The lecture has been given on Nov. 27th & Dec. 4th, 2010 by Dr. Mohammad Shaikhani.

1. Dr. Mohammad Shaikhani MBChB-CABM-FRCP-London. Assistant professor Sulaimani University College of Medicine. DISEASES OF THE PERIPHERAL NERVOUS SYSTEM:

3. TYPES /CAUSES OF NEUROPATHIES Focal (monoNP) Multifocal (mononeuropathy multiplex) Generalised (polyneuropathy) Neurophysiology Acute Chronic Acute Chronic Demyelinating NCS( slowed conduction / conduction block) Entrapment Diphtheria Leprosy Paraproteinaemia GBS Suramin toxicity Hereditary CIDP Lymphoma Osteoclastic MM IgM MM Arsenic Amiodarone Diphtheria Axonal: NCS: (reduced or absent action potentials, normal conduction velocities) Severe entrapment Diabetes Vasculitis Lyme disease Cryoglobulinaemia Diabetes Neoplastic Infiltration HIV Sarcoidosis Amyloid Alcohol GBS variants Toxins Critical illness Porphyria Paraneoplastic Tick paralysis Metabolic/ endocrine (as DM) Alcohol Drugs /toxins Vitamin defi Hereditary IgG MM Paraneoplastic Primary amyloidosis

4. Neuropathy: Systemic diseases/toxins Common Unusual Rare Metabolic/endocrine DM CRF Hypothyroidism Porphyria Acromegaly Toxic Alcoholism Chronic liver disease Drugs Lead Radiation Arsenic,Mercury Thallium,Organophosph ,Acrylamide Hexacarbons (glue) Immune-mediated/inflammatory Polyarteritis nodosa Churg-Strauss disease SLE Rheumatoid arthritis Sjögren's disease Cryoglobulinaemia Paraproteinaemia Coeliac disease Sarcoidosis Primary amyloidosis Infective HIV/AIDS Lyme disease Neoplastic Vit def Lymphoma Ca (infiltration /paraneoplastic) ,Myeloma B,E,FA.

7. Entrapment neuropathies Nerve Symptoms Muscle weakness/muscle-wasting Area of sensory loss Median (at wrist) (carpal tunnel syndrome) Pain /d paraesthesia on palmar aspect of hands &fingers, waking the patient from sleep. Pain may extend to arm and shoulder Abductor pollicis brevis Lateral palm & thumb, index, middle and medial half 4th finger Ulnar (at elbow) Paraesthesia on medial border of hand, wasting & weakness of hand muscles All small hand muscles, excluding abductor pollicis brevis Medial palm & little finger& medial half 4th finger Radial Weakness of extension of wrist and fingers, often precipitated by sleeping in abnormal posture, e.g. arm over back of chair Wrist and finger extensors, supinator Dorsum of thumb Peroneal Foot drop, trauma to head of fibula Dorsiflexion and eversion of foot Nil or dorsum of foot Lateral cutaneous nerve of the thigh (meralgia paraesthetica) Tingling / dysaesthesia on lateral border of the thigh Nil Lateral border of thigh

8. Investigations in NEUROPATHIES: First-line tests Second-line tests Occasionally useful tests Haematology Full blood count ESR B 12 / folate Biochemistry Urea, electrolytes, calcium Serum lipids, lipoproteins Vit as B12,E Creatinine Cryoglobulins Phytanic acid (Refsum's disease) Liver function tests Toxic metal / drug screen FBS,GTT/HbA lc Prostate-specific antigen Thyroid function tests Urinary porphyrins Plasma protein electrophoresis Urinary Bence Jones protein Faecal occult blood Immunology VDRL Antiganglioside antibodies Serum autoantibodies (ANF, dsDNA, RF, extractable nuclear antigens) Antineuronal antibodies Other Nerve conduction/EMG Genetic screening tests (e.g. hereditary neuropathies, Friedreich's ataxia) Chest X-ray/CT Mammogram, Abd imaging

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21. Blitaeral LMN Facial palsy:

29. Myopathies: hereditary Gower sign Calf p hypertrophy Winging scapula Type Inheritance . Age at onset. Muscles affected & associated signs. Duchenne MD. Becker Limb girdle. Fascio scapulo humeral Myotonia dystrophica. X linked Res. = AutosoRes. Autoso dominan Autosom D. 3 to 10 ys. Older 10 to 30 ys. 10 to 40. Any( 20 to 60) Proximal legs & arms ,then generalized. Pelvic or shoulder girdle or both. Less sever,may reach adulthood. Fascial ,shoulder & seratous anterior. Temporalis, fascial, sternocleidomastoid, distal limbs ptosis, catarct, testicular atrophy, frontal baldness & myotonia(slow relaxation of contracting muscles).

30. Diagnosis : Muscle enzymes as CPK –MM (myocardial band) which is very high in Duchenne & normal or moderately increased in others. EMG &muscle biopsy. Management: No specific treatment except for physiotherapy & rehabilitation ,although there reports of benefit of steroids in DMD. Genetic counseling: DMD & Myotonia Dyst. Can now be diagnosed by DNA analysis even in the preclinical period or in female carriers or during pregnancy Appropriate advice can be offered to couples before marriage or before deciding to have a child or to decide on abortion .

31. Prognosis : DMD & Myotonia Dystr. Die with HF & respiratory failure DMD within 10 years of diagnosis life span of FSH & limb girdle is not affected.

32. Toxic myopathies : Caused by: toxins as alcohol drugs as carbinoxolone, thiaxide, diuretics, steroids & penicilamine. Metabolic & endocrine myopathies: Causes of acute muscle weakness : Electrolyte abnormalities as hypo & hyperkalemia , hypo & hypercalcemia familial periodic paralysis which may be hypokalemic, normokalemic or hyperkalemic. Causes of chronic metabolic or endocrine proximal myopathies affecting proximal shoulder & pelvic girdle include: hyperthyroidism , hypothyroidism, cushing , Addison & it may be the first presentation of endocrine disorders .

33. MCQs: 1.The following test should be done in ever case of neuropathy to find the cause: A. Complete blood picture. B. ESR. C. ANF. D. Hb1C. E. Serum lead.

34. MCQs: 2.Bell’s palsy can be causes by: A. Pregnancy. B. HSV. C. VZV. D. Hypertension. E. Sarcoidosis.

35. MCQs: 3.Bilateral lower motor neuron facial pals is caused by: A. Sarcoidosis. B. HSV. C. VZV. D. GBS. E. Mumps.

36. MCQs: 4.Gullein-Barre syndrome can follow the following infections: A. CMV. B. Campylobacter. C. Helicobacter pylori. D. Yesrsina. E. E Coli.

37. MCQs: 5.Gullein-Barre syndrome can be manifested by the following: A. Autonomic dysfunction. B. Ataxia. C. Bulbar paralysis. D. subjective sensor loss. E. Opthalmoplegia.

38. MCQs: 6.Gullein-Barre syndrome bad prognostic features include: A. Young age. B. Early nerve conduction study changes. C. Respirator paralysis. D. Axonal loss on NCsS. E. Bulbar paralysis.

39. MCQs: 7. Bad prrognostic signs in Bells palsy include: A. Young age. B. Partial paralysis. C. Nerve conduction abnormalities early in the course. D. Nerve conduction abnormalities after the first week. E. Idiopathic type.

40. MCQs: 8. AIDP differs from CIDP by: A. Response to IVG. B. Response to plasmophariesis. C. Response to steroides. D. The causes. E. Prognosis.