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Neurology 13th peripheral neuropathy
- 1. NEUROLOGY Dr. Rami Abo Ali Neurology - Dr. Rami Abo Ali 1
- 3. PERIPHERAL NEUROPATHY Peripheral neuropathy (PN) describes disorders of peripheral nerves, including the dorsal or ventral nerve roots (radiculopathy) dorsal root ganglia brachial or lumbosacral plexus (plexopathy) and other sensory, motor, autonomic, or mixed nerves (neuropathy). 3 Neurology - Dr. Rami Abo Ali
- 4. Peripheral neuropathy (PN)are classified according to the following criteria: 1-pathology: demyelinating; axonal or mixed. 2-size: small or large nerve fibers. 3-function: sensory; motor; autonomic, or mixed. 4-distribution: Polyneuropathy involves widespread and symmetric dysfunction of the peripheral nerves. Mononeuropathies involves individual peripheral nerve (mononeuropathy simplex) or multiple individual peripheral nerves (mononeuropathy multiplex) plexopathy 4 Neurology - Dr. Rami Abo Ali
- 6. PERIPHERAL NEUROPATHY Etiology: Metabolic/endocrine : Diabetes mellitus, Chronic renal failure, hypothyroidism. Infective: HIV/AIDS, syphilis, leprosy, diphtheria, lyme disease. Immune-mediated/inflammatory: Guillain Barre Syndrome GBS, chronic inflammatory demyelinating neuropathy (CIDP),or vasculitic (Polyarteritis nodosa, Churg-Strauss disease, SLE, Rheumatoid arthritis, and Sjögren's disease). Toxic: HIV drugs, anticancer drugs, alcohol, heavy metals (lead, thallium and arsenic). Nutritional: vitamin B12, B1, B6 deficiencies. Hereditary: Charcot-Marie-Tooth disease (CMT). 6 Neurology - Dr. Rami Abo Ali
- 7. MONONEUROPATHY The most common causes are compression, entrapment, trauma. In an entrapment neuropathy, pressure initially damages the myelin sheath but sustained or severe pressure damages the integrity of the axons. Certain conditions increase the propensity to develop entrapment neuropathies. These include acromegaly, hypothyroidism, pregnancy, diabetes mellitus, and bone damage near the nerve (e.g. rheumatoid arthritis). 7 Neurology - Dr. Rami Abo Ali
- 8. ENTRAPMENT NEUROPATHY CARPAL TUNNEL SYNDROME Due to compression of the median nerve as it passes under the flexor retinaculum and through the carpal tunnel at the wrist; commonly bilateral. Predisposing conditions include: pregnancy local deformity (e.g. secondary to osteoarthritis, fracture) diabetes mellitus rheumatoid arthritis hypothyroidism acromegaly amyloidosis. It is characterized by pain and tingling paraesthesia in the hand or arm, typically worse at night, with wasting of the muscles of the thenar eminence and sensory loss in the distribution of the median nerve (radial three-and-a-half digits). 8 Neurology - Dr. Rami Abo Ali
- 9. Tinel’s (tapping over the median nerve) and Phalen’s (forced flexion of the wrist) tests may reproduce tingling paraesthesia. Diagnosis can be confirmed with Nerve conduction studies , and secondary causes should be sought. Treatment depends on severity, but may include splinting (especially at night), local injection of corticosteroids and surgical decompression 9 Neurology - Dr. Rami Abo Ali ENTRAPMENT NEUROPATHY CARPAL TUNNEL SYNDROME
- 11. ENTRAPMENT NEUROPATHY ULNAR NEUROPATHY Typically due to compression of the ulnar nerve at the elbow. (cubital tunnel syndrome) It is characterized by pain and/or paraesthesiae radiating along the ulnar border of the forearm ,with wasting of the small muscles of the hand but sparing of the thenar eminence; there is also sensory loss in the hand in the distribution of the ulnar nerve. Electromyography and Nerve conduction studies can be used to confirm the diagnosis; treatment may involve splinting and/or surgical decompression/transposition of the ulnar nerve. 11 Neurology - Dr. Rami Abo Ali
