1. DISEASES OF PERIPHERAL NERVES DR BASHAR SHAKER

2. Numerous inherited and acquired pathological processes may affect the nerve roots (radiculopathy), the nerve plexuses (plexopathy) and/or the individual nerves (neuropathy).

3. Cranial nerves 3-12 share the same tissue characteristics as peripheral nerves elsewhere and are subject to the same range of diseases. Nerve fibres of different types (motor, sensory or autonomic) and of different sizes may be variably involved. Disorders may be primarily directed at the axon, the myelin sheath (Schwann cells) or the vasa nervorum

4. PATTERN OF INVOLVMENT Mononeuropathy Simplex=Single Nerve Mononeuropathy Multiplex=Several Nerves Randomly &Noncontiguously Polyneuropathy( Peripheral Neuropathy)=Dysfunction of Numerous Peripheral Nerves at the Same Time leading to predominantly distal & symmetrical deficit usually affecting lower more than upper limbs

5. Types &Causes of peripheral neuropathy للاطلاع

6. Diseases of Peripheral Nerves SYMPTOMS &SIGNS 1.Sensory Disturbances 1.Sensory Disturbances A.Numbness, Hyperpathia, Impaired Sensation & A.Numbness, Hyperpathia, Impaired Sensation & SPONTANEOUS PAIN esp. in SMALL FIBER SPONTANEOUS PAIN esp. in SMALL FIBER involvement ;D. M., Porphyria, AIDS, Alcoholic, involvement ;D. M., Porphyria, AIDS, Alcoholic, Entrapment ….. Entrapment ….. B. Dissociated Sensory Loss B. Dissociated Sensory Loss Small Fib. Pain &Temp. Small Fib. Pain &Temp. Large Fib. Touch, Vib. & Position Large Fib. Touch, Vib. & Position

7. 2. MOTOR DEFICITS Weakness, Wasting, Fasciculation Weakness, Wasting, Fasciculation Diminished or Absent Reflexes Diminished or Absent Reflexes i.e. LMNL i.e. LMNL 3.AUTONOMIC DISTURBANCES Post. Hypotension, Coldness, Imp. Sweating, Impotence ….esp. GBS, Diabetes, Renal Failure, Porphyria …. Post. Hypotension, Coldness, Imp. Sweating, Impotence ….esp. GBS, Diabetes, Renal Failure, Porphyria …. 4. ENLARGED NERVES Leprosy, Amyloidosis, HSMN, Refsum Dis., Acromegaly.. Leprosy, Amyloidosis, HSMN, Refsum Dis., Acromegaly..

8. Causes of P. N. 1. Inflammatory: GBS, CIPD 1. Inflammatory: GBS, CIPD 2. Metabolic &Nutritional :D.M.,Uremia, Liver Failure, Hypothyroidism, Acromegaly, B12 Deficiency…. 2. Metabolic &Nutritional :D.M.,Uremia, Liver Failure, Hypothyroidism, Acromegaly, B12 Deficiency…. 3.Infectious &Granulomateous:AIDS, Leprosy, Diphtheria, Sarcoidosis… 3.Infectious &Granulomateous:AIDS, Leprosy, Diphtheria, Sarcoidosis… 4.Vasculitis: PAN, SLE, Rh.Arthritis… 4.Vasculitis: PAN, SLE, Rh.Arthritis… 5.Neoplastic &Paraneoplastic 5.Neoplastic &Paraneoplastic 6. Drugs &Toxins:Alcohol, INH, Vinicristine, Phenytoin, Heavy Metals…. 6. Drugs &Toxins:Alcohol, INH, Vinicristine, Phenytoin, Heavy Metals…. 7.Hereditory:HSMN, HSN, Refsum Disease,Porphyria.. 7.Hereditory:HSMN, HSN, Refsum Disease,Porphyria.. 8. IDIOPATHIC 8. IDIOPATHIC

9. Drugs causing peripheral neuropathy للاطلاع Amiodarone, statins, hydralazine Cardiovascular agents Cisplatine, thalidomide, vincristine Chemotheraputic agents Chloramphenicol, INH, ETB, nitrofurantoin, metronidazole Anti-infective agents Gold, disulfiram, pyridoxine, tacrolimus, colchicine, phenytoin Others

