Peripheral neuropathies
Hereditary neuropathies due to hypersensitivity to pressure.
Epidemiology The true prevalence of hereditary pressure hypersensitivity neuropathy (HNPP) is unknown, as the disease is underdiagnosed, but is estimated to be between 1/50,000 and 1/20,000. Clinical description The disease usually occurs between the second
Pain and breast cancer.
Pain in breast cancer depends on the disease itself and its local and metastatic course, but also on the treatments implemented. Cancer pain. Pain from excess nociception. Linked to its more or less inflammatory character,
Tomacular neuropathy
Or hereditary neuropathy with hypersensitivity to pressure (HNPP) Etiology The disease is caused by a mutation in the PMP22 (17p12) gene and other genes. PMP22 encodes peripheral myelin protein 22 (PMP22) which is expressed predominantly
Tarsal tunnel syndrome
Pathophysiology. Tarsal tunnel syndrome is a tunnel syndrome characterized by compression of the posterior tibial nerve and/or its distal terminal branches by the internal annular ligament. The posterior tibial nerve gives, in the retro-malleolar region,
Howship-Romberg syndrome
Anatomical reminder Corresponds to damage to the obturator nerve Born from roots L2-L3-L4 It first passes behind the psoas major, emerges from it via the medial edge and descends vertically into the pelvis from which
Meralgia paresthetica syndrome
Anatomical reminder Originates from the ventral branches of the 2nd and 3rd lumbar root It passes behind the psoas muscle (in red), exits the lateral part of the psoas towards its middle and crosses ventrally
Cervico-thoracic outlet syndrome
Anatomical reminder Cervico-thoracic parade syndrome corresponds to the compression of the brachial plexus (all of the upper limb nerves), the subclavian artery and/or the subclavian vein in the passage formed by the inter-scalènic parade and
Fibular nerve compression syndrome
Anatomical reminder The common peroneal nerve is formed by bifurcation of the sciatic nerve at the apex of the popliteal fossa. It runs along the medial edge of the biceps femoris muscle then its tendon.
Anterior interosseous nerve syndrome
Anatomical reminder The interosseous nerve is the main branch of the median nerve in the forearm. It appears between 5 and 8 cm below the elbow at the level of the pronator teres muscle. He
Idiopathic radial palsy
Anatomical reminder From the posterior and lower part of the axillary fossa, it runs downwards and outwards into the posterior brachial region along the posterior surface of the humerus in the radial groove (in contact
Serratus Major (Serratus) Nerve Syndrome
Anatomical reminder The serratus anterior muscle is innervated by the long thoracic nerve. This nerve arises from the 5th, 6th, and 7th spinal nerves at their junction forming the superior primary trunk of the brachial
Suprascapular nerve syndrome
Anatomical reminder The suprascapular nerve detaches from the upper primary trunk of the brachial plexus. It contains C5 C6 fibers. It passes through the coracoid notch (1) under the coracoid ligament (in green) then at
Guyon compartment syndrome
Anatomical reminder There is no synovium in Guyon’s canal where only the artery and the ulnar nerve pass. Consequently, any compression of the ulnar nerve in this compartment is of extrinsic origin. At the wrist,
Ulnar canal syndrome at the elbow
Anatomical reminder 1- Radial nerve 2- vessels 3-Median nerve 4- Brachialis muscle 5- Humerus 6-Ulnar nerve 7- Olecranon The ulnar nerve travels in the epitrochleo-olecranon groove on the inner side of the elbow. It relates
Carpal tunnel syndrome
Anatomical reminder Carpal tunnel syndrome is linked to compression of the median nerve in the wrist. The carpal tunnel is a narrow tunnel formed posteriorly by the carpal bones and anteriorly by the anterior annular
Canal syndromes
Root canal syndromes: definition Nerve compression syndromes, called “ductal syndromes” (entrapment neuropathy in the English) are defined as the clinical translation of a conflict between a peripheral nerve trunk and a particular anatomical region of
Cicatricial neuropathy
A new concept: Cicatricial neuropathy. The most misunderstood and yet the most frequent of the neuropathies responsible for neuropathic pain. Indeed, a recent study carried out by a Parisian pain center finds an incidence between
Pudendal nerve neuralgia ABONNE LIBRE
Pathophysiology of neuralgia of the pudendal nerve Classic pudendal nerve neuralgia is a tunnel syndrome (like carpal tunnel syndrome). There are two areas of possible conflict in the pelvic area: at the level of the
Algodystrophy and scarring neuropathy: are they the same thing?
