Abstract
Peripheral neuropathies are heritable or acquired.
-
A.
Heritable defects, among others:
-
1.
Chronic hereditary motor-sensory neuropathies (HMSN alias Charcot-Marie-Tooth), hereditary motor-sensory neuropathies (HMSN), hereditary sensory and autonomic neuropathies (HSAN), hereditary neuralgic amyotrophy (HNA)
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2.
Relapsing/progressive motor-sensory polyneuropathies (i.e., Refsum disease)
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3.
Chronic, progressive, motor-sensory polyneuropathies with early small-fiber involvement (e.g., familial amyloid polyneuropathies)
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4.
Painful neuropathies (e.g., Fabry disease)
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5.
Acute generalized polyneuropathies (porphyrias)
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6.
Chronic sensory neuronopathies associated with ataxias
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7.
Chronic sensory (and motor) neuropathies due to mitochondrial disorders
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8.
Recurrent focal neuropathies/plexopathies [hereditary neuropathy with liability to pressure palsies (HNPP); hereditary neuralgic amyotrophy (HNA)]
Outcome and disability – Most patients with CMT1A and CMT1X do not require ambulation aids. FAP, if untreated, has a relentless fatal course. HNPP has normal life expectancy and good quality of life in most patients.
Acute intermittent porphyria: the long-term prognosis depends on successful prevention of attacks.
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1.
-
B.
Acquired forms, among others:
Immunomediated neuropathies: acute inflammatory polyradiculoneuropathies (AIDP), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN)
Outcome and disability – AIDP: 5 % of patients die. CIDP: age <45 years predicts a better outcome; axonal loss forecasts poorer prognosis. MMN: very uncommon spontaneous remissions. Anti-MAG: 25 % of patients disabled after 10 years. POEMS: chronic and invalidating; 50 % of patients are bedridden; mean survival ranges 12–165 months.
Non-systemic vasculitic neuropathy (NSVN): usually not fatal; NSVN may become systemic in 10 % of patients.
Diabetic Neuropathies
Distal symmetric polyneuropathy (DSP): slowly progressive; can stabilize or improve with tight control of diabetes. Autonomic neuropathy: increases the risk of adverse cardiovascular events. Radiculoplexopathy: monophasic, self-limiting evolution.
Carpal tunnel syndrome (CTS): pain tends to decrease with time. One-third of patients have spontaneous improvement.
Bell palsy: healing occurs within 6 months in 80 % of patients.
Peripheral nerve injury (PNI) – Neurapraxic lesions usually recover within a few months. Surgery: the sooner the nerve is repaired the better the functional recovery will be.
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Fabrizi, G.M., Zanette, G. (2015). Disorders of Peripheral Nerves. In: Sghirlanzoni, A., Lauria, G., Chiapparini, L. (eds) Prognosis of Neurological Diseases. Springer, Milano. https://doi.org/10.1007/978-88-470-5755-5_35
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