Neuropathy from the internal medicine view

1. Neuropathies
Amr Shaaban Hanafy
Assistant professor of medicine
and Gastroenterology
Zagazig University-Egypt

2. • Definition
• Inflammation or degeneration of the
peripheral or cranial nerves, impairing their
conductivity leading to sensory, motor or
autonomic manifestations.

3. • Classification
• 1-mononeuropathy: single nerve trunk in one
limb
• Causes:
• 1-Trauma: wrong injection as sciatic nerve or
callus compression.
• 2-Inflammation: as Bell’s palsy, trigeminal
neuralgia
• 3-Compression: entrapement neuropathy
• 4-Metabolic: early diabetes
• 5- Vascular: volkman’s ischemic contracture

4. • 2-mononeuropathy multiplex: more than one
nerve trunk in one limb
• Causes:
• 1-Trauma: radiation.
• 2-Inflammation: leprosy and herpes zoster, SLE,
rheumatoid arthritis, scleroderma, sarcoidosis
• 3-Compression: brachial neuralgia
• 4-Metabolic: diabetes
• 5- Vascular: poly arteritis nodosa

5. • 3-polyneuropathy: bilateral symmetrical
affection of the peripheral nerves
• Causes:
• I: Heridofamilial
• 1-hypertrophic interstitial
polyneuropathy(Dejere sottas Disease)
• 2- peroneal muscle atrophy(Charcot Marie
tooth)
• 3- Refsum Disease
• 4- Friedrich’s ataxia

6. • II- Symptomatic:
• 1- infections:
• VIRAL: Mumps , measles, AIDS
• Bacterial : diphtheria, typhus, typhoid, tetanus
• Mycobacterial: leprosy
• Lyme Disease:Borrelia bacteria, by ticks.
expanding area of redness on the skin, known as
erythema migrans, that appears at the site of the
tick bite about a week after it occurred.

7. • 2- Toxic:
• - inorganic: lead, mercury, arsenic, gold, thalium
• -organic: alcohol, insecticides.
• 3-Nutritional:
• Beri Beri, Pellagra, SCD
• 4-Metabolic:
• Diabetes, uremia, porphyria, amyloidosis

8. 5- Endocrinal:
Acromegally, myxedema
6- Neoplastic:
Brochogenic carcinoma, lymphoma, myloma,
macroglobulimia(Waldenstrom)
7- Autoimmune: SLE, PAN, Rheumatoid ARTH.,
Scleroderma, Landry Guillian Barre SYNDROME

9. • III- Idiopathic:
• Critical illness Polyneuropathy

10. • CLINICAL PICTURE OF POLYNEUROPATHY
• I: motor:
• Weakess or paralysis of LMNL pattern:
bilateral, symetrical, more distal,
extensors>flexors leading to foot and hand
drop
• Lost ankle with preserved knee, high steppage
gate.
• Affection of cranial nerves 3,6,7,10

11. • II: Sensory:
• Superficial sensory loss of glove and stock
• Deep sensory loss with lost deep reflexes and in
advanced cases sensory ataxia.
• Tender calf muscles and nerve trunks.
• III: Autonomic:
• Vasomotor: hyperemia the coldness and cynosis
• Sudomotor: initial excessive sweating then
dehydration.
• IV: Trophic changes:
• Loss of hair, brittle nails, trophic ulcers, charcot’s
joints

12. • - specific forms of neuropathy
• 1-hypertrophic interstitial polyneuropathy
(Dejerene sottas Disease)
• In the 1st and 2nd decade, progressive, sensory ,
more distal, the affected nerves are thick tender
may be visible and palpable ("onion-bulb"
appearance).
• 2-Peroneal muscle atrophy:
• Heridofamilial, 1st , 2nd decades, grad onset, slowly
progress.course weakness and wasting of L.L
muscles mainly peronii then the anterior tibial
group, then ascend to the lower one third of the
thigh ( inverted Champagne bottle)