Discussion based on an example Clinical case : Mrs X, 65 years old, is referred to a pain consultation for the management of algodystrophy diagnosed 4 months ago which appeared 3 months after the insertion
Hyperesthetic depigmented spot
In certain painful pathologies, the origin of the pain can be identified on depigmented areas of the skin, round or lenticular in shape, sometimes grouped together. These depigmented areas have the particularity of being hyperesthetic
Peripheral neuropathies ABONNE LIBRE
Definition. The peripheral nervous system is composed of: – Spinal nerve roots, – the posterior spinal ganglia, – the peripheral nerves up to their terminal ramifications, – the autonomic nervous system, – the cranial nerves
Functional neuropathy
Seen when there is an area with damage to small localized fibers, particularly in scars or after-effects of hematoma. These neuropathies can be extensive even though the responsible lesion is small. The different types of
Dysautonomic neuropathy ABONNE
It corresponds to damage to the autonomic nervous system (sympathetic and parasympathetic). This attack is responsible for different manifestations: Sudomotor manifestations: Anhidrosis (absence of sweat secretion). Abnormal vasomotor response to temperature changes. Lacrimal dryness (dry
Diffuse Scarring Hyperesthesia Syndrome (DCHS)
This new clinical entity is found in some patients with chronic pain. Its current incidence is not known. It consists of a scarring neuropathy affecting all of the scars found in a patient. This entity
Ocular signs associated with peripheral neuropathy ABONNE
Corneal opacities: Found in Fabry disease, amyloid neuropathy and Tangier disease. Cataract: In Fabry disease and Refsum disease. Optic atrophy: Found in Refsum disease and Charcot-Marie-Tooth disease. Retinitis pigmentosa: Found in Refsum disease and Charcot-Marie-Tooth
Mucocutaneous signs associated with certain neuropathies ABONNE
Ulcers and poor perforation: Found in diabetic neuropathy, hereditary sensory and dysautonomic neuropathies, and in neuropathy linked to leprosy. Purpura: Found in cryoglobulinemia and vasculitis. Mees lines: (definition) Seen in arsenic and thallium poisoning. Bullous
Toxic neuropathies ABONNE
Below is a list of toxic agents used in industry and potentially toxic to peripheral nerves. This list is not exhaustive. – Acrylamide (flocculating agent) causes sensory damage to large nerves with sensory ataxia. –
Dejerine-Sottas syndrome
Hereditary sensorimotor disease type III or Dejerine-Sottas disease Onset in childhood, most often before the age of three. Causes muscular atrophy of the extremities which can spread towards the roots with sometimes children having to
Familial amyloid polyneuropathy
Etiology Linked to a genetic defect on chromosome 18, with alteration of transthyretin (protein). The disease is caused by a methionine for valine substitution at residue 30 of the mature TTR protein, which is encoded
Hereditary sensory neuropathies (HSN I and II)
Pathophysiology and clinical These two forms cause damage to spinal ganglion neurons. They cause sensitivity disorders without motor impairment. The pathology begins in childhood or before the age of 30. Form 1 is autosomal dominant
Dysautonomic hereditary sensory neuropathy
Dysautonomic hereditary sensory neuropathy, Type 1. HSAN1 Autosomal dominant disease whose genetic anomaly is known to differentiate 4 subtypes of this disease. Symptoms peak between 10 and 40 years of age. Patients have a loss
Tangier disease
Definition Very rare genetic neurometabolic disease characterized, biochemically, by an almost total absence of high density lipoproteins (HDL) in the plasma and, clinically, by enlargement of the liver, spleen, lymph nodes and tonsils, as well
Fabry disease
Etiology Linked to alpha-galactosidase A enzyme deficiency. X-linked with pathogenic variants of the GLA gene (Xq21.3-q22). This enzyme deficiency leads to an accumulation of globotriaosylceramide (Gb3) and its deacylated derivative (lyso-Gb3) in the lysosomes, which
Refsum’s disease
Autosomal recessive transmission disease. Linked to a deficiency in an enzyme allowing the degradation of phytanic acid and therefore linked to an accumulation of the latter. Etiology Refsum’s disease is caused by mutations in the
Peroneal atrophy or Charcot-Marie-Tooth polyneuropathy (CMT)
This neuropathy, also called peroneal muscular atrophy, is an inherited transmission neuropathy. Mode of transmission, phenotype and severity are variable. there are 3 forms: The most frequent phenotype is a demyelinating neuropathy of autosomal dominant
Genetically determined neuropathies
Etiologies They are many : – Peroneal atrophy or Charcot-Marie-Tooth polyneuropathy (CMT). – Hereditary amyloid neuropathy. – Dejerine-Sottas disease. – Refsum disease. – Hereditary sensory neuropathies (HSN I and II). – Hereditary dysautonomic sensory neuropathies
Subacute neuropathies ABONNE
Etiology Installation in several months. In general : – Metabolic neuropathies, – neuropathies of deficiency origin, – toxic neuropathies, – alcoholic neuropathies, – dysproteinemia neuropathies (myeloma, plasmacytoma, cryoglobulemia, monoclonal gammopathy), – lymphoma neuropathies, – acquired
Chronic forms of neuropathies ABONNE
Etiologies: Established over years, sometimes since childhood. Hereditary polyneuropathies. Chronic polyradiculoneuritis. Polyradiculopathies associated with monoclonal IgM gammopathy.
