Autoimmune diseases


CIDP IN Children

CIDP in childhood

Pain Med. 2002 Jun;3(2):119-27.

Human pooled immunoglobulin in the treatment of chronic pain syndromes.

Goebel A, Netal S, Schedel R, Sprotte G.
Klinik fur Anaesthesiologie, University Wurzburg, Wurzburg, Germany.

Objective. To examine the use of intravenous immunoglobulin (IVIG) in chronic pain. Design. A prospective multiple-dose, open-label cohort study in 130 consecutive patients who suffered from 12 chronic pain syndromes. The largest symptom groups were (number of patients): Fibromyalgia (48); Spinal pain (20); Complex regional pain syndrome (CRPS, 11); Peripheral neuropathic pain (12); and Atypical odontalgia or atypical facial pain (11). All patients had insufficient pain relief with established treatments. Pain relief was recorded using average pain intensity values as documented in standardized diaries. A specific treatment protocol was developed, and patients were enrolled over a 36-month period. Results. Overall, 20% of patients had>70% pain relief and 27.7% of patients reported relief between 25% and 70%. Six patients (4.6%) had moderately increased pain levels for a duration of up to 9 weeks. Good relief, of more than 70%, was found in all major symptom groups. Patients with pain of short duration (<2 years) reported high relief rates (33.8% of patients in this group reported relief openface>70%). No serious adverse events were reported. Conclusions. IVIG may be effective in patients suffering from chronic pain. Controlled studies are needed to evaluate the efficacy of IVIG in these patients. Patients with a good response to IVIG may be models for the study of neuroimmune interactions in chronic pain.

PMID: 15102158 [PubMed - in process]

Childhood chronic inflammatory demyelinating polyneuropathy.

Nevo Y.
The Institute for Child Development, Division of Pediatrics, Dana Children's Hospital, Sackler School of Medicine, Tel Aviv University, Israel.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic disorder of the peripheral nervous system with sensory and motor involvement, and insidious onset over a period of months. In children and adults, both proximal and distal muscles are affected. Muscle stretch reflexes are absent or depressed. Laboratory findings include elevated cerebrospinal fluid protein with no increase of mononuclear cells. Electrophysiological and pathological studies show evidence of demyelination. No control studies of the efficacy of immunomodulating therapy in childhood CIDP are available. However, several studies have indicated clinical improvement after treatment with prednisolone, plasmapheresis and intravenous immunoglobulin, but disappointing results with other immunosuppressive agents. While some children have a monophasic course, with complete recovery, others have a protracted course, with either a slowly progressive or a relapsing-remitting course, resulting in prolonged morbidity and disability.

PMID: 10726588 [PubMed - indexed for MEDLINE]

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It has been generally reported that children respond well to IVIg. Read the research articles below. Steroids may not help or may even worsen CIDP in children. Some children may develop relapses. More frequently seen in males , CIDP starts at two months of age and usually presents as loss of ambulation.

Warning for children with CIDP BELOW

Do not give steroids to children they can develop permanent damage

Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies, and initial clinical course, with comparison to adults.

Simmons Z, Wald JJ, Albers JW.

Division of Neurology, Pennsylvania State University College of Medicine, Hershey Medical Center, Hershey 17033, USA.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare in children. We reviewed features of 15 children with  CIDP, and compared these to 69 adults with  CIDP. Children demonstrated many similarities to adults: (1) Antecedent events were uncommon. (2) There was a high frequency of weakness and reflex loss, a relatively high frequency of sensory loss, and a low frequency of pain and cranial neuropathies. (3) Cerebrospinal fluid protein levels were usually elevated. (4) On electrodiagnostic testing, not all nerve segments were abnormal, and not all children satisfied electrodiagnostic criteria for CIDP. Children differed from adults with CIDP in several ways: (1) The onset of symptoms was usually more precipitous. (2) Gait abnormalities were a more frequent presenting symptom. (3) Children always presented with significant neurological dysfunction, and not with the minor symptoms initially seen in some adults. The initial response of children with CIDP to immunomodulating therapy was excellent.

Chronic inflammatory demyelinating polyradiculoneuropathy in children: II. Long-term follow-up, with comparison to adults.

Simmons Z, Wald JJ, Albers JW.

Division of Neurology, The Pennsylvania State University College of Medicine, Hershey Medical Center, 17033, USA.

We previously reviewed the presentation, initial clinical course, and electrodiagnostic features of children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We now report the long-term follow-up of 12 children with CIDP, and compare these to 62 adults with  CIDP. Children often had more rapidly fluctuating courses than adults. A relapsing course was significantly more common in children than in adults. The recovery of children from each episode of deterioration was usually excellent, and better, on average, than in adults. Ventilatory support was never required for children with slowly evolving illness; only 2 children with a precipitous onset clinically resembling Guillain-Barre syndrome required ventilatory support. Prednisone, plasma exchange, and intravenous immunoglobulin (IVIg) usually were effective in children. Multiple courses of IVIg could be given with continued efficacy. Treatment often could be discontinued in children with relapsing courses. The prognosis for children was excellent. Adults demonstrated a good, but more variable, outcome.

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