10. EVALUATION OF PATIENTS TIME COURSE=Acute; Inflamm., Infectious, Toxins… TIME COURSE=Acute; Inflamm., Infectious, Toxins… Chronic;Hereditory, Metabolic… Chronic;Hereditory, Metabolic… AGE= Early; Hereditory AGE= Early; Hereditory Late; Metabolic, Neoplastic Late; Metabolic, Neoplastic OCCUPATION= Exposure to toxins OCCUPATION= Exposure to toxins MEDICAL HISTORY MEDICAL HISTORY DRUGS DRUGS FAMILY HISTORY FAMILY HISTORY

11. DIFFERENTIAL DIAGNOSIS Diseases of muscles &n.m. junction Diseases of muscles &n.m. junction normal sensation& tendon reflexes normal sensation& tendon reflexes Diseases of spinal cord Diseases of spinal cord pyramidal signs &sensory level below the lesion pyramidal signs &sensory level below the lesion Radiculopathies Radiculopathies dermatomal &myotomal distribution dermatomal &myotomal distribution

12. INVESTIGATIONS CONFIRM DIAGNOSIS CONFIRM DIAGNOSIS EMG = DENERVATION EMG = DENERVATION ENG &NCV = Demyelination or Axonal Degeneration ENG &NCV = Demyelination or Axonal Degeneration REVEAL UNDERLYING CAUSE REVEAL UNDERLYING CAUSE

13. INVESTIGATION OF PERIPHERAL NEUROPATHY للاطلاع

15. TREATMENT UNDERLYING CAUSE NURSING CARE ? ULCERS &CONTRACTURES RESPIRATORY MONITORING &MANAGEMENT CARE OF SKIN &NAILS RELIEF OF PAIN ----Lancinating Pain--PHENYTOIN C ARBAMAZEPINE M MEXILETINE C Constant Pain----AMITRIPTYLINE G GABAPENTINE

16. GUILLIAN BARIE SYNDROME ACUTE ASCENDING POLYRADICLONEUROPATHY This syndrome of acute paralysis develop in 70 % of patients 1-4 weeks after respiratory infection or diarrhoea (particularly Campylobacter). In Europe and North America, acute inflammatory neuropathy is most commonly demyelinating ( AIDP ). Axonal variants,either ( AMAN ) or (ASMAN) are more common in China and Japan.

17. Clinical features Distal paraesthesia and limb pains precede a rapidly ascending muscle weakness from lower to upper limbs, more marked proximally than distally. Facial and bulbar weakness commonly develops, and respiratory weakness requiring ventilatory support occurs in 20 % of cases. In most patients weakness progresses for 1-3 weeks but rapid deterioration to respiratory failure can develop within hours

18. On examination there is diffuse weakness with widespread loss of reflexes. An unusual axonal variant described by Miller Fisher comprises the triad of ophthalmoplegia, ataxia and areflexia. 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 and evidence of axonal loss on EMG.

19. Investigations The CSF protein is abnormal at some stage of the illness, but may be normal in the first 10 days. There is usually no rise in CSF cells ( lymphocytosis of > 50/ml suggests an alternative diagnosis ). Electrophysiological studies are often normal in the early stages but show typical changes after a week or so, with conduction block and multifocal motor slowing, sometimes most evident proximally as delayed F-waves.

20. Management During the phase of deterioration, regular monitoring of respiratory function ( vital capacity and arterial blood gases) is required, as respiratory failure may develop with little warning and require ventilatory support. Ventilation may be needed if the vital capacity falls below 1L, but ventilation is more often required because of bulbar weakness leading to aspiration.

21. General management to protect the airway and prevent pressure sores and venous thrombosis is essential. Corticosteroids have been shown to be ineffective. Plasma exchange and intravenous immunoglobulin therapy shorten the duration of the illness,reduce severity and improve prognosis provided treatment is started within 14 days of the onset of symptoms.

22. Chronic demyelinating polyneuropathy للاطلاع It is either hereditary or immune-mediated. Charcot-Marie-Tooth (CMT) disease which is of many types ;the most commom 70-80 % is the autosomal dominant one causing distal wasting (inverted champagne bottle or stork leg ) often with pes cavus and a predominantely motor involvement

23. Chronic demyelinating polyneuropathy Presents with a relapsing or progressive generalized neuropathy. Sensory,motor or autonomic nerves can be involved but the signs are predominantely motor. Multifocal Motor Neuropathy MMN is a variant with motor involvement only.