Acute forms of neuropathies. ABONNE
Installation in a few hours: Mainly vasculitis (polyarteritis nodosa) The picture is more of a multineuritis with multiple radicular or truncal involvement. Installation in a few days: – Guillain-Barrè syndrome and its variants. – Neuropathy
Relapsed forms of neuropathies ABONNE
Etiology Hereditary neuropathies due to hypersensitivity to pressure. Chronic relapsing polyradiculoneuritis.
Neuropathies and hypothyroidism ABONNE
Two types of neurological damage: mononeuropathies and polyneuropathies. Be careful, these forms can be associated with hypothyroidism myositis responsible for muscle pain and cramps. Mononeuropathy: The most common is damage to the median nerve at
Neuropathies and hyperthyroidism ABONNE
Description This complication is rare and presents in the form of sensory-motor impairment with predominance of sensitivity disorders. This damage is reversible with treatment of hyperthyroidism (minimum 6 months). Not to be confused with the
Neuropathies and hyperlipidemia ABONNE
Rare, it causes sensory impairment distally with a sensation of paresthesias at the tips of the feet. Treatment of hyperlipidemia results in recovery from neuropathy within a few months.
Neuropathies and Acromegaly ABONNE
Linked to excessive secretion of growth hormone. Symmetrical nerve damage, predominantly in the lower limbs with paresthesias of the feet or even legs. We can find an enlargement of the nerves in onion bulbs. If
Neuropathies that are painful from the outset ABONNE
Different origins are possible: Idiopathic: Distal symmetrical small fiber polyneuropathy. Inflammatory: Vasculitis and Perineuritis. Hereditary: Fabry disease, Hereditary dysautonomic sensory neuropathy type V, Tangier disease, Hereditary amyloidosis. Metabolic: Damage linked to diabetes. Toxic: Thallium, Arsenic.
Diabetic neuropathies
Diabetic neuropathies. The frequency of diabetic neuropathy is more than 50% in patients with diabetes for more than 25 years. There are an estimated 100,000,000 patients with diabetic neuropathy worldwide. At the time of diagnosis
Lyme disease
Causal agent: Due to a spirochete bacteria of the genus Borrelia. Three species of Borrelia are known to cause Lyme disease: Borrelia Burgdorferi, Borrelia Garinii, Borrelia Afzelii. Epidemiology : Lyme disease is transmitted by tick
Chagas disease or American trypanosomiasis
Described in 1909 by Carlos Chagas. Endemic tropical parasitic anthropozoonosis in 21 countries in Central and South America. 18 million people are estimated to be infected in Latin America. Between 2 to 3 million individuals
Leptospiroses
Causal agent: Leptospiroses are zoonoses (diseases transmitted by animals) caused by bacteria of the leptospira genus. There are more than 130 serotypes grouped into 23 serogroups The most common in France is Leptospira ictero-haemorragiae. Epidemiology
Brucellosis
Brucellosis or Malta fever, melitococcal disease. Causal agent: Three germs from the same family: Brucella melitensis, Brucella abortus bovis, Brucella abortus suis. Asporulate aerobic gram-negative coccobacillus. In France the number of human cases is decreasing,
Neuropathies of infectious origin
Neuropathies of infectious origin Three types of infectious agent can be directly or indirectly responsible for neuropathy: viruses, bacteria and parasites. Neuropathies of viral origin: Herpes virus infection Varicella zoster virus infection Cytomegalovirus (CMV) infection
Multiple deficiency syndrome.
