AN INTERESTING CASE OF QUADRIPARESIS - APIapikarnataka.org/events/Aug16/AN INTERESTING CASE OF...
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AN INTERESTING CASE OF PROGRESSIVE QUADRIPARESIS DR SHILPA
Transcript of AN INTERESTING CASE OF QUADRIPARESIS - APIapikarnataka.org/events/Aug16/AN INTERESTING CASE OF...
AN INTERESTING CASE OF
PROGRESSIVE
QUADRIPARESIS
DR SHILPA
CASE:
A 50yr old female, homemaker, hailing from Mandya,
Chief complaints
1. Weakness of left upper limb since 9months.
2. Weakness of right upper limb since 3months.
3. Weakness of left lower limb since 1month.
4. Weakness of right lower limb since 15days.
HISTORY OF PRESENT ILLNESS
c/o left upper limb weakness,
Gradual in onset and was progressive, initially weakness
was seen in fingers. Patient found difficulty in opening jar
and holding utensils while washing.
Then the weakness gradually progressed over a period of
3-4 months to involve the arm & later on patient
developed difficulty in trying to lift objects from the shelf.
c/o right upper limb weakness,
Gradual in onset and progressive. Patient found difficulty
in mixing food, tearing chapathi, buttoning and
unbuttoning clothes, because of which patient took help
from her family members to take food, gradually pt
developed weakness suggestive of proximal muscles, like
difficulty in combing hair.
c/o weakness in left lower limb,
Gradual in onset and progressive. Patient had difficulty in
negotiating and gripping slippers. Later on patient
developed weakness getting up from chair, getting up
from bed and rolling over.
No history of neck pain.
No history suggestive of any sensory abnormality.
No history suggestive of cranial nerves involvement .
No history suggestive of bowel and bladder involvement.
No history of palpitation/ dry eyes/ inapproriate
sweating.
No history of fever.
No history of trauma.
No history of fasiculations or muscle spasm.
No history of exposure to heavy metals.
PAST HISTORY
No H/O diabetes, hypertension, tuberculosis, seizures,
thyroid disorder.
No H/O previous surgeries.
No H/O prolonged drug intake.
PERSONAL HISTORY
Diet : Mixed
Appetite : Reduced
Sleep : Disturbed
Bowel and Bladder : Normal and Regular
Habits : Nil
FAMILY HISTORY
Nothing significant.
GENERAL PHYSICAL EXAMINATION
Middle aged female, moderately built and nourished
conscious, co-operative and well oriented to time, place
and person.
No pallor/ icterus/ cyanosis/ clubbing/ edema/
lymphadenopathy.
Pulse : 84bpm regular high volume on right side and no
radio-radial/ radio-femoral delay. All the peripheral
pulses were felt.
Blood Pressure : 180/110mmhg supine in right arm.
Temperature : 97.60 F
No neurocutaneous markers seen.
No skin lesions noted.
CENTRAL NERVOUS SYSTEM EXAMINATION
Higher mental function: Normal
No cranial nerve abnormality.
Motor system:
1. Inspection: Bilateral wasting of thenar and hypothenar
muscles of hand.
2. Nutrition: RIGHT LEFT
Forearm 19.5cm 19.5cm
Arm 22cm 22cm
Midthigh 40cm 39cm
Midleg 28cm 27cm
3. Tone : Hypotonia noted in all the 4 limbs
4. Power: RIGHT LEFT
a. Shoulder joint
Abduction 1/5 1/5
Adduction 1/5 1/5
Flexion 1/5 1/5
Extension 1/5 1/5
b. Elbow joint
Flexion 1/5 1/5
Extension 1/5 1/5
c. Wrist joint: RIGHT LEFT
Flexion 0/5 0/5
Extenssion 0/5 0/5
d. Hand grip: Weak Weak
e. Hip joint:
Flexion 1/5 1/5
Extension 1/5 1/5
Adduction 1/5 1/5
Abduction 1/5 1/5
f. Knee joint RIGHT LEFT
Flexion 3/5 0/5
Extension 3/5 0/5
h. Toe grip Present Weak
Superficial reflexes: present
Deep tendon reflex: Absent
Plantars: Bilaterally Mute
Sensory system:
Touch, pain, temperature, vibration and joint position sense
normal.
Gait and co-ordination could not be assessed.
Spine and skull: normal and no tenderness.
CVS: S1 S2 heard, no murmurs.
R S: B/L NVBS, no added sounds.
P/A: soft, non tender, bowel sounds present.
PROVISIONAL DIAGNOSIS
CHRONIC INFLAMMATORY DEMYELINATING
POLYNEUROPATHY.