13. • - despite marked wasting, little affection of the power,
impaired sensation with lost vibration sense, skletal
deformities as pes cavus
• - may be associated with HF ataxia.
• 3- REFSUM’ S DISEASE
• AR- Heridofamilial lipid storage disease
• Onset in the first and second decades
• Hypertrophic neuropathy associated with night blindness,
retinitis pigmentosa, nerve deafness, skletal deformitis,
Cardiac conduction abnormalities, icthyosis
• Due to accumlation of phytanic acid due to def of phytanic
acid Co-A hydroxylase
• Treated by plasma pharesis and restriction of diet containg
phytanic acid. (almonds, legumes, rice and wheat brans)

14. • ENTRAPEMENT NEUROPATHY SYNDROME
• 1-Spinal cord and nerve roots
• - spondylosis, Pott’s, fracture dislocation of the
vertebrae, disc prolapse, 1ry and 2nd of the
vertebrae, neurofibromatosis of the nerve
roots, pachmeningtis hypertrophica of SYPHILIS
• 2- Lower brachial plexus:
• Cervical disc, osteophytes, cervical rib, pancost
tumor, LN, subclavian artery aneurysm

15. • 3- peripheral nerves: 4 syndromes
• A- ulner neuropathy
• Entrapment in cubital tunnel by fibrous band
connecting 2 heads of flexor carpi ulnaris
• Claw hands
• B: Carpal tunnel syndrome:
• median nerve at the carpal tunnel. The main
symptoms are pain, numbness, and tingling, in
the thumb, index finger, middle finger, and the
thumb side of the ring fingers.

16. • C- Tarsal Tunnel Syndrome:
• posterior tibial neuralgia, the tibial nerve is
compressed by flexor retinaculum as it travels
through the tarsal tunnel behind the medial
malleolus The posterior tibial artery, tibial nerve,
and tendons of the tibialis posterior, flexor
digitorum longus, and flexor hallucis longus
muscles travel in a bundle through the tarsal
tunnel.
• numbness in the foot radiating to the big toe and
the first 3 toes, pain, burning, electrical
sensations, and tingling over the base of the foot

17. • D- meralgia parathetica
• lateral femoral cutaneous nerve focal
entrapment of this nerve as it passes through
the inguinal ligament through a tunnel
formed by the lateral attachment of the
inguinal ligament and the anterior superior
iliac spine.
• c/p: paresthesias and numbness of the upper
lateral thigh area may be painful.
• Symptoms are typically unilateral. However,
they may be bilateral in up to 20% of cases.
• Walking or standing may aggravate the
symptoms; sitting relieve them.

18. • Diabetic neuropathy
- Diabetic microangiopathy of vasa nervosa
- Astherosclerosis of vasa nervosa
- Polyurea induced loss of vit B1,6,12)
- METABOLIC : Increased sorbitol and fructose with
increased osmolarity and swelling of axons and
schawan cells, and decreased myoinistol

19. • I: SENSORY:
• - In early or pre-diabetes, it is mononeuropathy (sciatic
N, Femoral, Lat. Popleteal, ulner, median, Cranial III, VI,
VII), in advanced diabetes it is polyneuropathy.
• -peripheral symmetrical para or hypersthesia with
nocturnal intensification.
• -superficial sensory loss of glove and stock
• - deep sensory loss with 1st to disappear is vibration
• - deep reflexes are lost , 1st is ankle reflex
• Muscle sense is increased 1st leading to tender calf
muscle
• - trunkal neuropathy : affection of spinal nerves leading
to acute abdominal or chest pain.

