Diseases Diseases

About Neuronopathy

Introduction

The term neuronopathy was introduce to describe a neurological syndrome characterized by primary damage of the nerve cell body.

Neuronopathy it an inherited neuromuscular disease that causes progressive muscle wasting weakness in the limbs without any sensory impairment. It is also a functional disturbance or pathological change in the peripheral nervous system complication of disease. Disorder of the peripheral nervous system.

Paraneoplastic origin is one of the causes of sensory neuronopathy.The main clinical complains at the onset are pain and paresthesias with asymmetric distribution tha involve arms rather than legs and later pain is replaced by numbness, limb ataxia, and psedoathetotic movements of the hands.

Subacute sensory neuronopathy is a paraneoplastic syndrome, which occurs mostly in lung, breast, ovarian malignancies and lymphoma. Sensory neuronopathy as a preceding sign of recurrence in colon adnocarcinoma has not previously been reported.

Axonal degeneration represents one of the earliest pathological features in motor neuron diseases.It remains unclear however whether axonal dying back is triggered in the axon itself of whether it is a consequence of degenerative changes originating in the cell body.

History

The account presented in this provides a detailed historical review of the development of thought relating to the causes of neuromuscular disease.

A state of the art period to 1850-1890 were a period of parallel and interdependent advances in both normal neuro-anatomy and neurophysiology, and diseases recognized as variation from such norms. In 1891 was described the first case of childhood neuronopaty recognized as such, and with this development was ushered in the modern era of clinical differential diagnosis of childhood neuromuscular disease.

Features

Symptoms of neuronopathy are weak extensor muscles of big toe,muscle weakness in lower legs, paralysis of lower leg muscles, wasting of lower leg muscles, paralysis of lower arm muscles , wasting of lower arm muscles, reduced reflexes in legs and absent reflexes in legs.

Diabetic neuropathy is a complication of diabetes causing damage to the nerves that allow you to feel sensations such as pain. The areas of the body most commonly affected by diabetic peripheral neuropathy are the feet and legs. Nerves damage in the feet can result in a loss of foot sensation, increasing ones risk of foot problems.

Tips and comments

In paraneoplastic neurological syndromes associated with neuronal damage, sensory neuronopathy rarely improves with treatment. The best chance to at least stabilize the syndrome is to induce a complete response of the tumor. Immunotherapy rarely is effective but a trial with intravenous immunoglobulins, steroids or plasmapeheresis in indicated because there are a few patient who improved.

The continual further refining of an exact diagnosis is the only way in which a realistic prognosis can be forecast, correct genetic counseling can be offered, and if the condition is treatable optima therapy can be introduced.

In the case of subacute sensory neuronopathy without an obvious underlying etiological factor, an occult malignity should always be researched in clinical practice.

Signs of terminal axonal reinnervation, such as motor axon reflex, may be observed in axonal neuronopathies, whereas only collateral reinnervation occurs in motor neuronopathies. Sensory axonopathies are usually characterized by clinical and electrophysiological findings that are axon length-dependent, by the course of the disorder that is most often chronic, and by possible reversibility.

By Emma Wanjiku, published at 04/03/2012
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