Elsevier

The Lancet

Volume 363, Issue 9427, 26 June 2004, Pages 2151-2161
The Lancet

Seminar
Peripheral neuropathy

https://doi.org/10.1016/S0140-6736(04)16508-2Get rights and content

Section snippets

Epidemiology

The overall prevalence of the condition is about 2400 (2·4%) per 100 000 population, but in people older than 55 years, the prevalence rises to about 8000 (8%) per 100 000.1 Since these figures do not include traumatic peripheral nerve injuries, the total burden of peripheral neuropathy on society is even greater. Although traumatic nerve injuries are important, they are not covered in this review since their diagnosis and management are highly specialised. Comprehensive coverage of peripheral

Diagnosis

The clinical manifestations of peripheral neuropathy vary widely. Presenting features encompass varying combinations of altered sensation, pain, muscle weakness, or atrophy, and autonomic symptoms. Accurate diagnosis rests on the skill with which clinical symptoms, signs, and electrodiagnostic study findings can be woven together. In accordance with this diagnostic approach, patients whose history and clinical examination suggest the presence of a peripheral neuropathy should have confirmation

Mononeuropathy

The term mononeuropathy implies a focal lesion of a single peripheral nerve. The usual causes are trauma, focal compression, and entrapment. The most common mononeuropathy is carpal tunnel syndrome caused by entrapment of the median nerve in the carpal tunnel.16, 17, 18, 19 Ulnar neuropathy due to compression of the nerve at or near the elbow is the second most common mononeuropathy.20, 21 Electrodiagnostic studies are indispensable in the accurate diagnosis of mononeuropathies. They serve to

Mononeuropathy multiplex

Mononeuropathy multiplex describes the involvement of multiple separate noncontiguous peripheral nerves either simultaneously or serially. The pattern of nerve involvement is random, multifocal, and frequently evolves quickly. In some cases, the neuropathy might progress to a point at which individual nerve lesions summate, resulting in confluent and symmetric deficits that can mimic a distal symmetrical polyneuropathy. Attention to the pattern of early symptoms is important in correct

Polyneuropathy

The most common variety of polyneuropathy is distal symmetrical polyneuropathy.15, 43 In this setting, nerve fibres are believed to be affected in a length–dependent way; length in this context meaning distance from the parent nerve cell body (either the dorsal root ganglion sensory neuron or the anterior horn motor neuron). Thus, toes and soles of the feet are affected first. This pattern of neuropathy is associated with several acquired systemic diseases, metabolic disorders, and exogenous

Subclassification of polyneuropathies

Separating polyneuropathies into acute and chronic forms is very helpful for refining the diagnosis and treatment.

Chronic demyelinating polyneuropathy

This is the easiest category to define since the causes are limited (panel 1). Demyelinating polyneuropathies are either genetically determined or acquired. Electro-diagnostic features are helpful in making this distinction because uniform symmetrical slowing of nerve conduction usually indicates a genetically determined neuropathy, whereas multifocal slowing and conduction block are indicative of acquired demyelinating neuropathies.

Most genetically determined demyelinating polyneuropathies are

Chronic axonal polyneuropathy

This is the most common variety of polyneuropathy and has many possible causes. The commonest cause is diabetes mellitus, which should be foremost in the differential diagnosis. However, a range of systemic diseases and metabolic disorders such as nutritional deficiencies, chronic renal failure, and malignant diseases as well as exogenous intoxications from medications, alcohol abuse, or chemical agents can result in this pattern of neuropathy (Table 1, Table 2).

Laboratory investigations of peripheral neuropathy

The cause of most peripheral neuropathies is evident when the information obtained from the medical history, neurological examination, and electrodiagnostic studies is combined with simple screening laboratory tests (panel 2). All patients with a peripheral neuropathy should have a complete blood count, erythrocyte sedimentation rate or C-reactive protein, comprehensive metabolic panel (fasting blood glucose, renal function, liver function), thyroid function tests, urinalysis, serum B-12 and

Role of nerve biopsy in evaluation of neuropathies

Nerve biopsy is most useful in documentation of inflammatory disorders such as vasculitis, sarcoidosis, CIDP, infectious diseases such as leprosy, or infiltrative disorders such as amyloidosis or tumour.24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34 The procedure is best reserved for patients in whom the diagnosis cannot be obtained by other means. Nerve biopsies should always be processed in experienced laboratories with the capability to do teased fibre preparations, semi-thin plastic sections,

Treatment

Treatment of peripheral neuropathy is divided into those that are specific for the subtype of neuropathy, and those that are useful for neuropathies in general. Medical causes such as diabetes mellitus, renal insufficiency, hypothyroidism, vitamin B-12 deficiency, or systemic vasculitis need specific and active treatment. Immune-mediated neuropathies such as Guillain-Barré syndrome or CIDP respond to specific treatments. If treated appropriately, many patients with dysimmune neuropathies have a

Search strategy and selection criteria

We searched the Cochrane Library (January, 1990 to October, 2003), OVID MEDLINE (January, 1990 to October, 2003), OVID Excerpta Medica (EMBASE: January, 1990 to October, 2003), and OVID Current Contents (January, 2002 to October, 2003). The search included reports of research in human beings only, and all languages. The search terms selected were “peripheral neuropathy”, “polyneuropathy”, and “guideline”. We then limited the search using the terms “epidemiology”, “diagnosis”, “nerve conduction

First page preview

First page preview
Click to open first page preview

References (97)

  • DG Kline et al.