20. • II: Motor :
• EARLY DIABETES:
• MONONEUROPATHY: isolated affection of sciatic
or femoral nerves or cranial nerves as VII palsy,
external ophthalmoplegia preceded by severe
frontal headache
• MONONEURITIS MULTIPLEX
• POLYNEUROPATHY: Late diabetes
• A-diabetes amytrophy: bilateral symmetrical
affection of pelvic girdle and thigh muscles.
• B- diabetic neuropathic cachexia: bilateral
symmetrical peripheral weakness with
asscociated wt loss, anorexia

21. • III: Autonomic:
• 1- CVS:
• Postural hypotension
• Persistant tachycardia
• Painless myocardial infarction
• 2- GIT:
• -delayed gastric empyting with gastroparesis
• -Intermittent nocturnal diaarrhea alternating
with
• -disturbed innervation of anal sphincter cause
fecal incontence
• - unawareness of hypoglycemia

22. • 4- Genito urinary:
• -Impotence with loss of deep testicular
sensation
• -bladder: early straining, dribbling – decrease
stream force then sensory atonic bladder
• -Urine retension
• 5- Sweat dysfunction:
• early excessive nocturnal sweating mainly
frontal then anhydrosis mainly L.L
• 6- Vasomotor :

23. • DIAGNOSIS
• TREATMENT
• ● Glycemic control
• ●Foot care
• ●Treatment of pain
• Level A: Pregabalin, 300–600 mg/day
• Level B Gabapentin, 900–3600 mg/day Sodium valproate,
500–1200 mg/day Venlafaxine, 75–225 mg/day Duloxetine,
60–120 mg/day Amitriptyline, 25–100 mg/day
Dextromethorphan, 400 mg/day Morphine sulphate, titrated
to 120 mg/day Tramadol, 210 mg/day Oxycodone, mean 37
mg/day, max 120 mg/day
• Capsaicin, 0.075% qid
• Isosorbide dinitrate spray
• Electrical stimulation, percutaneous nerve stimulation x 3–4
weeks

24. • PORPHYRIA
• -metabolic defect cause excess secretion of
porphyrins in urine
• -Acute : acute relapsing motor neuropathy
associated with acute porphyria with is ppt -
by barbiturate or alcohol with acute -
abdomen, convulsions skin pigmentation ,
burgundy red urine

25. • The treatment goal is to decrease heme synthesis and
reduce the production of porphyrin precursors.
• High doses of glucose can inhibit heme synthesis and
/mild attacks/ 5% glucose in 0.9% sodium chloride
solution, infused intravenously at a rate of 2 L/24 h.
Intravenous glucose in water solutions (eg, dextrose
5% or 10% [D5W, D10W]), should be avoided as they
may aggravate hyponatremia.
• severe attacks, especially those with severe
neurologic symptoms, should be treated with
hematin in a dose of 4 mg/kg/d for 4 days.

26. -pain control is best achieved with narcotics.
-Laxatives and stool softeners should be administered with
the narcotics to avert exacerbating existing constipation.
-Symptomatic treatment also includes the use of beta-
blockers to control tachycardia and prevent arrhythmia;
-Nausea and vomiting can be controlled with olanzapine,
lorazepam, or prochlorperazine.
-Treat seizures with gabapentin. Most classic antiseizure
medicines are contraindicated, as they can lead to acute
porphyria attacks.
-gonadotrophin-releasing hormone analogues (for women
with attacks related to their menstrual cycles) and
prophylactic hematin infusions.
-Rarely, liver transplantation may be indicated for patients
with intractable recurrent attacks that are life-threatening
or severely affect quality of lif

27. • Guillian Barre Syndrome
• Allergic or auto immune reaction to previous exposure
to vaccines, upper RTI, Gastroenteritis (EBV, HERPES,
HIV), Mycoplasma, Cambylobacter Gastroenteritis
• C/P: FEBRILE stage, Latent Stage,
• Paralytic stage: acute progressive weakness or paralysis
involve L.L and ascend to affect abdominal and
respiratory muscles. Wasting is not comparable to
weakness
• Superfacial and deep sensory loss with autonomic
affection, tender calf muscles
• Cranial N affections as bilater facial and bulber
symptoms
• CSF : cytoalbuminous dissociation, NERVE
CONDUCTION VELOCITY, EMG