INVESTIGATIONS
Complete blood count:
WBC: 10,000cells/cumm
RBC: 3.5millions/cumm
HB%:10.6gm%
PC: 2.5 lakh/cumm
Peripheral smear: Microcytic hypochromic anemia.
Urine routine: Normal
Blood Urea: 20mg/dl
Serum Creatinine: 0.9mg/dl
ESR : 140mm/hr
Creatinine phosphokinase : 52U/L
C R P : Negative
HbsAg : negative
HIV I and II : negative
VDRL : negative
RA factor: Negative
Nerve conduction studies:
1. Bilateral median and ulnar nerve inexcitable and sensory
conduction normal.
2. Bilateral common peroneal nerve very low to absent
sensory motor action potential.
3. Bilateral sural and superficial peroneal nerve absent
sensory motor action potential.
Suggestive of pure motor neuropathy in upper limb.
Bilateral common peroneal, sural and superficial peroneal
axonopathy in lower limb. Suggestive of mononeuritis
multiplex.
MONONEUROPATHY MULTIPLEX
Diabetes
Polyarteritis nodosa and other inflammatory angiopathic
neuropathies
Mixed cryoglobulinemia
Sjogren sicca syndrome
Sarcoidosis
Ischemic neuropathy with peripheral vascular disease.
Lyme disease
CSF Analysis:
Volume 1.5ml
Cell Count: 02cells/cumm
Cell Type : Lymphocytes
Negative for malignant cells
LDH : 96IU/L
Glucose : 63mg/dl
Protein : 214.2mg/dl
ANA PROFILE: RO 52 POSITIVE
pANCA : Negative
cANCA : Negative
Nerve Biopsy : left sural nerve: S/O VASCULITIC
NEUROPATHY
Biopsy shows transmural infiltrate around nutrient vessel
composed of lymphocytes, histiocytes.
Dense inflammation also seen around small epineurial
arterioles and venules sparing endoneural vessels. There is
no neovascularisation.
Myelinated fiber loss appears multifocal non uinform, in
sectorial pockets. Axonal regenaration is present with
secondary demyelination. Occasional fibers show acute
myelin degeneration.
DIAGNOSIS
ISOLATED VASCULITIC PERIPHERAL
NEUROPATHY.
TREATMENT
Injection Methylprednisolone 1gm OD.
Tablet Amlong 5mg BD.
Tablet Metoprolol 50mg BD.
Tablet Prazosin 2.5mg BD.
Physiotherapthy.
TREATMENT
Tablet Hydroxychloroquine 100mg BD.
Tablet Methotrexate 7.5mg once a week.
Tablet Amitryptaline 25mg OD.
Tablet Pregabalin 75mg OD.
DISCUSSION
A full description of the syndrome of peripheral
neuropathy due to vasculitis without any manifestations of
vasculitis in other systems was first made by Dyck et al. in
1987.
L. MATHEW et al. Department of Neurology, Radcliffe
Infirmary, Oxford, Bristol, UK.
Vasculitis restricted to the peripheral nervous system
(PNS), referred to as nonsystemic vasculitic neuropathy
(NSVN).
DISCUSSION
Vasculitis of the peripheral nervous system (PNS) is rare.
There are no controlled treatment trials, and clinical
practice is guided by experience from case series and
indirectly by analogy with systemic vasculitis.
Of 106 cases, 95 had systemic vasculitis and 11 had
vasculitis confined to the PNS.
DISCUSSION
M. P.COLLIN et al.Department of Neurology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
All vasculitic neuropathies are axonal by electrodiagnostic/pathologic criteria.
Laboratory testing is unremarkable except for mildly elevated erythrocyte sedimentation rate (ESR) in 50%.
Highly elevated ESRs, leukocytosis, rheumatoid factors, and antineutrophil cytoplasmic antibodies (ANCAs) raise concern for underlying systemic vasculitis.
Without a specific clinical/laboratory marker, the condition depends on nerve biopsy for diagnosis.
CONCLUSION
Incidence: 10-15%
Isolated vasculitis affecting the peripheral nerves can
have an indolent presentation and may not be
recognizable as mononeuritis multiplex by the time a
patient presents.
It can only be diagnosed with certainty on nerve biopsy
and a firm diagnosis provides the basis for therapy that is
likely to provide a clear benefit to the patient.
CONCLUSION
It should be considered in any patient who presents with a
progressive sensory and motor neuropathy where a
definite cause cannot be established.
TAKE HOME MESSAGE
Nerve biopsy is the gold standard test for isolated
vasculitis of peripheral nervous system.
Nerve biopsy is frequently delayed or avoided as it is an
invasive procedure .
Vasculitis is not often suspected as an etiological cause for
neuropathy in the elderly.
