Polyneuropathy is a generalized disease of the peripheral nerves with the distal nerves usually most severely affected. There are various causes including toxic, metabolic (chronic liver disease), endocrine (diabetes mellitus, hypoglycemia, hypothyroidism etc.) immune-mediated, carcinomatous, idiopathic and inherited. It most commonly presents as symmetric numbness, paraesthesias and dysaesthesias.
The signs and symptoms of polyneuropathy include pain, accompanied by numbness, hypersensitivity and weakness in the affected arms and legs. In the majority of cases, there is development of tingling sensation in the affected area .
Sensory neuropathy occurs along with ataxia and hereditary polyneuropathy favors the development of high arches, hammer toes and scoliosis. In conditions when the autonomic nervous system is affected, there is onset of constipation accompanied by orthostatic hypotension and loss of control over bowel movements. Affected individuals also complain of skin turning pale, along with dryness and significant reduction in sweat production.
Entire Body System
We herein report this first case of amyloid polyneuropathy and myocardial amyloidosis after DLT from hereditary ATTR amyloidosis with a transthyretin Ser50Arg mutation and discuss similar cases of other mutations. [ncbi.nlm.nih.gov]
[…] in the extremities. familial amyloid polyneuropathy autosomal dominant amyloid polyneuropathy occurring in hereditary amyloidosis; subtypes include Finnish type, Indiana type, Iowa type, and Portuguese type. [medical-dictionary.thefreedictionary.com]
New drugs for TTR amyloidosis A number of new drugs for TTR amyloidosis are in various stages of development, around the world, offering hope for the near future to patients with TTR amyloidosis. [ttrstudy.com]
amyloidosis E85.1 Neuropathic heredofamilial amyloidosis E85.2 Heredofamilial amyloidosis, unspecified E85.3 Secondary systemic amyloidosis E85.4 Organ-limited amyloidosis E85.8 Other amyloidosis E85.81 Light chain (AL) amyloidosis E85.82 Wild-type transthyretin-related [icd10data.com]
- Muscular Atrophy
Despite the reduction of oral cyclosporine dose along with medical therapy for the cytomegalovirus infection and diabetes mellitus, his muscular weakness and atrophy did not improve. [jmedicalcasereports.biomedcentral.com]
HMSN I: Charcot-Marie-Tooth disease HMSN II: Neuronal peroneal muscular atrophy HMSN III: Dejerine-Sottas disease HMSN IV: Refsum disease HMSN V: Hereditary spastic paraplegia HMSN VI: HMSN with optic atrophy HMSN VII: HMSN with retinitis pigmentosa Charcot-Marie-Tooth [lecturio.com]
Inflammatory Neuropathies Demyelinating Acute - Guillain Barre Syndrome (GBS) CIDP - often associated with monoclonal gammopathies Hereditary Multifocal Motor Neuropathy Weakness and muscular atrophy & Responds to IVIg Infection Varicella Zoster ; Poliomyelitis [enotes.tripod.com]
In general, neuropathies with extensive axonal loss, denervation and atrophy have a poor prognosis for full recovery. [now.aapmr.org]
They include Immune-mediated disorders (eg, Guillain-Barré syndrome, multifocal motor neuropathy with conduction block) Lead toxicity Dapsone use Porphyria Spinal muscular atrophy (a motor neuron disorder that mimics motor polyneuropathy) Others affect [msdmanuals.com]
- Muscle Cramp
cramps, predominantly at night Image : “The foot of a person with Charcot-Marie-Tooth. [lecturio.com]
If you have become severely impaired, you may need physical therapy to help retain strength and avoid muscle cramping and spasms. Surgical treatment may be recommended for people with nerve damage from injury or nerve compression. [webmd.com]
[…] weakness but may also produce uncontrolled muscle twitches (fasciculations), painful muscle cramps, muscle atrophy with decreased reflexes similar to patients who are diagnosed with ALS, Motor Neuron Disease, Alzheimer and Parkinson’s disease. [stemcellthailand.org]
[…] loss Paresis, reduced muscle tone, muscle atrophy, reduced reflexes Muscle cramps, fasciculations Muscle cramps on strength testing, fasciculations Autonomic Dry skin Hypo- and anhidrosis Body hair loss, skin changes Trophic disorders Sensation of glare [ncbi.nlm.nih.gov]
Needle electromyography (EMG) should include tibialis anterior, medial gastrocnemius, lumbar paraspinal muscles, possibly foot intrinsic muscles, and first dorsal interosseous (hand), followed by at least one contralateral muscle if any of the aforementioned [now.aapmr.org]
- Guillain-Barré Syndrome
Differential diagnosis [ 2 ] Predominantly motor peripheral neuropathies include Guillain-Barré syndrome, Charcot-Marie-Tooth syndrome, porphyria, lead poisoning and diphtheria. [patient.info]
In some patients, presentation can be acute and closely mimic Guillain-Barré syndrome (GBS) 2,4. CIPD is an acquired demyelinating disease of the peripheral nervous system. [radiopaedia.org]
CIDP is similar to Guillain-Barré syndrome, which appears suddenly and generally improves spontaneously. Although CIDP was once called “chronic Guillain-Barré syndrome,” it is now regarded as a related, but distinct condition. [foundationforpn.org]
[…] main clinical symptoms in polyneuropathy (selected from [40, e15]) Patient history: personal, system, occupational, social, and family history Main symptoms and clinical findings Symptoms Clinical findings* Sensory Sensation of furriness and numbness Hypesthesia [ncbi.nlm.nih.gov]
- Sexual Dysfunction
In a small minority of the patients, the autonomic nervous system may also be involved and the patients may experience persistent nausea, vomiting, diarrhea, constipation, incontinence, sweating abnormalities or sexual dysfunction. [hopkinsmedicine.org]
Sexual dysfunction and abnormally low blood pressure also can occur. Joints are particularly vulnerable to stress in people with polyneuropathy because they are often insensitive to pain. [powerofpain.org]
Typical symptoms are constipation, sexual dysfunction, and fluctuating blood pressure—most notably a sudden fall in blood pressure when a person stands up ( orthostatic hypotension ). The skin may become pale and dry, and sweating may be reduced. [msdmanuals.com]
Sexual dysfunction and abnormally low blood pressure also can occur. [webmd.com]
A preliminary physical examination along with past medical history is taken initially. This is important for gathering information about the duration, severity and nature of symptoms that are being experienced by the individual. In many cases, physical examination is enough for arriving at a definite diagnosis. In cases when this isn’t enough, additional tests may be required .