28. • Variants:
• 1-Fisher syndrome:
• Ophthalmoplegia, fascial diplegia, ataxia,
areflexia
• 2-acute axonal motor neuropathy:
• more severe

29. • Cytoalbuminologic dissociation is the finding of
high protein in the cerebrospinal fluid without
many (usually less than 6) white blood cells.
This is a characteristic of Guillain-Barre
syndrome but is not completely diagnostic.
• OTHERS:
• TRANSVERSE MYLEITIS
• DIPHTHERIA, LEAD POISING
• INFRATENTORIAL TUMORS
• VENOUS SINUS THROMBOSIS
• SPINAL COMPRESSION BY TUMOR OR ABSCESS

30. • Diphtheria neuropathy
• 2-8 weeks after appearance of diphtheria
• Cranial nerves as III, VII, X with bulber
symptoms
• Or motor polyneuropathy

31. • LEPROTIC NEUROPATHY
• MYCOBACTERIUM LEPRA
• The nerves are infiltrated by leprous granuloma
wit thickening and degeneration
• - mainly lateral popleteal, ulner, greater auricular,
trigeminal and fascial
• Nodules, fibrosis, trophic changes and gangrene
• - SENSORY NEUROPATHY
• 1-NODULAR
• 2-MACULOANETHETIC: loss of pain with
preserved touch
• 3-MIXED

32. • Histology and detection of organism from
nodules, nasal mucosa, thickened nerves
• Lepromin test
• TREATMENT
• DAPSON 300 mg/week for 3 years
• Rifampicin: 500 mg.d

33. • ALCOHOL NEUROPATHY
• Sensory
• Due to decreased absorption of vit B1
• Associated Korsakov's Spychosis
• HEAVY METAL NEUROPATHY
• 1-LEAD:
• Motor, UL>LL with spare of brachioradialis, blue
line of gum, basophilic stippling of RBC’s,
constipation , abdominal pain, hypertension
• Treatment: EDTA 1gm dissolved in 500 cc55
dextrose iv for 5 days

34. • 2- ARSENIC:
• Sensory with affection of deep sensation
• Diarrhea, hyperkeratosis of palm and sole
• White line in nail
• Treatment:
• BAL (british anti lewisite) 3-5 mg/kg /6hr IM
FOR 1 WEEK

35. • NUTRITIONAL DEFICIENCY
• VIT B1: THIAMINE
• VIT B2: RIBOFLAVIN
• B3: NIACIN
• B5: PANTOTHENIC
• B6: PYRIDOXIN
• B7: BIOTIC
• B9: FOLIC
• B12: COBALAMYN

36. • 1-PELLAGRA:
• deficiency of niacin
• MAIZE CONSUMER
• Malabsorption, liver cirrhosis, alcoholism, carcinoid
syndrome: tryptophan to serotoin
• Hartnup disease
• INH therapy
• C/P:
• Cutanous
• Gastrointestinal: diarrhea , glossitis, dysphagia ,
hepatomegally due to decrease of lipotrophic factors
• - neurological : sensory neuropathy, pellageral lateral
sclerosis, psychosis ,suicidal attempts

37. • 2-
• SUBACUTE COMBINED DEGENERATION
• VEGETERIANS
• PERNICIOUS ANEMIA, ATROPHIC GASTRITIS,
GASTECTOMY, MALABSORPTION OR D. LATUM
INFESTATION.
• INFANCY AND PREGNANCY
• LIVER CIRRHOSIS

38. • C/P:
• ANEMIA
• GIT:
• NEUROLOGICAL:
• # PYRAMIDAL , PN, POST COLUM
• INVESTIGATIONS
• TREATMENT

39. • BERI –BERI
• Deficiency of thiamine with affection of CVS
(WET) or CNS (DRY).
• C/P:
• WET: CHF
• DRY: Sensory
• Cerebral: wernickes, amnesia, nystagmus,
ophthalmoplegia