Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) – Symptoms, Diagnosis And Treatment
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by a chronic, symmetric sensorimotor disorder with cytoalbuminologic dissociation and interstitial and perivascular endoneurial infiltration by lymphocytes and macrophages. This features make CIDP to be considered the chronic clinical equivalent of acute inflammatory demyelinating polyradiculonevritis (Guillain-Barre syndrome). There are a variety of subtypes with different immunological and inflammatory aspects that can be framed in class of chronic acquired demyelinating neuropathies.
CIDP subtypes also include distal simetric disorders with predominantly sensory symptoms, with multifocal sensorimotor neuropathy or sensorimotor mononeuropathy multiplex with prominent conduction block (Lewis-Sumner neuropathy) and subtypes associated with other systemic disorders.
CIDP associated with immunoglobulin M paraproteins; with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) and with antimyelin-associated glycoprotein antibodies (MAG antibodies) are considered to be distinct forms of CIPD because they have a specific cause and respond differently to treatment than other subtypes of CIDP.
Different from Guillain-Barre syndrome by evolution in time, chronic inflammatory demyelinating polyradiculoneuropathy is an immune mediated neurological disease that can occur at all ages, between 2 and 70 years, with a slight predominance in males. Most commonly occurs between 50 and 60 years, and recurrent forms of the disease are more common in young people.
CIDP can develop after acute respiratory infections, vaccinations, surgery and pregnancy. Similar manifestations to CIDP were observed in non-Hodgkin’s lymphoma, AIDS, malignant melanoma, myasthenia gravis, multiple sclerosis, chronic liver diseases and carcinomas.
The exact cause of the disease is unknown, but is assumed to be an immunologically mediated disease, characterized by segmental demyelination of peripheral nerves. There may be a genetic predisposition for this condition.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy Symptoms
Typical symptomatology of CIDP is charcaterized by symmetrical, sensorimotor polyneuropathy with gradual development of motor deficits (sometimes deficits may exacerbate). Reflexes are depressed. Although some symptoms are similar to Guillain-Barre syndrome, respectively aspect of cerebrospinal fluid and electrophysiological abnormalities (aspects of demyelinating polyneuropathy), CIDP differentiates from Guillain-Barre syndrome by the following aspects:
- Slow progression, lasting for a period of 2-3 months;
- A more reduced severity compared with the acute form;
- Fluctuations of disease severity and symptoms over the years;
- Higher levels of proteins in cerebrospinal fluid;
- A much lower nerve conduction velocity;
- Associated with systemic diseases like lupus or Hodgkin’s lymphoma.
CIDP debuts slowly, insidiously, with a evolution of months to its maximum intensity. Initial symptoms are present in the limbs, predominating motor impairments. The disease is characterized by progressive symmetric polyneuropathy with symmetrical paresis, proximal and distal, with early onset. Paresis are getting worsen after 6-9 weeks of evolution of the disease, are moderate or severe and are associated with depressed reflexes, decreased or normal muscle tone, muscle atrophy and fasciculations. Respiratory disturbances are rare, and when they occur assisted ventilation is required.
Sensory disturbances are characterized by numbness in the fingers,which are not accompanied by pain. Proprioceptive and vibratory sense deficits are characteristic and gait is frequently abnormal. Type of gait depends on location of weakness and degree of proprioceptive loss, patients may present steppage gait or a slapping gait.
Autonomic nervous system impairments are characterized by orthostatic hypotension, cardiac disorders and sphincter disorders.
Cranial nerves are rarely affected. Impairment of facial nerve is leading to unilateral or bilateral facial paresis. In less than 15% of patients were found oro-pharyngeal muscle paralysis. Ophthalmoparesis may occur in 5% of patients and is unilateral or bilateral, symmetrical and accompanied by palpebral ptosis. Diplopia can occur and is produced by impairment of cranial nerves III, IV, or VI.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy Diagnosis
Chronic inflammatory demyelinating polyradiculoneuropathy is diagnosed based on symptoms and paraclinical examinations.
Lumbar puncture reveals a cerebrospinal fluid with normal pressure and acellular. The most important anomaly is represented by an increased protein concentration over 1000 mg / dl, but without a high cellularity. This anomaly is called albuminocytological dissociation.
Sensory and motor nerve conduction velocity show demyelinating and axonal changes.
Nerve biopsy shows myelin degeneration in 40% of cases in the peripheral nerves and posterior roots of spinal nerves and interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages with local edema. Evidence exists of segmental demyelination and remyelination with onion bulb formation, in10%-40% of cases.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy Treatment
Corticosteroid therapy has proven effective in treating CIDP, as well as intravenous administration of immunoglobulin. Plasmapheresis is useful in treating this disease, but not as useful as imunoglubuline therapy. Other therapies are represented by alpha-interferon, anti-D immunoglobulin and cyclosporin A.
Chronic inflammatory demyelinating polyradiculoneuropathy is a slowly progressive disease with relapses and remissions. Mortality is between 5% -10% and most commonly occurs by intercurrent infections. Corticosteroid therapy associated with plasmapheresis confer a very good prognosis, even with full recovery.