24. CIDP للاطلاع CIDP usually responds to immunosuppressive treatment ;corticosteroids, methotrexate or cyclophosphamide OR to immunomodulatory treatments (plasma exchange or IVIg ),which is the best of patients with MMN. About 10 % of patients with acquired demyelinating polyneuropathy have an abnormal serum paraprotein, sometimes associated with a lymphoproliferative malignancy.

25. DIABETIC NEUROPATHY AMYOTROPHY = PAIN &WEAKNESS WITH ATROPHY PELVIC GIRDLE &THIGH MUSCLES WITH ABSENT KNEE REFLEX &LITTLE SENSORY LOSS AMYOTROPHY = PAIN &WEAKNESS WITH ATROPHY PELVIC GIRDLE &THIGH MUSCLES WITH ABSENT KNEE REFLEX &LITTLE SENSORY LOSS MONONEUROPATHY = ACUTE PAINFUL CRANIAL NERVES MONONEUROPATHY = ACUTE PAINFUL CRANIAL NERVES BOTH HAVE GOOD PROGNOSIS BOTH HAVE GOOD PROGNOSIS

26. Entrapment neuropathy Focal compression or entrapment is the usual cause of mononeuropathy. However, some patients present with what initially appears to be a single nerve lesion and then go on to develop multiple nerve lesions.This is termed mononeuritis multiplex.

27. Symptoms and signs Area of sensory loss Muscle weakness/muscle -wasting SymptomsNerve Lateral palm and thumb, index, middle and medial half 4th finger Abductor pollicis brevis Pain and paraesthesia on palmar aspect of hands and fingers, waking the patient from sleep. Pain may extend to arm and shoulder Median (at wrist) (carpal tunnel syndrome) Medial palm and little finger, and medial half 4th finger All small hand muscles, excluding abductor pollicis brevis Paraesthesia on medial border of hand, wasting and weakness of hand muscles Ulnar (at elbow) (at elbow)

28. Area of sensory loss Muscle weakness/musc le-wasting SymptomsNerve 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 Nil or dorsum of foot Dorsiflexion and eversion of foot Foot drop, trauma to head of fibula Peroneal Lateral border of thigh Nil Tingling and dysaesthesia on lateral border of the thigh Lateral cutaneous nerve of the thigh (meralgia paraesthetica)

29. In an entrapment neuropathy, pressure initially damages the myelin sheath, and neurophysiology will show slowing of conduction over the relevant site. Sustained or severe pressure damages the integrity of the axons, demonstrable as loss of the sensory action potential distal to the site of compression.

30. Certain conditions increase the propensity to develop entrapment neuropathies. These include acromegaly, hypothyroidism, pregnancy, any pre-existing mild generalised axonal neuropathy (e.g. diabetes), and oseophytes. Patients with multiple recurrent entrapment neuropathies, especially at unusual sites, should be screened for autosomal dominant hereditary neuropathy with liability to pressure palsies (HNPP).

31. Unless axonal loss has occurred, entrapment neuropathies will recover, provided the pressure on the nerve is relieved, either by avoiding precipitating activities or limb positions, or by surgical decompression.

32. Entrapment neuropathy CTS MEDIAN N. COMPRESSION AT THE WRIST MEDIAN N. COMPRESSION AT THE WRIST IDIOPATHIC, PREGNANCY, TRAUMA, ARTHRITIS, MYXOEDEMA, ACROMEGALY, TENOSYNOVITIS …… IDIOPATHIC, PREGNANCY, TRAUMA, ARTHRITIS, MYXOEDEMA, ACROMEGALY, TENOSYNOVITIS …… PAIN, NUMBNESS IN MEDIAN N. DISTIBUTION ?SHOULDER PAIN, NUMBNESS IN MEDIAN N. DISTIBUTION ?SHOULDER > AT NIGHT > AT NIGHT WEAKNESS & ATROPHY OF THENAR MUSCLES WEAKNESS & ATROPHY OF THENAR MUSCLES TINEL SIGN, PHALEN MANEUVER TINEL SIGN, PHALEN MANEUVER Rx LOCAL STEROIDS, WRIST SPLINT, SURGERY Rx LOCAL STEROIDS, WRIST SPLINT, SURGERY

33. Facial nerve palsy Idiopathic facial nerve palsy or Bells palsy is a common condition affecting all ages and both sexes.The lesion is within the facial canal and may be due to reactivation of latent herpes simplex virus 1 infection.Symptoms usually develop subacutely over a few hours, with pain around the ear preceding the unilateral facial weakness. Patients often describe the face as numb but there is no objective sensory loss (except possibly to taste).

