Hereditary sensory neuropathy

Diabetic sensory neuropathy

Vacor intoxication

Xanthomatous neuropathy of primary biliary cirrhosis

Peripheral neuropathies that are often associated with pain

Cryptogenic sensory or sensorimotor neuropathy

Diabetes mellitus

Vasculitis

Guillain–Barré syndrome

Amyloidosis

Toxic (arsenic and thallium)

HIV related distal symmetrical polyneuropathy

Fabry’s disease

Autonomic symptoms

Enquire if the patient has fainting spells or orthostatic lightheadedness, sweating abnormalities or any bowel, bladder, or sexual

dysfunction.

Examples:

Acute:

Pandysautonomia

Botulism

Porphyria

Guillain-Barré syndrome

Amiodarone

Vincristine

Chronic:

Amyloid

Diabetes

Sjogren’s

HSN 1 and 3

Chagas disease

Paraneoplastic

PATTERNS OF NEUROPATHY

Pattern 1

Symmetric Proximal and Distal Weakness with Sensory Loss

Inflammatory demyelinating polyneuropathy (GBS and CIDP).

Pattern 2

Symmetric Distal Weakness with Sensory Loss

Metabolic disorders, hereditary toxins drugs.

Pattern 3

Asymmetric Distal Weakness with Sensory Loss

Multiple nerves—vasculitis

Single nerves/regions—compressive mononeuropathy and radiculopathy.

Pattern 4

Asymmetric Distal Weakness without Sensory Loss

Motor neuron disease—with upper motor neuron findings

Multifocal motor neuropathy—without upper motor neuron findings.

•

•

•

•

•

•

•

•

•

•

•

•

•

Pattern 5

Asymmetric Proximal and Distal Weakness with Sensory Loss

Polyradiculopathy or plexopathy due to diabetes mellitus

Meningeal carcinomatosis.

Pattern 6

Symmetric Sensory Loss without Weakness

Cryptogenic sensory polyneuropathy (CSPN), metabolic (diabetes and others) drugs, and toxins.

Pattern 7

Symmetric Sensory Loss and Distal Areflexia with Upper Motor Neuron Findings

B12 deficiency, HIV, and hepatic disease.

Pattern 8

A Symmetric Proprioceptive Sensory Loss without Weakness

Sensory neuronopathy (ganglionopathy).

Pattern 9

Autonomic Symptoms and Signs

Neuropathies associated with autonomic dysfunction.

Pattern 10

Syndrome of Acute Ascending Motor Paralysis

Guillain-Barré syndrome/acute idiopathic polyneuritis

Diphtheria

Porphyria

Triorthocresyl phosphate (TOCP) poisoning

Paraneoplastic

Postvaccinial.

Pattern 11

Syndrome of Subacute Sensory Motor Neuropathy

Deficiency—alcoholic beriberi, pellagra, and vitamin B12

Toxins = arsenic, lead, Hg, and Pb

Drugs = nitrofurantoin, INH, dapsone, disulfuram, and clioquinol

Uremic

DM, PAN and sarcoidosis.

•

•

•

•

•

•

•

•

•

•

Fig. 6E.13: Simplified diagram showing types of polyneuropathy.

(CIDP: chronic inflammatory demyelinating polyneuropathy; CKD: chronic kidney disease; CMT:

Charcot-Marie-Tooth; POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal

gammopathy and skin changes; DM: diabetes mellitus; HIV: human immunodeficiency virus; AIDP:

acute inflammatory demyelinating polyneuropathy; AMAN: acute motor axonal neuropathy; AMSAN:

acute motor and sensory axonal neuropathy)

General examination in neurpoathy

Purpura, livedo reticularis Vasculitis

Skin hypopigmentation Leprosy

Hyperpigmentation Osteosclerotic myeloma—POEMS

Bullous lesions Variegate porphyria

Purpura Vasculitis, cryoglobulinemia

Ichthyosis Refsum’s disease

Mee’s lines Arsenic/thallium intoxication

Alopecia Thallium poisoning

Curled hair Giant axonal neuropathy

Nerve thickening Leprosy

CMT

CIDP

Amyloidosis

Neurofibromatosis

Refsum’s disease

Dejerine-Sottas disease

Roussy Levy syndrome

Acromegaly

Idiopathic

•

•

•

•

•

•

•

•

–

–

–

–

–

–

–

–

–

–

–

–

–

–

–

–

–

•

–

–

–

–

•

–

–

–

•

•

•

•

•

•

•

•

•

•

•

(POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes;

