AN INTERESTING CASE OF QUADRIPARESIS - APIapikarnataka.org/events/Aug16/AN INTERESTING CASE OF QUADRIPARESIS.pdfNo neurocutaneous markers seen. ...

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.