- 13. ENTRAPMENT NEUROPATHY RADIAL NEUROPATHY Pressure on the radial nerve in the upper arm may lead to wrist drop – most commonly seen with prolonged abnormal posture of the upper arm (e.g. draped over the back of a chair). 13 Neurology - Dr. Rami Abo Ali
- 15. OTHER ENTRAPMENT NEUROPATHY Brachial plexus lesions – including: Erb’s palsy (upper nerve roots) and Klumpke’s palsy (lower nerve roots) typically due to traction injury (e.g. as a result of birth injury ); cervical rib; Pancoast tumour (may be associated with Horner syndrome). Meralgia paraesthetica – numbness in the thigh due to compression of the lateral cutaneous nerve of the thigh as it passes under the inguinal ligament. Lateral popliteal palsy– the common peroneal nerve is susceptible to pressure damage as it travels around the neck of the fibula, resulting in foot drop (weakness of ankle dorsiflexion and eversion and extensor hallucis longus, with variable sensory disturbance); it is more common in diabetes mellitus 15 Neurology - Dr. Rami Abo Ali
- 19. MULTIFOCAL NEUROPATHY/ MONONEURITIS MULTIPLEX An asymmetrical neuropathy affecting two or more peripheral nerves at one time, producing symptoms of numbness, paraesthesiae and sometimes pain in their sensory distribution with associated muscle weakness and wasting. Vasculitis is a common cause, either as part of a systemic disease or isolated to the nerves, or it may arise on a background of a polyneuropathy (e.g. diabetes). 19 Neurology - Dr. Rami Abo Ali
- 21. PERIPHERAL POLYNEUROPATHY Diffuse disease of the peripheral nerves classified according to whether there is sensory or motor involvement or both, and whether the site of disease is the myelin sheath (demyelinating neuropathy) or the nerve fibre (axonal neuropathy). Long-standing disease may result in claw deformities of the foot (pes cavus) and hand and sensory loss may lead to neuropathic ulceration and joint deformity (Charcot arthropathy). Coexistent autonomic symptoms may be present . Numerous causes are recognized : diabetes mellitus carcinomatous neuropathy vitamin B deficiency (including B12) Guillain–Barré syndrome Charcot–Marie–Tooth disease (CMT) 21 Neurology - Dr. Rami Abo Ali
- 23. DIABETIC NEUROPATHY Diabetes mellitus causes a distal, predominantly sensory neuropathy commonly affecting the lower limbs in a stocking distribution. Symptoms of numbness, paraesthesiae and sometimes pain in the feet are associated with loss of vibration and position sense and loss of the ankle reflex. It may be associated with Charcot arthropathy. 23 Neurology - Dr. Rami Abo Ali
- 24. CARCINOMATOUS NEUROPATHY Cancer may be associated with either a sensory neuropathy in a ‘glove-and-stocking’ distribution or motor neuropathy in which there is muscle weakness and wasting, usually of the proximal limb muscles. The neuropathy may be mixed 24 Neurology - Dr. Rami Abo Ali
- 25. VITAMIN B DEFICIENCY Vitamin B1 deficiency, usually seen in patients with alcoholism, presents with a sensory neuropathy characterized by numbness (‘walking on cotton wool’), paraesthesiae, pain and soreness of the feet. In vitamin B12 deficiency the peripheral neuropathy may be associated with megaloblastic anaemia and subacute combined degeneration of the cord Vitamin B12deficiency symptoms may include: strange sensations, numbness, or tingling in the hands, legs, or feet. anemia. a swollen, inflamed tongue. difficulty thinking and reasoning (cognitive difficulties), or memory loss. weakness. fatigue. 25 Neurology - Dr. Rami Abo Ali
