Clinical neneurology lectures, 5th year medical college.

1. Dr. Mohammad Shaikhani Assistant professor Sulaimani University College of Medicine. DISEASES OF THE PERIPHERAL NERVOUS SYSTEM:

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

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

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

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

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22. Blitaeral LMN Facial palsy:

30. 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).

31. 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 .

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

33. 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 .