CMT: Charcot-Marie-Tooth; CIDP: chronic inflammatory demyelinating polyneuropathy)

CRANIAL NERVE EXAMINATION IN NEUROPATHY

Anosmia—Refsum’s disease and B12 deficiency

Optic atrophy—demyelinating disease may suggest an inherited syndrome, B12 deficiency

Anisocoria and impaired pupillary light reflexes—parasympathetic damage and may be isolated, as in

Adie’s syndrome, diabetic neuropathy or acute dysautonomia as in GBS

Impaired ocular mobility suggests botulism or Miller Fisher syndrome

Facial weakness—GBS, CIDP, Lymes disease, and leprosy

Trigeminal sensory loss—Sjogren neuropathy

Lower cranial nerve palsies—Kennedy’s disease.

Medications causing neuropathies

Axonal

Vincristine

Paclitaxel

Nitrous oxide

Colchicine

Isoniazid

Hydralazine

Metronidazole

Pyridoxine

Didanosine

Lithium

Dapsone

Phenytoin

Cimetidine

Disulfiram

Chloroquine

Ethambutol

Amitriptyline

Demyelinating

Amiodarone

Chloroquine

Suramin

Gold

Neuronopathy

Thalidomide

Cisplatin

Pyridoxine

COMMON NEUROPATHIES

Guillain-Barré Syndrome (Tables 6E.6 and 6E.7)

Table 6E.6: Diagnostic criteria of GBS.

Required features

Progressive weakness in both arms and legs

Areflexia (or hyporeflexia)

Features supportive of diagnosis

Progression of symptoms over days to 4 weeks

Relative symmetry

Mild sensory signs or symptoms

Cranial nerve involvement, especially bilateral facial weakness

Recovery beginning 2–4 weeks after progression ceases

Autonomic dysfunction

Absence of fever at onset

Typical CSF (albuminocytologic dissociation)

EMG/nerve conduction studies (characteristic signs of a demyelinating process in the peripheral nerves)

Features casting doubt on the diagnosis

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

•

■

■

■

■

■

■

■

■

■

■

■

■

■

Asymmetrical weakness

Persistent bladder and bowel dysfunction

Bladder or bowel dysfunction at onset

>50 mononuclear leukocytes/mm3 or presence of polymorphonuclear leukocytes in CSF

Distinct sensory level

Features that rule out the diagnosis

Hexacarbon abuse

Abnormal porphyrin metabolism

Recent diphtheria infection

Lead intoxication

Other similar conditions: Poliomyelitis, botulism, hysterical paralysis, toxic neuropathy

(CSF: cerebrospinal fluid; EMG: electromyogram)

Table 6E.7: Variants of GB syndrome.

Common variants Less common variants

Acute motor and sensory axonal neuropathy (AMSAN)

Acute motor axonal neuropathy (AMAN)

Miller-Fisher variant

Pure motor variants

Pure sensory variants

Pure dysautonomia variant

Pharyngeal-cervical-brachial variant

Paraparetic variant (Ropper variant)

Acral paresthesias with diminished reflexes in either arms or legs

Facial diplegia or abducens palsies with distal paresthesias

Isolated postinfectious ophthalmoplegia

Bilateral foot drop with upper limb paresthesias

Acute ataxia without ophthalmoplegia

Bickerstaff’s brainstem encephalitis (BBE)

Diabetes Mellitus (Box 6E.1)

Box 6E.1: Classification of diabetic neuropathy.

Polyneuropathy

Symmetrical, mainly sensory and distal

Asymmetrical, mainly motor and proximal (including amyotrophy)

Mononeuropathy and mononeuritis multiplex

Cranial nerve lesions

Isolated peripheral nerve lesions

Autonomic (visceral) neuropathy

Cardiovascular

Gastrointestinal

Genitourinary

Sudomotor

Vasomotor

Pupillary

Polyradiculopathies

Diabetic amyotrophy (lumbar polyradiculopathy)

Thoracic polyradiculopathy

Diabetic neuropathic cachexia

Treatment-induced neuropathy of diabetes

•

–

–

•

•

–

–

•

•

•

•

•

•

•

•

•

•

•

•

•

–

–

–

–

•

–

–

•

–