leprous neuritis management by aseem
Post on 31-May-2015
378 Views
Preview:
TRANSCRIPT
MANAGEMENT OF LEPROUS NEURITIS
Introduction
• Inflammation of the pereipheral nerves (Dermal / Cutaneous / Nerve Trunks)
• Centripetal, Ascending in nature (KGK Dehio) akin to ‘fish swimming upstream’ (Khanolkar)
• Lepra Bacilli invades Peripheral Nerves Inflammation NFI ( S / M / A )
• Mediated by – Schwann cell bacillation– Contact Demyelination – Immune / Inflamm reactions– Mechanical Compression by Intra / Perineural
edema– Segmental demyelination Wallerian / Axonal
degeneration
Stages of nerve involvement
• Stage of parasitization
• Host response
• Clinical involvement
• Nerve damage
• Nerve destruction
CLINICAL FEATURES• Neuritis/neuropathy : Acute/ subacute/ chronic, demyelinating,
nonremitting event involving cutaneous nerves and larger trunks
• NFI : sensory, motor & autonomic nerve deficits
due to pathological processes from infection of nerve
NFIearly Late
Sensory :Altered heat & cold sensitivity, hypoesthesia
Sensory :Hypoesthesia, anesthesia leading to neuropathic ulcers
Motor :Mild motor weakness
Motor :Severe motor weakness progressing to paralysis
Autonomic :Decreased sweating
Autonomic :Severe dryness with fissuring of skin
• Silent (Quiscent) neuritis : progressive sensory or motor impairment
without pain, paraesthesia or tenderness of nerve & no signs of reaction
• Neuropathic pain : Pain initiated or caused by a primary lesion or
dysfunction in peripheral or central nervous system
Grading of neuropathic painGrade Degree Description
0 None No nerve pain
1 Mild Complains of nerve pain even when not asked
2 Moderate Complains severe nerve pain, sleep not disturbed, it is aggravated by repeated use of the limb
3 Severe Pain is severe & it interferes with sleep; patient keeps the limb in rest position & avoids movement
Classification of Neuritis
• Acute neuritis : swelling due to nerve abscess or recent onset rapidly progressing neurological deficit < 06 mo
• Chronic neuritis : long standing > 06 mo of gradually progressive neurological deficit with nerve tenderness or pain
• Recurrent neuritis : an episode of neuritis recurring after a symptom free interval of min 03 mo
• Catastrophic paralysis : sudden paralysis
• Completely destroyed nerves : no residual nerve function and electrophysiological studies show no conduction
Principles of Therapy
• MDT continuation
• Treating complicating Reactional States
• Prolonged Anti-inflammatory therapy
• Surgery
• Rest / Physical Therapy
• Physiotherapy
Anti-inflammatory Therapy
• Corticosteroids
• Clofazimine
• Thalidomide
• AZA
• CsA
• NSAIDs
• Intraneural Drugs
Corticosteroids
• Anti-inflammatory + Immunosuppressive
• Genomic Action (Nuclear Receptors) – Immediate Action (Dec Edema / Pro-inflamm CKs)
• Non-Genomic Action (Cystoplamic Receptors) - Immunosuppressive Action
• Indicated in ACUTE NEURITIS ; as early as detected
WHO regime
Initiate Prednisolone at 40 mg – taper every 02 weeks over 12 weeks (40-30-20-15-10-5-X)
Prolonged Therapy (24 weeks) OR High-dose Therapy (02 mg/kg)
Favourable Response :Sensory > Motor NFI (BANDS)Acute > Chronic > Recurrent Neuritis
(AMFES)
ADRs (TRIPOD)
• Minor (20%) Gastric Intolerance / Fungal Inf / Acne
Major (02%) Peptic Ulcer / Bacterial Sepsis / DM
Immunosuppression may interfere with killing of Bacilli and reduction in Antigenic Load ; Concomitant CLOFAZIMINE
Clofazimine
• Phenazine derivative
• Dec Granulocyte Chemotaxis / stabilizes Lysosomes ; binds to Mycobacterial DNA
