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Chronic Inflammatory Demyelinating Polyneuropathy in Children:A Case Study

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder characterised by patchy demyelination of nerve roots and distal nerves. The course may be monophasic progressive or relapsing remitting. CIDP is less common in children than in adults. Children with CIDP present with proximal and distal weakness and loss of deep tendon reflexes. Children are most often brought to medical attention due to gait disturbance and falling. Immunological treatment is the mainstay of treatment in modern medicine. Here is a case study of a 10 year old girl, a diagnosed case of CIDP. She was managed with steroids for the past 1 year, but she had frequent relapses and the condition progressed. She was managed following the principles of Ayurveda, and the child, who earlier could walk only when completely supported at the axilla, was able to walk without support, run, jump and climb stairs. She did not have any relapses for the past 1 year and steroids were completely withdrawn. This shows the immense possibility of Ayurveda in managing many progressive immunological and neurological conditions, a major part of which is yet to be explored.

Key Words: Chronic inflammatory demyelinating polyneuropathy, CIDP, Guillain Barre Syndrome, Immunology

INTRODUCTION

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, acquired neuropathy, presumably of immunological origin. The main pathogenesis taking place is the inflammation of peripheral nervous systems resulting in damage of myelin sheath leading to demyelination and thus an impaired signal conduction in the neurons. The disease has an extremely variable clinical presentation and course. Typical cases are associated with progressive or relapsing-remitting motor and sensory deficit. The repeated episodes of demyelination and re-myelination also causes axonal degeneration. The motor and sensory spinal nerve roots and peripheral nerves are most often affected. The cranial nerves are less often involved and CIDP usually spares the nerves that supply muscles of respiration and the autonomic nerves that control the bladder, bowel, and circulation (Said G, 2006). As compared to adults children usually have a more precipitous onset, with gait abnormalities as a more frequent presenting symptom with significant neurological dysfunction. (Simmons, Z., Wald, J. J., & Albers, J. W., 1997).

The diagnosis is made by a combination of clinical signs and symptoms, evidence of demyelination on nerve conduction tests, and by excluding other causes. Supportive tests, such as cerebrospinal fluid analysis, magnetic resonance imaging of spinal nerve roots and nerve trunks and nerve biopsy are also used to confirm the diagnosis.The first line treatment of CIDP in modern medicine includes corticosteroids, immunoglobulin and plasmapheresis (Koller H et al, 2005). These modalities are capable of providing a temporary relief but is associated with complications of long term steroid use like potential risk for hypertension, diabetes mellitus and obesity (Markowitz, J. A., Jeste, S. S., &Kang, P. B., 2008). There is a need for an effective Ayurveda management as the condition is disabling and can affect the quality of life of these patients.

Ayurveda has not described the disease as such, but a proper look into the samprapti ghataka involved and a chikitsa based on the vyadhyavatha could revert the samprapti and manage the condition effectively and thus help to prevent the further progression of the condition.

CASE REPORT

The 10 year old female child was bought to the OPD of Department of Kaumarabhritya, Govt. Ayurveda College, Thiruvananthapuram with complaints of inability to walk without support, difficulty in doing fine motor activities like buttoning and weight gain for the past 1 year. She was a diagnosed case of Chronic Inflammatory Demyelinating Polyneuropathy since January 2018 and is under constant follow up from a tertiary care centre at Thiruvananthapuram. The complaints started with a difficulty to climb stairs and difficulty to raise from sitting position and was managed with steroids. The disease had a relapsing course on discontinuation of steroids. The symptoms worsened with each relapse. Gradually she started to have frequent falls in walking and slipping of chapels. Subsequent relapses resulted in dragging of feet while walking, inability to walk without support and difficulty to do fine motor movements like buttoning. Each episode was managed with steroids and due to which she had puffiness of face and she also started to gain weight. She had no respiratory complaints and no change in bowel bladder function.

EXAMINATION ON ADMISSION

The child had a pitta kapha prakriti with a sthoola (obese) configuration with a puffy face; coated tongue and badha mala (constipated bowel). Nervous examination was notable for proximal and distal weakness of the lower extremities, diffusely diminished deep tendon reflexes with flexor plantar response and reduced touch sensation. The muscle power on examination was 2/5 in the lower extremities and 3/5 for the upper extremities. She could make only a few steps when completely supported at the axilla. There were no cranial nerve abnormalities and other sensory modalities were intact.Cardiovascular system and respiratory system were intact.

 

Date Published: 
Monday, October 2, 2023
Article Language : 
Authors: 
Athira S
Mini S Muraleedhar
Reenu Mathew