Specialized nerve tests such as nerve conduction testing and electromyography are employed for further evaluation of the condition. Both these procedures are painful, wherein a needle is pricked into the muscles and electric shocks are given to understand the severity of condition. Quantitative sensory testing is another type of nerve test that is employed for diagnosing polyneuropathy. This test gives information about how well the patient is able to tolerate temperature changes and respond to vibration.
Treating the underlying cause and effective management of symptoms forms the basis of treatment. In many instances, it has been observed that with correction of the underlying disease condition (for example diabetes), neuropathy begin to show signs of improvement without any additional treatment .
- Medications: For mild pain, over the counter pain medications may do the needful. In severe cases, corticosteroids may have to be employed for reducing the pain and associated inflammation. Anti-seizure medications have also shown to have a profound effect on nerve pain.
- Transcutaneous electrical nerve stimulation: This method employs administration of electrical impulses to specific affected areas. It can be given along with other treatment methods for relieving acute and severe forms of pain .
- Antidepressants: Antidepressants have also been found to be helpful in relieving the nerve pain. They work by blocking the chemical processes in the brain that get activated in response to pain.
The prognosis of the condition is favorable but depends on the extent of severity of symptoms and its underlying cause. Symptoms improve with initiation of treatment and if underlying etiology is effectively treated or managed. However, if initiation of treatment is delayed then various debilitating complications can set in.
Acute polyneuropathy is caused due to infections, exposure to toxins, HIV/AIDS, various drugs, autoimmune reactions and diseases such as cancer. When the cause of polyneuropathy cannot be determined, it is known as idiopathic polyneuropathy. Drug induced polyneuropathy is a result of cancer chemotherapy drugs. Autoimmune reactions can cause the body’s own immune system to mistakenly attack the peripheral nerves giving rise to Guillan-Barre syndrome; also known as acquired polyneuropathy .
Chronic form of polyneuropathy occurs as a secondary complication of various conditions and factors such as diabetes mellitus, alcohol consumption, impaired tolerance to glucose and connective tissue diseases characterized by degeneration of the nerves. In rare cases, nutritional deficiencies, kidney or liver failure and Lyme disease can also cause polyneuropathy .
Anatomically, the peripheral nerves pass through the spinal cord and reach the skin, muscles, glands and internal organs. In polyneuropathy, malfunctioning of nerves may be noticed simultaneously in several regions of the body. The basic phenomenon that induces polyneuropathy is injury to the peripheral nerves that eventually disrupt the blood supply to the body part, interfering with oxygen and nutrient supply that are required for appropriate nerve functioning, ultimately giving rise to several debilitating symptoms .
Polyneuropathy is classified into 3 distinct categories based on the type of nerve cell that is affected. These include distal axonopathy, myelinopathy and neuronopathy. Distal axonopathy primarily occurs due to metabolic derangement of neurons. Myelinopathy develops as a result of loss of myelin sheath. Neuronopathy occurs due to destruction of peripheral nervous system .
Effective management of conditions that can increase the risk of developing polyneuropathy should be done. Individuals are advised to remain healthy, lead an active lifestyle and adopt healthy dietary practices. It is also necessary to avoid the factors that can lead to nerve damage.
Polyneuropathy is a disease affecting the peripheral nerves and is therefore, also known as peripheral polyneuropathy. The condition can be both acute or chronic in nature. In the preliminary stages, the condition strikes the feet and hands and then eventually affects the arms and legs. As the disease progresses to the advanced stages, it can also significantly affect the autonomic nervous system. Polyneuropathy affects almost the same areas on both sides of body. In such a type of condition, many body parts are affected at almost the same time .
Polyneuropathy occurs due to damage of the peripheral nerves which eventually leads to development of debilitating signs and symptoms. The condition can significantly affect both the sides of body. Various factors such as diabetes, HIV/AIDS, inherited disease, alcoholism, autoimmune diseases, vitamin deficiencies and trauma or injury to nerve can predispose an individual to develop polyneuropathy. Symptoms of polyneuropathy include pain and numbness in the affected limb, along with reduced tolerance to heat and a decreased production of sweat. Affected individuals also lose control over bowel movements and there is development of dizziness and /or lightheadedness. Treatment is geared towards pain management and correction of the underlying cause. Various medications are administered to relieve the debilitating pain and inflammations. In many instances, anti-seizure medications may also have to be employed.
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