Painful polyneuropathy with ocular involvement and deafness. Seen in disadvantaged populations, especially in times of war. Linked to multiple deficiencies. Are associated with it: A painful distal polyneuropathy, Fatigue, irritability, sleep disturbances in the context
Alcoholic polyneuropathy. ABONNE
It is linked to direct toxicity of alcohol and its metabolites on the neurological tissue linked to vitamin malabsorption (competition between vit B1 and alcohol in the digestive tract, gastropancreatic disorders causing malabsorption of vitamins)
Vitamin E deficiency
Linked to pathologies of biliary excretion (biliary cirrhosis, fibrosis of the bile duct, Crohn’s disease, blind loop syndrome) or to a familial deficiency of the alpha-tocopherol receptor. He gives : Peripheral motor and sensory neuropathy
Thiamine deficiency (Vitamin B1) or Beriberi
The manifestations of thiamine deficiency are: Polyneuropathy (affecting several nerves) distal and symmetrical with cramps, burns, dysesthesia, balance disorders (ataxia). Global cardiac involvement with high output heart failure, pulmonary and peripheral edema. Encephalopathy (Gayet-Wernicke). These
Pyridoxine deficiency (Vitamin B6).
This deficiency is rare, vitamin B6 being very widespread in the diet. Note that 3 drugs antagonize it: isoniazid, penicillamine and hydralazine. Clinical signs : Seborrheic skin lesions Glossostomatitis Distal sensitive polyneuropathy with burning of
Cobalamin deficiency (Vitamin B12).
Etiology. The main cause of this deficiency is the intrinsic factor deficiency of Biermer’s anemia. Digestive pathologies with malabsorptions are also responsible: Crohn’s disease with involvement of the terminal ileum, Wipple’s disease, a blind loop
Pantothenic acid deficiency
Extremely widespread in food. Pantothenic acid deficiency is very rare. It causes : Headaches, Sleep disorders, Distal sensory polyneuropathy.
Folic acid deficiency
There is an interdependence of folic acid and vitamin B12. Gives the same table as Vitamin B12 deficiency: Peripheral nerve damage Combined spinal sclerosis, Encephalopathy or psychiatric disorders, Ataxia, Optic nerve atrophy.
Neurological impairment of celiac disease
This disease causes chronic malabsorption linked to gluten intolerance. She can give: Peripheral sensory neuropathy Cerebellar or spinal ataxia (combined spinal sclerosis) Myositis.
Alcoholic and nutritional neuropathies ABONNE
Linked to a deficiency in essential nutrients. These neuropathies are frequent in developing countries, deficiencies being endemic. They mainly affect vitamin B deficiencies: B1 (beriberi), B6, B9 In developed countries, they are rather linked to
Peripheral neuropathy: drug cause
The main drugs that can cause peripheral neuropathy are: – Periwinkle alkaloids: Vinblastine, Vincristine, Vindesine, Vinorelbine. -Almitrine – Amiodarone – Nucleoside analogues (didanosine, zalcitabine, stavudine, etc.) – Chloroquine – Cimetidine – Cisplatin – Dapsone –
Neuropathy and kidney failure ABONNE
Peripheral neurological involvement is related to renal failure, not dialysis. Uremic polyneuropathy: Its occurrence is linked to the severity of the kidney damage and its duration. Diabetes is a significant cofactor (see diabetic neuropathy). Polyneuropathy
Small fiber neuropathy
Due to damage to the small A delta and C fibers Definition : Small fiber neuropathies are neuropathies affecting small somatic fibers (sensation) and/or fibers of the autonomic nervous system. As a result, autonomic functions,
Peripheral nerves
Nerves are formed by the axons of nerve cells (the axon is an extension of the nerve cell). These axons are or are not surrounded by myelin sheath from other cells: Schawnn cells. Anatomy: A
Hypoglycemic neuropathy ABONNE
Etiology Two pathologies are likely to cause neuropathy (rare forms): Insulinoma (benign or malignant tumor secreting insulin), Insulin treatment responsible for repeated hypoglycaemia. Clinical Description It is a predominantly distal motor and sensory neuropathy (feet
Evolution of polyradiculoneuritis sensory impairment ABONNE
Polyradiculoneuritis. Possible development The topographic evolution of this pathology is dependent on the length of the nerves. Its evolution can be schematized as follows (in red):
Primary erythermalgia
Description : Primary or idiopathic erythermalgia (PE) is characterized by intermittent episodes of redness, warmth, and burning of the extremities. These episodes are caused by exercise, prolonged standing or exposure to heat. Patients tend to wear