Evaluation of the patient

with
Peripheral Neuropathy
Nani Kurniani

Three basic challenges

Complaints and the physical finding
are the result of the peripheral
neuropathy
Type and cause of neuropathy
The Apropiate theraphy.

Table. 1. Anatomic Distribution of Neuropathies and

differential Diagnosis
MONONEUROPATHY
Entrapment neuropathies
Trauma
Every cause listed under multiple mononeuropathies(below)
may start with single nerve involvement
MULTIPLE MONONEUROPATHIES
Hereditary liability to pressure palsies
Vasculitic neuropathy
Diabetic mononeuropathies and lumbosacral radiculoplexus
neuropathy
Sarcoid
Leprosy
Multifocal motor neuropathy
AIDS
Tangier disease
Focal variant of chronic inflammatory demyelinating
polyradiculoneuropathy (may affect one or more nerves of a

Table. 1. Anatomic Distribution of Neuropathies and

differential Diagnosis
SYMMETRIC POLYNEUROPATHIES
Inflammatory/Immune-Mediated Neuropathies
Guillain Barres Syndrome
Chronic inflammatory demyelineting polyradiculoneuropathy
Vasculitic neuropathy
Sarcoid neuropathy
Neuropathies associated with connective tissue disease
Toxic Neuropathies
Drugs, metals, alcohol
Inherited Neuropathies
Hereditary motor sensory neuropathies
Giant axonal neuropathy
Hereditary sensory and autonomic neuropathies
Peroxisomal disorder (adrenomyeloneuropathy, refsum
disease)
Porphyric neuropathies
Lipoprotein disorders (Tangier disease, abetaliproteinemia)
Lysosomal enzyme deficiency (Fabry disease)

Table. 1. Anatomic Distribution of Neuropathies and

differential Diagnosis
SYMMETRIC POLYNEUROPATHIES (contd)
Vitamin Deficiensies
Cobalamin, vitamin E, thiamine
Neuropathies Associated with Cancer
Remote effects of cancer
Direct tumor infiltration
Paraproteinnemia-Related Neuropathies
Diabetic Polyneuropathy
Neuropathies Associated with Organ System Failure
Kidney, lung, liver
Critical illness polyneuropathy
Neuropathies associated with organ transpalntation
Neuropathies Associated with Infection
HIV
Leprosy
Lyme disease

History and Examination of the

Patient
Historical information :
Chief complaint and History of present
illness
Past medical history
Family history
Sosial history
Review systems

Table. 2. Drugs Causing Peripheral

Neuropathy Nitrous oxide
Almitrine
Amiodane
Chloroquine
Cisplatin
Colchicine
Dapsone
Didanosine
Disulfiram
Doxorubicin
Ethambutol
FK 506
Gold salts
Isoniazid
Metronidazole
Misonidazole

Nitrofurantoin
Non-depolarizing neuromuscular
blocking agents
Paclitaxel (taxol)
Perhexiline
Phenytoin
Procainamide
Pyridoxine excess
Stavudine
Suramin
Thalidomide
Vinca alkyloids (vinblastine,
vincristine, vindesine,
vinorelbine)
Zalcitabine

Table. 3. Occupation and Habits Predisposing to

Peripheral Neuropathy
Occupations

Neuropathy

Dentist,dental hygienist

Nitrous oxide

Cabinetmakers, painters

Hexacarbons

Farmers

Organophosphates

Dry cleaner, rubber workers

Trichloroethylene

Manufactures of batteries,
plastics, paints, welder,
printers, demolition crews,
firearms instructors

Lead

Copper smelters, tree

sprayers, taxidermists,
farmers, jewelers, painters,
textile printers

Arsenic

Plastic industry workers

Acrylamide

Rayon industry workers

Carbon disulfide

Table. 3. Occupation and Habits Predisposing to

Peripheral Neuropathy
Habit/Behavior

Neuropathy

Smoking

Paraneoplastic

Excess alcohol

Nutritional/vitamin deficiency

Sexual preference

HIV-related neuropathy

Medications and Vitamin B6

Toxic

Nitrous oxide abuse

Cobalamin deficiency

Cocaine use

Vasculitic neuropathy

Intravenous drug use

HIV-related neuropathy

Strict vegetarian diet (vegan)