34. Hyperacusis can occur if the nerve to stapedius is involved, and there may be diminished salivation and tear secretion. Examination reveals an ipsilateral lower motor neuron facial nerve palsy. Vesicles in the ear or on the palate indicate that the facial palsyis due to herpes zoster rather than Bells palsy.

35. Prednisolone 40-60 mg daily for a week speeds recovery if started within 72 hours.Artificial tears and ointment prevent exposure keratitis and the eye should be taped shut overnight. About 80% of patients recover spontaneously within 12 weeks. A slow or poor recovery is predicted by complete paralysis, older age and reduced facial motor action potential amplitude after the first week. Recurrences can occur but should prompt further investigation. Aberrant re-innervation may occur during recovery, producing unwanted facial movements (e.g. eye closure when the mouth is moved) or 'crocodile tears' (tearing during salivation).

36. MOTOR NEURONE DISEASE DEGENERATION OF MOTOR NEURONS IN SPINAL CORD MOTOR NUCLIE OF LOWER CRANIAL NERVES, DEGENERATION OF MOTOR NEURONS IN SPINAL CORD MOTOR NUCLIE OF LOWER CRANIAL NERVES, ONSET 30 -60 YEARS, > IN MALES, 2/ 100000, ONSET 30 -60 YEARS, > IN MALES, 2/ 100000, SPORADIC, 5- 10 %FAMILIAL ( AUT. DOM. ) CHR 15 SPORADIC, 5- 10 %FAMILIAL ( AUT. DOM. ) CHR 15 CAUSE : UNKNOWN CAUSE : UNKNOWN ?AUTOIMMUNE ?AUTOIMMUNE INCREASED OFR FORMATION INCREASED OFR FORMATION REDUCED NEUROTROPHIC FACTORS REDUCED NEUROTROPHIC FACTORS EXCITOTOXINS EXCITOTOXINS

37. CLINICAL TYPES &FEATURES A. PROGRESSIVE BULBAR PALSY = LMN CRANIAL NERVES B.PSEUDOBULBAR PALSY = UMN CRANIAL NERVES C.SPINAL MUSCULAR ATROPHY = LMN SPINAL CORD AHC D. PURE LATERAL SCLEROSIS = UMN IN LIMBS E. AMYOTROPHIC LAT. SCLEROSIS = MIXED C. & D. MAY BE WITH A. & B. NO EXTRA OCULAR MUSCLES INVOLVEMENT NO SPHINCTER INVOLVEMENT NO SENSORY DEFICIT NORMAL CSF EMG Rx = RILUZOLE 100mg /day may slow progression & reduce mortality. It is GLUTAMATE ANTAGONIST. Side effects = fatigue, dizziness, GIT diturbance, raised liver enzymes. Anticholinergics for drolling of saliva Physiotherapy FEEDING = Semisolid diet, NG tube, Gastrostomy. Tracheostomy Prognosis = Bad especially in Bulbar type Progressive &fatal in 3 -5 years CLINICAL TYPES &FEATURES A. PROGRESSIVE BULBAR PALSY = LMN CRANIAL NERVES B.PSEUDOBULBAR PALSY = UMN CRANIAL NERVES C.SPINAL MUSCULAR ATROPHY = LMN SPINAL CORD AHC D. PURE LATERAL SCLEROSIS = UMN IN LIMBS E. AMYOTROPHIC LAT. SCLEROSIS = MIXED C. & D. MAY BE WITH A. & B. NO EXTRA OCULAR MUSCLES INVOLVEMENT NO SPHINCTER INVOLVEMENT NO SENSORY DEFICIT NORMAL CSF EMG Rx = RILUZOLE 100mg /day may slow progression & reduce mortality. It is GLUTAMATE ANTAGONIST. Side effects = fatigue, dizziness, GIT diturbance, raised liver enzymes. Anticholinergics for drolling of saliva Physiotherapy FEEDING = Semisolid diet, NG tube, Gastrostomy. Tracheostomy Prognosis = Bad especially in Bulbar type Progressive &fatal in 3 -5 years