    Nerve injuries

    (1995)
  • KMV Narayan et al.

    Lifetime risk for diabetes mellitus in the United States

    JAMA

    (2003)
  • RAC Hughes

    Peripheral neuropathy

    BMJ

    (2002)
  • PJ Dyck et al.

    Vibratory and cooling detection thresholds compared with other tests in diagnosing and staging diabetic neuropathy

    Diabetes Care

    (1987)
  • PJ Dyck et al.

    Longitudinal assessment of diabetic polyneuropathy using a composite score in the Rochester Diabetic Neuropathy Study cohort

    Neurology

    (1997)
  • EL Feldman et al.

    A practical two-step quantitative clinical and electrophysiological assessment for the diagnosis and staging of diabetic neuropathy

    Diabetes Care

    (1994)
  • GentileS et al.

    Simplified criteria for diabetic distal polyneuropathy: preliminary data of a multicentre study in the Campania region

    Acta Diabetol

    (1995)
  • LL Teunnissen et al.

    Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy. A clinical and electrophysiological study

    Brain

    (1997)
  • PJ Dyck et al.

    Clinical and neuropathological criteria for the diagnosis and staging of diabetic polyneuropathy

    Brain

    (1985)
  • PJ Dyck et al.

    The Rochester Diabetic Neuropathy Study: reassessment of tests and criteria for diagnosis and staged severity

    Neurology

    (1992)
  • JW Albers

    Clinical neurophysiology of generalized polyneuropathy

    J Clin Neurophysiol

    (1993)
  • Diabetic polyneuropathy in controlled clinical trials: consensus report of the Peripheral Nerve Society

    Ann Neurol

    (1995)
  • Proceedings of a consensus development conference on standardized measures in diabetic neuropathy

    Neurology

    (1992)
  • NR Rosenberg et al.

    Diagnostic investigation of patients with chronic polyneuropathy: evaluation of a clinical guideline

    J Neurol Neurosurg Psychiatry

    (2001)
  • D Rempel et al.

    Consensus criteria for the classification of carpal tunnel syndrome in epidemiologic studies

    Am J Public Health

    (1998)
  • AAM Gerritsen et al.

    Splinting vs surgery in the treatment of carpal tunnel syndrome

    JAMA

    (2002)
  • N Katz et al.

    Carpel tunnel syndrome

    N Engl J Med

    (2002)
  • JD England

    Entrapment neuropathies

    Curr Opin Neurol

    (1999)
  • W Campbell et al.

    The electrodiagnostic evaluation of patients with ulnar neuropathy at the elbow: literature review of the usefulness of nerve conduction studies and needle electromyography

    Muscle Nerve

    (1999)
  • JD Stewart

    Focal peripheral neuropathies

    (2000)
  • PPA Lenssen et al.

    Hereditary neuropathy with liability to pressure palsies: phenotypic differences between patients with the common deletion and a PMP22 frame shift mutation

    Brain

    (1998)
  • D Pareyson et al.

    Detection of hereditary neuropathy with liability to pressure palsies among patients with acute painless mononeuropathy or plexopathy

    Muscle Nerve

    (1998)
  • L Davies et al.

    Vasculitis confined to peripheral nerves

    Brain

    (1996)
  • PJB Dyck et al.

    Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy

    Neurology

    (2000)
  • WMJ Bosboom et al.

    Diagnostic value of sural nerve demyelination in chronic inflammatory demyelinating polyneuropathy

    Brain

    (2001)
  • M Deprez et al.

    Nerve biopsy: indications and contribution of the diagnosis of peripheral neuropathy

    Acta Neurol Belg

    (2000)
  • P Flachenecker et al.

    Clinical outcome of sural nerve biopsy: a retrospective study

    J Neurol

    (1999)
  • CM Gabriel et al.

    Prospective study of the usefulness of sural nerve biopsy

    J Neurol Neurosurg Psychiatry

    (2000)
  • G Said

    Indications and value of nerve biopsy

    Muscle Nerve

    (1999)
  • G Said

    Indications and usefulness of nerve biopsy

    Arch Neurol

    (2002)
  • AK Asbury et al.

    Peripheral nerve disorders

    (1995)
  • JM Vallat et al.

    Nerve biopsy

    Curr Opin Neurol

    (1995)
  • RJ Barohn et al.

    Chronic inflammatory denyelinating polyneuropathy: clinical characteristics, course, and recommendations for diagnostic criteria

    Arch Neurol

    (1989)
  • C Bouchard et al.

    Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy

    Neurology

    (1999)
  • M Busby et al.

    Chronic dysimmune neuropathy: a classification based upon the clinical features of 102 patients

    J Neurol

    (2003)
  • DS Saperstein et al.

    Clinical spectrum of chronic acquired demyelinating polyneuropathies

    Muscle Nerve

    (2001)
  • GJ Parry et al.

    Pure motor neuropathy with multifocal conduction block masquerading as motor neuron disease

    Muscle Nerve

    (1988)
  • SR Beydoun

    Multifocal motor neuropathy with conduction block misdiagnosed as multiple entrapment neuropathies

    Muscle Nerve

    (1998)
  • Cited by (0)

    View full text