Meningeal signs
Gait- Not able to check
Spine & Cranium - N
Other Systemic Examination - N
9.
10. Summary
Mr.X 55yrs old gentleman, Businessman , Rt.handed person
from - Known HTN/T2DM/CKD- on MHD/CLD - HCV +ve
on Antivirals/ Rt. PE- on Empirical ATT came with ℅
weakness of both lower limbs distal > proximal - 10 days,
sensory impairment below knee - 6 yrs and breathing difficulty
- 2 days, with skin rash, without cough & expectoration, with
no h/o incontinence, back pain, neck pain, fever, loose stools,
loss of appetite , weight loss, toxin exposure.
Probable Diagnosis:
25. Treatment
Patient started on IV IG for 5 days
Supportive treatment
The day 2 after IV IG - patient started worsening in
his weakness as well as breathlessness
Patient got intubated
26.
27.
28. Onset,
duration
& evaluation
of
symptoms
Acute(days to 4weeks) GBS,Vasculitis,Radiculopathie
s,Toxic neuropathies,Acute
intermittent Porphyria)Subacute (4-8
weeks)
Chronic(>8 weeks) Most Neuropathies
(Diabetes,CRF,CIDP,Paraneopl
astic, Hereditary motor
sensory neuropathies)
Course Monophasic
Progressive
Relapsing CIDP,Porphyria,Toxic,HIV/AIDS,
29. Axonal Neuropathy Demyelinating Neuropathy
Usually Gradual and insidious Onset Usually Acute or subacute
Largeand long axonsare
affected early, hence initially
lower extremeties areaffected
Diffuse process. Starts in lower
limbs. Butnot always distal
Stocking-glove sensory motor loss
results in symmetrical distal clinical
signsin legs andarms
Generalized Weaknessand mild
sensory loss.
Distal involvement Proximal and distal involvement
Ankle jerk lost early and proximal
tendonreflexes
preserved
All reflexes are lost early
Muscle wasting Common Relatively absent
CSFProteinsnormal CSFProteins elevated(since nerve
rootsare
involved
Slow Recovery Rapid Recovery
Residual deformity Common Residual deformity lesscommon
Normal Conduction normal or slightly
lowered
Nerve Conduction isslowed
35. AsymmetricSensoryLosswith DistalWeakness
Involvement of
Multiple Nerves -
Mulitifocal CIDP,Vasculitis,Cryglobulinemia,
Amyloidoisis,Sarcoid
Infectious (leprosy,Lyme,hepatitis B,C,or E,HIV,
CMV)
Tumorinfiltration
Hereditary Neuropathy with liability topressure
palsies
Involvement of
single
Nerves/Region
Compressive mononeuropathy,
plexopathy, or
radiculopathy
36.
37.
38. HCV associated PN
Peripheral neuropathy is the most common symptom in patients with
mixed cryoglobulinaemia associated with HCV infection
It may be the first clinical manifestation.
Peripheral neuropathy occurs in type II and type III cryoglobulinaemia,
rather than in type I, and may be present clinically as mononeuropathy,
multiple mononeuropathy, or polyneuropathy.
The neuropathy may be classified as predominantly sensory axonopathy.
Nerve biopsy shows mainly axonal degeneration; two main pathogenic
mechanisms have been suggested: interference with the vasa nervorum
microcirculation by intravascular deposits of cryoglobulins and
vasculitis-induced ischaemia.
A rarely reported third possible mechanism, immunologically mediated
demyelination, has not been supported by subsequent studies.