Cobalamin deficiency

Table. 4. Neuropathies Associated with Organ System

Impairment
Review
of systems
Neuropathy
Endocrine

Diabetic, hypothyroid

Cancer

Paraneoplastic

Anemia

Cobalamin deficient. lead

Cardiac

Amyloid, Refsum disease, Fabry disease

Lung

Sarcoid

Kidney

Amyloid, Fabry disease

Skin

Fabry disease, Refsum disease,

connective tissue disease

Joints

Connective tissue disease

Gastrointestinal
Chronic constipation
Inflammatory bowel
disease

Amyloid
Chronic inflammatory demyelinating
polyradiculoneuropathy

Genitourinary
Incontinence, impotence

Amyloid

Hearing loss

Inherited neuropathies, Refsum disease

Visual loss

Refsum disease, Fabry disease, amyloid,

diabetic, toxic

Examining the patient

General physical Examination
Neurological Examination

General physical Examination

General physical examination

Skeletal abnormalities
Changes in skin hair and nails
Lesions of the tongue and the oral
and genital mucosa
Ocular manifestation
Organomegaly

Table. 5. Neuropathies Commonly Associated with

Painless Injuries

Diabetic polyneuropathies
Amyloid neuropathy
Hereditary sensory and autonomic
neuropathies
Tabes dorsalis
Leprous neuropathies

Table. 6. Skin, Hair, and Nail Changes Associated with

Peripheral Neuropathies

Abnormality

Neuropathy

Ulcerations

Hereditary sensory and autonomic

neuropathies, diabetic

Hypopigmentation

Sarcoid, leprous

Hyperpigmentation

POEMS*, adrenomyeloneuropathy,
cobalamin deficient, eosinophiliamyalgia syndrome

Purpura

Cryoglobulinemic neuropathy (vasculitis)

Livido reticularis

Cryoglobulinemic neuropathy (vasculitis)

Ichthyosis

Refsum disease

Angiokeratomas

Fabry disease

Vesicles and bullae

Variegate porphyria

Alopecia

Hypothyroid, systemic lupus

erythematomasus, thallium

Curly hair

Giant axonal neuropathy

Mees lines

Arsenic and thalium toxicity

White nails

POEMS*

* POEMS = polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes

(chapter
14)
Clubbing
POEMS*, paraneoplastic (lung cancer),
Chronic inflammatory demylinating polyradiculoneuropathy
neuropathy with pulmonary disease,

Table. 7. Oral and Genital Mucosal, Tongue, and Salicary

Gland Changes
Abnormality

Neuropathy

Oral and genital ulcers

Behet disease, AIDS

Hairy leukoplakia, Kaposi

syndrome, lymphoma

AIDS

Xerostomia, Xerosis or cutaneous

sicca, vaginitis sicca

Sjgrens syndrome

Salivary gland swelling

Sjgrens syndrome, sarcoid

Bald tongue

Cobalamin deficient, vitamin B

complex deficient

Enlarged, orange tonsils

Tangier disease

Table. 8. Ocular findings Associated with Peripheral

Neuropathies
Abnormality

Neuropathy

Dry eyes

Sjgrens syndrome, gelsolin amyloid,

hereditary sensory autonomic neuropathy
type 3

Scleritis

Connective tissue disease, vasculitic

Uveitis

Sarcoid, Behet disease, rheumatoid

arthritis, inflammatory bowel disease

Corneal opacities

Fabry disease, gelsolin amyloid

Vitreous opacities

Transthyretis amyloid, Refsum disease

Cataracts

Fabry disease, Refsum disease

Lens subluxation

Refsum disease

Optic atrophy

Refsum disease, Charcot-Marie-Tooth

neuropathy

Retinitis pigmentosa

Refsum disease, Charcot-Marie-Tooth

neuropathy

Neurogical Examination

Neurological Examination
Cranial nerve findings
Muscle weakness
Muscle stretch reflex changes
Sensory abnormality
Autonomic Nervous System
Abnormalities

Table. 9. Causes of Tonic Pupil

Guillain Barre syndrome
Chronic inflammatory demyelinating
polyradiculopathy
Sjgrens syndrome
Selective postganglionic parasympathetic or
more generalized autonomic neuropathy
Paraneoplastic neuropathy (may be relatively
select for postganglionic parasympathetic
fibers )
Adie syndrome