• Steroid-sparing agent = Anti-inflamm + Anti-leprosy agent
• ENL / Reduces incidence of T1R
• Slower onset of action
REGIME
• 300 mg daily PO X 12 weeks
• 200 mg daily PO for a few months
• 100 mg daily PO continued
ADRs
Cutaneous / Mucosal pigmentationGastrointestinal IntoleranceIchthyosis
Thalidomide
• Glutamic Acid derivative
• Anti TNF-A
• Immunomodulatory / Anti-inflamm / Hypnosedative effects
• FDA-approved for ENL
• 100-400 mg daily till pain subsides decrease by 50mg every 02-04 weeks
• ADRs
Paradoxical Peripheral Neuropathy50% Reduction in SNAP-a with Normal NCV
Teratogenicity
Proximal Muscle Weakness
Somnolence
Leukopenia
AZA
• Immunosuppessive + Anti-inflamm + SSA
• 6-TP (Guanine) ; purine analogue inhibits cell division , T & B cell function
• 2nd Line Treatment for T1R (ILEP)
• 03 mg/kg/day x 12 weeks with Prednisolone 40mg tapered over 08 weeks
• Pancytopenia / Hepatotoxicity / GI Intolerance
CsA
• Immunosuppressant
• Calcineurin Inhibitor Calcium-Calmodulin complex dec activity of NFAT-1 inhibit IL-2 production Dec activity of CD4+ T-cells ; Reduction of Anti-Nerve Growth Factor (NGF) ABs
• Chronic ENL / T1R / Chronic Neuritis
• 5 mg/kg (upto 7.5 mg/kg) tapered over 12 months
• Nephrotoxicity / Hypertension / Dyselectrolemia / Hypertriglycidemia / Gum Hyperplasia
Intraneural Therapy
• Severe Uncontrolled Neuritic Pain
• Isoxsurpine / Tolazoline (VASODILATORS) help spread Corticosteroids under LA
• Treatment of Claw Hand in 60 yr old over 06 months by Nashed et al
• Intense pain, Nerve fibre damage potential
Chr Neuropathic Pain
• Primary lesion / dysfunction of Nerve produces pain – continuous, burning, Glove-and-Stocking distt
• Late complication of Hansen’s
• Small fibre neuropathy / Persistent Intraneural Inflamm
• MDT-completion + Not in Reaction + No NFI
• NSAIDs not effective
• TCAs (NTP / Amytriptyline)
• AEDs (CBZ)
• GABA–analogues (Gabapentin / Pregablin)
• Opioids - Tramadol
Surgical Correction
• Nerve Sx - improves function Recon Sx – improves disability
• Corticosteroid coverage ?
Indications
• Corticosteroid failure (No improvement / Contraindicated / ADRs)
• Intractable pain despite Medical Management• Nerve Abscess• Sudden paralysis (Catastrophic / Hyperacute Neuritis)
EXTRA-NEURAL NEUROLYSISDecompression Sx – removes fibrotic bands / ligaments to open fibro-osseous channels – relives external pressure
INTRA-NEURAL NEUROLYSISLongitudnal Incisons in Nerve Sheath Epineurium
INTERFASCICULAR NEUROLYSISIndividual Nerve Fibres dissected and separated ; risk of damaging Vasa Nervorum , Fibrosis
NERVE ABSCESS DRAINAGELongitudnal incision drain Caseous material
NERVE TRANSPOSITIONMedial Epicondylectomy for Ulnar Nerve
General Measures
• Rest for Acutely inflamed Nerve• Avoidance of trauma• Immobilization with padded splints• Graduated Exercises in Recovery phase• SWD / UST / TENS for added pain control• Hand / Foot Care• Counselling and MDT
PREVENTION
• Early Detection of Hansen’s / Reactions
• Prompt initiation of MDT
PROPHYLAXIS
• 20mg/day Prednisolone with 1st 04 months of MDT lowered risk of T1R
• 300mg/day Clofazimine for 1st 03 months of MDT lowered incidence of Neuritis
EXPERIMENTAL THERAPY
• Drugs and Vaccines blocking Mycobacterial attachment to Schwann Cell-Axon Unit / Specific Bacterial Unit causing Nerve tropism
• Neutrotropic Factors (NTFs)
Regulate Schwann Cells to regenerate Axons in PNS by increasing Impulse Transmission across Axons blocked by Mycobacterial AGs
THANK YOU
top related