38. MND IN CHILDREN للاطلاع INFANTILE = WERDING HOFFMANN DIS. ONSET AT 3 MONTHS, DEATH INFANTILE = WERDING HOFFMANN DIS. ONSET AT 3 MONTHS, DEATH AT 3 YEARS, AR, DIFFICULT SUCKING, SWALLOWING& AT 3 YEARS, AR, DIFFICULT SUCKING, SWALLOWING& VENTILATION, ATROPHY & FASCICULATION OF TONGUE & VENTILATION, ATROPHY & FASCICULATION OF TONGUE & LHMB MUSCLES, NO SENSORY DEFICIT, NO Rx LHMB MUSCLES, NO SENSORY DEFICIT, NO Rx INTERMEDIATE = CHRONIC W-H-DISEASE INTERMEDIATE = CHRONIC W-H-DISEASE AR, 2 nd of 1 st year, LESS BULBAR INVOLVEMENT, AR, 2 nd of 1 st year, LESS BULBAR INVOLVEMENT, > BENIGN, SLOWLY PROGRESSIVE, KYPHOSCOLIOSIS > BENIGN, SLOWLY PROGRESSIVE, KYPHOSCOLIOSIS &CONTRACTURES, SURVIVE TO ADULTHOOD &CONTRACTURES, SURVIVE TO ADULTHOOD JUVENILE = KUGELBERG – WELANDER DISEASE JUVENILE = KUGELBERG – WELANDER DISEASE AR, COULD BE AD OR X – LINKED AR, COULD BE AD OR X – LINKED ONSET AT CHILDHOOD OR EARLY ADOLESCENCE ONSET AT CHILDHOOD OR EARLY ADOLESCENCE MORE IN PROXIMAL MUSCLES, LITTLE BULBAR INVOLV. MORE IN PROXIMAL MUSCLES, LITTLE BULBAR INVOLV. DISABILITY IN EARLY ADULT LIFE DISABILITY IN EARLY ADULT LIFE NO Rx NO Rx

39. SYRINGOMYELIA للاطلاع CAVITATION OF SPINAL CORD CAVITATION OF SPINAL CORD COMMUNICATING = CENTRAL CANAL &THE CAVITY COMMUNICATING = CENTRAL CANAL &THE CAVITY NON – COMMUN. = CYSTIC DILATATION OF SPINAL CORD NON – COMMUN. = CYSTIC DILATATION OF SPINAL CORD CLINICALLY = DISSOCIATED SENSORY LOSS CLINICALLY = DISSOCIATED SENSORY LOSS WEAK. & WASTING OF MUSCLES ; CERVICAL &T 1 WEAK. & WASTING OF MUSCLES ; CERVICAL &T 1 PYRAMIDAL SIGNS &SPHINCTER DIST. BELOW PYRAMIDAL SIGNS &SPHINCTER DIST. BELOW NECK & RADICULAR PAIN NECK & RADICULAR PAIN HORNER SYNDROME HORNER SYNDROME SYRINGOBULBIA SYRINGOBULBIA MAY BE ASSO. WITH TUMOR, TRAUMA, ARACHNOIDITIS OR ANMALIES LIKE ARNOLD – CHIARI MALFORMATION MAY BE ASSO. WITH TUMOR, TRAUMA, ARACHNOIDITIS OR ANMALIES LIKE ARNOLD – CHIARI MALFORMATION Rx = DECOMPRESSION OF DISTENDED SYRINX Rx = DECOMPRESSION OF DISTENDED SYRINX

40. SUBACUTE COMBINED DEGEN. OF THE CORD للاطلاع B12 DEFICIENCY B12 DEFICIENCY PARASTHESIA &WEAKNESS OF THE EXTREMITIES = PERIPHERAL PARASTHESIA &WEAKNESS OF THE EXTREMITIES = PERIPHERAL NERVES NERVES SPASTIC PARAPARESIS = PYRAMIDAL FIBERS SPASTIC PARAPARESIS = PYRAMIDAL FIBERS SENSORY ATAXIA = POSTERIOR COLUMN TRACT SENSORY ATAXIA = POSTERIOR COLUMN TRACT LHERMITTS SIGN LHERMITTS SIGN SCOTOMAS = OPTIC ATROPHY SCOTOMAS = OPTIC ATROPHY BEHAVIOURAL OR PSYCHIATRIC SYMPTOMS BEHAVIOURAL OR PSYCHIATRIC SYMPTOMS ANEMIA MEGALOPLASTIC NOT NECESSARILY PRESENT ANEMIA MEGALOPLASTIC NOT NECESSARILY PRESENT Rx B 12 I. M. ……. Rx B 12 I. M. …….