CIDP & GBS in Children

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 , starts at two months of age and usually presents as loss of ambulation.

Muscle Nerve. 1997 Aug;20(8):1008-15. Related Articles, Links

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.

PMID: 9236792 [PubMed - indexed for MEDLINE]

Muscle Nerve. 1997 Dec;20(12):1569-75. Related Articles, Links

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.

PMID: 9390670 [PubMed - indexed for MEDLINE]

Singapore Med J. 2004 Nov;45(11):536-7. Related Articles, Links

Chronic inflammatory demyelinating polyneuropathy in a child: clinical-spinal MR imaging correlation.

Likasitwattanakul S, Visrutaratna P.

Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. slikasit@mail.med.cmu.ac.th

Spinal magnetic resonance (MR) imaging of a 3-year-old girl with chronic inflammatory demyelinating polyneuropathy (CIDP) showed thickened and marked enhancement of the lumbosacral nerve roots. These abnormalities resolved after steroid treatment. MR imaging of the cauda equina may be helpful in the diagnosis of CIDP.

Figure 7 : Chronic inflammatory demyelinating polyneuropathy (CIDP) in a 13-year-old boy with peripheral neuropathy and gait disturbance. Sagittal fat-saturated T1-weighted images off the midline to the right (A), at the midline (B), and off the midline to the left (C). Chronic inflammatory demyelinating polyneuropathy (CIDP) in a 13-year-old boy with peripheral neuropathy and gait disturbance. D, E, F, and G, Axial postcontrast T1-weighted fat-saturated images through the lower thoracolumbar junction (D), conus medullaris (E), and nerve roots (F, G). The images show marked enhancement of the spinal nerve roots (arrows in A through F), with expansion of the nerve roots seen as they exit through the neural foramina (asterisks in F and G). This enlargement is typical of CIDP.

Pediatr Neurol. 2003 Sep;29(3):236-8. Related Articles, Links

Progressive muscle weakness after high-dose steroids in two children with CIDP.

Rostasy KM, Diepold K, Buckard J, Brockmann K, Wilken B, Hanefeld F.

Department of Pediatrics and Neuropediatrics, Georg-August-Universitat Gottingen,., Gottingen, Germany.

Corticosteroids and intravenous immunoglobulins belong to the first line of treatment in chronic inflammatory demyelinating polyneuropathy. In patients with a progressive course, plasma exchange and immunomodulatory drugs are added to the regimen. To reduce the side effects of long-term oral prednisolone, high-dose pulsatile intravenous methylprednisolone treatment has been advocated. We report two children with chronic inflammatory demyelinating polyneuropathy who, after high-dose intravenous pulsatile methylprednisolone, experienced a significant clinical deterioration with profound loss of muscle strength. Both patients improved after changing treatment to immunoglobulins in one and cyclosporine combined with immunoglobulins and oral prednisolone in the other.

Publication Types:
Case Reports

PMID: 14629908 [PubMed - indexed for MEDLINE]

Neuromuscul Disord. 2000 Aug;10(6):398-406. Related Articles, Links

Childhood chronic inflammatory demyelinating polyneuropathy: clinical course and long-term outcome.

Ryan MM, Grattan-Smith PJ, Procopis PG, Morgan G, Ouvrier RA.

Department of Neurology, The Royal Alexandra Hospital for Children, Sydney, Australia. ryan_mo@a1.tch.harvard.edu

We reviewed the clinical history, electrophysiologic and pathologic findings, and response to therapy of 16 children with chronic inflammatory demyelinating polyneuropathy. The majority presented with lower limb weakness. Sensory loss was uncommon. The illness was monophasic in seven children, relapsing in six, and three had a slowly progressive course. All patients were treated with immunosuppressive agents. In 11, the initial treatment was prednisolone. All had at least a short-term response but five went on to develop a relapsing course. Intravenous immunoglobulin was the initial treatment in four patients. Three responded rapidly, with treatment being stopped after a maximum of 5 months. In resistant chronic inflammatory demyelinating neuropathy, in addition to prednisolone and immunoglobulin, plasma exchange, azathioprine, cyclosporine, methotrexate, cyclophosphamide and pulse methylprednisolone were tried at different times in different patients. On serial neurophysiologic testing slowing of nerve conduction persisted for long periods after clinical recovery. Follow-up was for an average of 10 years. When last seen 14 patients were asymptomatic, two having mild residual deficits. Childhood chronic inflammatory demyelinating neuropathy responds to conventional treatment and generally has a favourable long-term outcome.

Publication Types:
Clinical Trial

PMID: 10899445 [PubMed - indexed for MEDLINE]

Eur J Paediatr Neurol. 1998;2(4):169-77. Related Articles, Links

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.

Publication Types:
Review

PMID: 10726588 [PubMed - indexed for MEDLINE]

Pediatr Neurol. 2001 Mar;24(3):177-82. Related Articles, Links

Chronic inflammatory demyelinating polyneuropathy in childhood.

Connolly AM.

Department of Neurology, St. Louis Children's Hospital, Washington University of Medicine, St. Louis, Missouri 63110, USA.

Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is relatively rare. However, it has been recognized for many years. In patients presenting with this disease, subacute onset of weakness usually develops over at least 2 months and often progresses to a loss of ambulation. Some children's initial presentations may mimic Guillain-Barre syndrome. Dysasthesias are common. Males are affected more than females, and antecedent illnesses or vaccinations occur in approximately half of patients. Physical examination reveals diffuse, proximal greater than distal weakness, with an absence or depression of muscle stretch reflexes. Electrophysiology confirms demyelination, and spinal fluid examination demonstrates albuminocytologic dissociation. The clinical presentation, diagnosis, and prognosis of childhood CIDP are reviewed. Treatment and immunologic features are also discussed in this article.

Publication Types:
Review

PMID: 11301217 [PubMed - indexed for MEDLINE]