- 26. GUILLAIN–BARRÉ SYNDROME Guillain–Barré syndrome (GBS) is a heterogeneous group of immune-mediated conditions with an incidence of 1–2/100 000/ year. In Europe and North America, the most common variant is an acute inflammatory demyelinating polyneuropathy (AIDP). Axonal variants, either motor (acute motor axonal neuropathy, AMAN) or sensorimotor (acute motor and sensory axonal neuropathy, AMSAN), are more common in China and Japan, and account for 10% of GBS in Western countries The hallmark is an acute paralysis evolving over days or weeks with loss of tendon reflexes. About two thirds of those with AIDP have a prior history of infection, and an autoimmune response triggered by the preceding infection causes demyelination. 26 Neurology - Dr. Rami Abo Ali
- 28. GUILLAIN–BARRÉ SYNDROME CLINICAL FEATURES Distal paraesthesia and pain precede muscle weakness that ascends rapidly from lower to upper limbs and is more marked proximally than distally. Facial and bulbar weakness commonly develops, and respiratory weakness requiring ventilatory support occurs in 20% of cases. Weakness progresses over a maximum of 4 weeks (usually less). Rapid deterioration to respiratory failure can develop within hours. Examination shows diffuse weakness with loss of reflexes. A proximal variant of Guillain–Barre´ syndrome exists, involving ocular muscles and associated with areflexia and ataxia (Miller–Fisher syndrome) 28 Neurology - Dr. Rami Abo Ali
- 29. NOTE : Bulbar weakness: lower motor neuron lesion of cranial nerves IX, X, XI and XII results in difficulty in chewing, weakness of the facial muscles, dysarthria, palatal weakness and regurgitation of fluids, dysphagia, and dysphonia 29 Neurology - Dr. Rami Abo Ali
- 30. GUILLAIN–BARRÉ SYNDROME INVESTIGATIONS Diagnosis is usually clinical The CSF protein is raised, but may be normal in the first 10 days. There is usually no increase in CSF white cell count Electrophysiological changes may emerge after a week or so, with conduction block and multifocal motor slowing. Antibodies to the ganglioside GM1 are found in about 25%, usually the motor axonal form. 30 Neurology - Dr. Rami Abo Ali
- 31. GUILLAIN–BARRÉ SYNDROME MANAGEMENT Active treatment with plasma exchange or intravenous immunoglobulin therapy shortens the duration of ventilation and improves prognosis. In severe GBS, both intravenous immunoglobulin (IVIg) and plasma exchange started within 2 weeks of onset hasten recovery with similar rates of adverse effects but IVIg treatment is significantly more likely to be completed than plasma exchange. Supportive measures to prevent pressure sores and deep venous thrombosis are essential. Regular monitoring of respiratory function (vital capacity) is needed in the acute phase, as respiratory failure may develop with little warning 31 Neurology - Dr. Rami Abo Ali
- 32. Overall, 80% of patients recover completely within 3– 6 months, 4% die and the remainder suffer residual neurological disability, which can be severe. Adverse prognostic features include older age rapid deterioration to ventilation evidence of axonal loss on EMG. 32 Neurology - Dr. Rami Abo Ali GUILLAIN–BARRÉ SYNDROME PROGNOSIS
- 33. HEREDITARY NEUROPATHY CHARCOT–MARIE–TOOTH DISEASE (CMT) Charcot–Marie–Tooth disease (CMT) is an umbrella term for the inherited neuropathies. The members of this group of syndromes have different clinical and genetic features. The most common CMT is the autosomal dominantly inherited CMT type 1, usually caused by a mutation in the PMP-22gene. Common signs are distal wasting (‘inverted champagne bottle’ legs), often with pes cavus, and predominantly motor involvement. X-linked and recessively inherited forms of CMT, causing demyelinating or axonal neuropathies, also occur 33 Neurology - Dr. Rami Abo Ali