Table. 10. Causes of Facial Weakness and

Peripheral Neuropathy
Guillain Barre syndrome
Chronic inflammatory demyelinating
polyradiculopathy
Tangier disease
Gelsolin amyloid
Sarcoidosis

Table. 11. Large-Fiber Neuropathies Causing Loss of

Kinesthetic Function
Immune-Mediated Disorders
Sensory ganglionitis
Paraneoplastic sensory neuropathy
Sensory neuropathy associated with sicca syndrome
Idiopathic sensory neuropathy
Demyelinating neuropathies
Guillain Barre Syndrome (including Fisher variant)
Chronic inflammatory demyelinating polyradiculoneuropathy
IgM paraproteinemia ( > light chains )
Infectious Disorders
Tabes dorsalis
Diphtheritic polyneuropathy
HIV-associated neuropathy (rarely selctive for large fibers)
Tropical ataxic neuropathy due to HTLV-1 infection
Toxic Disorders
Vitamin B6
Cisplatin
Metronidazole
Vacor

Table. 12. Causes of Painful Neuropathies

ACQUIRED NEUROPATHIES
AIDS distal symmetric polyneuropathy
Amyloid polyneuropathy
Diabetic neuropathy (see Chapter 19)
Distal symmetric diabetic polyneuropathy
Diabetic neuropathic cachexia
Insulin neuritis
Diabetic truncal radiculoneuropathy
Diabetic lumbosacral radiculoplexus neuropathy (diabetic
amyotrophy)
Drugs* and toxic agents (alcohol, arsenic, thallium)
Guillain Barre syndrome
Idiopathic painful sensory neuropathy
Infiltrative neoplasm ( usually in roots or proximal nerve trunks )
Leprous neuropathy
Necrotizing vasculitis ( associated with any cause )
Sensory neuronopathy associated with cancer or Sjgrens disease
Sjgrens disease neuropathy (length-dependent)
Sarcoid neuropathy
Uremic polyneuropathy

Table. 12. Causes of Painful Neuropathies

INHERITED NEUROPATHIES
Amyloid polyneuropathy ( transthyretin most often painful )
Fabry neuropathy
Hereditary sensory and autonomic neuropathy ( HSAN I most
often )
Porphyric neuropathy
Tangier neuropathy
Guillain-Barre syndrome differ from most others because of its predominant motoro
component and rapid evolution; however, some cases are thought to be mainly sensory.
* Perhexilene, paclitaxel (taxol), suramin, zalcitabine, didanosine, stavudine, nitrofurantoin,
thalidomide, gold, almitrine, disulfiram, misonidazole, metronidazole

Table. 13. Neuropathies with Prominent

Autonomic Dysfunction
Diabetic polyneuropathy (particularly the small
fiber variants)
Familial and acquired amyloid polyneuropathies
Acute pandysautonomia (panautonomia)
Paraneoplastic autonomic neuropathy
Pure autonomic failure
Drug-induced neuropathies: vincristine,
perhexilene, taxol, cisplatin, amiodarone
Toxic neuropathies; Vacor, Thallium, arsenic,
mercury, organic solvents, acrylamide
Alcoholic neuropathy (occasional)
Posphyria
Guillain Barre syndrome

Evaluation of the peripheral

neuropathy
Electrodiagnostic testing
Quantitative sensory testing
Autonomic reflexs test
Autoantibody testing .
Skin biopsi

Normal
electrodiagnos
tic studies

Somatofor
m disorder
Dorsal
radiculopat
hy
Myelopath
y

Axonal
sensorimotor
neuropathy

Demyelinating
neuropathy

A few axonal
Recovered
neuropathies
Guillain-Barre
with sensory
syndrome
predominance
IgM
monoclonal
gammopathy

Pure
sensor
y fiber
loss

Friedreichs Idiopathic
ataxia
sensory
neuropathy

Rare cases of active

Guillain-Barre syndrome,
chronic inflammatory
demylinating
polyradicaloneuropathy,
and the Fisher syndrome

Fig. 1. Electrodiagnostic differentiation of ataxic

neuropathies

Carcinomato
us sensory
neuropathy
Sjgrens syndrome
with ataxic
neuropathy
Some
neurotoxic
disorders