Neurology

INDICATIONS

IVIG has proven effectiveness in different neurological disorders. Use of IVIG in different neurological disorders has increased over last few decades. Evidence from a series of randomized & control trials support the use of IVIG in different neurological disorders. Some of key neurological disorders that respond well to IVIG treatment are listed below with brief description.

CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHIES (GBS, MILLER FISHER SYNDROME)

CIDP typically starts insidiously and evolves slowly, in either a slowly progressive or a relapsing manner, with partial or complete recovery between recurrences; periods of worsening and improvement usually last weeks or months. Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made. Symptoms reported include the following preceding infection (infrequent), initial limb weakness, both proximal and distal, sensory symptoms (eg, tingling and numbness of hands and feet), motor symptoms (usually predominant).

MULTIFOCAL MOTOR NEUROPATHY

In patients with multifocal motor neuropathy, a distal asymmetric weakness of the extremities usually develops, often affecting the upper limbs first, then usually progressing slowly over decades, with minimal or no sensory or cranial nerve involvement. The involved nerves show diagnostic multifocal persistent partial conduction blocks involving motor fibers that somewhat spare sensory nerves.

MYASTHENIA GRAVIS

Myasthenia gravis (MG) is a relatively rare autoimmune disorder in which antibodies form against acetylcholine nicotinic postsynaptic receptors at the neuromuscular junction of skeletal muscles.[1, 2] MG is sometimes identified as having an ocular and generalized form, although one is not exclusive of the other and the ocular form is considered an initial, milder form of illness that progresses to the more severe generalized form in most but not all patients.

The usual initial complaint is a specific muscle weakness rather than generalized weakness. Extraocular muscle weakness or ptosis is present initially in 50% of patients and occurs during the course of illness in 90%. The disease remains exclusively ocular in only 16% of patients. Rarely, patients have generalized weakness without ocular muscle weakness. Bulbar muscle weakness is also common, along with weakness of head extension and flexion. Limb weakness may be more severe proximally than distally. Isolated limb muscle weakness is the presenting symptom in fewer than 10% of patients. Weakness progresses from mild to more severe over weeks or months, with exacerbations and remissions. Weakness tends to spread from the ocular to facial to bulbar muscles and then to truncal and limb muscles. About 87% of patients have generalized disease within 13 months after onset. Less often, symptoms may remain limited to the extraocular and eyelid muscles for years.

MULTIPLE SCLEROSIS

Multiple sclerosis (MS) involves an immune-mediated process in which an abnormal response of the body’s immune system is directed against the central nervous system (CNS), which is made up of the brain, spinal cord and optic nerves. The exact antigen — or target that the immune cells are sensitized to attack — remains unknown, which is why MS is considered by many experts to be “immune-mediated” rather than “autoimmune”.

ALZHEIMER'S DISEASE

Alzheimer’s disease is the most common cause of dementia. The word dementia describes a set of symptoms that can include memory loss and difficulties with thinking, problem-solving or language. These symptoms occur when the brain is damaged by certain diseases, including Alzheimer’s disease. This factsheet describes the symptoms of Alzheimer’s disease, how it is diagnosed, and the factors that can put someone at risk of developing it. It also describes the treatments and support that are currently available.

LAMBERT-EATON SYNDROME

Lambert-Eaton myasthenic syndrome (LEMS) is a rare presynaptic disorder of neuromuscular transmission in which quantal release of acetylcholine (ACh) is impaired, causing a unique set of clinical characteristics, which include proximal muscle weakness, depressed tendon reflexes, posttetanic potentiation, and autonomic changes. The initial presentation can be similar to that of myasthenia gravis (MG), but the progressions of the 2 diseases have some important differences.

STIFF-PERSON SYNDROME

Stiff person syndrome is rather unique among neurologic diagnoses because of its lack of significant similarity to any other neurologic diseases. Although rare, once observed it is quite unforgettable. Possibly the closest related disease is tetanus because both conditions affect peripheral inhibition via central mechanisms and both conditions inhibit central gamma-aminobutyric acid (GABA) systems.