AN INTERESTING CASE OF PROGRESSIVE QUADRIPARESIS DR SHILPA

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 : 97.6 0 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.