CIDP in Children

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By CIDPUSA.ORG,
Sat, 31 May 2010 17:55:07

CIDPUSA

CIDP seen in Children

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Childhood CIDP may occur in its typical form, but even ~50% of children can present as an atypical variant including distal, pure motor, or pure sensory. Most children have a good prognosis; however, many of them may require long-term treatment. This highlights the importance of an early diagnosis and treatment for childhood CIDP.

CIDP symptoms, by definition, progress gradually over a period of more than 8 weeks in most cases, although especially in children, the disease onset might be acute, developing in <4 weeks, or subacute in 4-8 weeks. An acute onset of childhood CIDP may help establish the correct diagnosis of inflammatory neuropathy. However, slow progression, especially in younger children or even infants, can be overlooked and lead to a Charcot-Marie-Tooth (CMT) disease diagnosis.

The course of CIDP may be polyphasic-relapsing-remitting in 61% of patients, or monophasic, progressive in 39% (3). The typical presentation includes symmetric proximal and distal weakness and/or sensory dysfunction of limbs with hyporeflexia or areflexia.

In most children weakness was distributed similarly between proximal and distal weakness.
Cranial nerve involvement was more frequent in children <4 vs. 4-13 years old dditional concurrent symptoms, including involuntary facial muscle movements and hand tremor. We have observed autonomic dysfunction in two patients (2/37, 5.4%)-gastrointestinal (constipation) in both cases.anisocoria L>R; (2) fasciculation of tongue muscles; (3) bilateral facial nerve weakness, bilateral damage of trigeminal nerves,episodes of choking and ptosis.

The two patients with CIDP onset at younger than 4 years presented with intradural nerve root (cauda equina) enhancement. In one of them, intradural nerve root thickening and trigeminal nerve enhancement were seen. Four of seven patients with CIDP onset between 7 and 13 years old presented with intradural nerve root enhancement. Two patients in this onset age group presented with intradural nerve root thickening (one with enhancement and one without). Two of five patients with CIDP onset at older than 13 years presented with intradural nerve root enhancement this information taken from Department of Neurology, Medical University of Warsaw, Warsaw, Poland Anna Kostera-Pruszczyk MD.Pediatric CIDP: Diagnosis and Management. A Single-Center Experience

The mean age of onset was 7 years and 3 months; range 2-16 years. Symptom onset was acute (<4 weeks) in five, three had a sub-acute (4-8 weeks) and more than 50% had a chronic (>8 weeks) onset (n = 13). Two children included in the chronic group had leg pains and foot deformity for more than a year and then became non-ambulant acutely. The interval between symptom onset and maximum disability ranged from <1-44 months. Preceding infectious illness was present in less than half (n = 11).

The majority of children presented with gait difficulties (n = 11); all subsequently developed 4 limb involvement. All children were areflexic with one hyporeflexic at presentation. 3/21 (14%) had bulbar weakness with weak cry or swallowing/speech difficulty with two others having cranial nerve involvement with anisocoria/optic neuritis and bilateral lower facial weakness, respectively. STUDY DONE BY Great Ormond Street Institute of Child Health,Dr P Munot Clinical spectrum, treatment and outcome of children with suspected diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy

PMID: 9040714 [PubMed - indexed for MEDLINE]
Diabetes Metab. 2001 Apr;27(2 Pt 1):155-8.
An unusual neuropathy in a diabetic patient: evidence for intravenous immunoglobin-induced effective therapy.
Romedenne P,.
-St Joseph Medical Center, Mons, Belgium.

We report the case of a 68-year old type-2 diabetic male patient who was admitted to hospital for progressive weakness in the right lower limb. Although his metabolic control was good, he lost more than 20 kg of weight. Despite intensive physio- and vitaminotherapy, his neurological condition kept on degrading with a severe amyotrophy ( hip weakness) and pain of the right thigh. He was unable to walk and to stand alone. Besides a yet known sensitive polyneuropathy, the electrophysiological study signs of demyelination and axonal degeneration. Combined with the albuminocytologic dissociation observed in the cerebrospinal fluid, a diagnosis of inflammatory neuropathy was diagnosed. The patient underwent a treatment by methylprednisolone and with immunoglobins a striking improvement of his neurological condition. This case report illustrates that rare forms of neuropathy such as inflammatory neuropathies close to chronic inflammatory demyelinating polyneuropathy (CIDP) can occur in diabetic patients and superimpose on the more commonly described forms of neuropathies. It recalls the importance of inflammatory neuropathies are perfectly curable.

Neurol Sci. 2000 Feb 15;173(2):129-39.
(the following study shows that AAN guidelines for CIDP fail to help in diagnosing patients) 
The spectrum of chronic inflammatory demyelinating polyneuropathy.
Rotta FT, Sussman AT, Bradley
Department of Neurology, University of Miami School of Medicine,

Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by committee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP.
In our study the clinical spectrum of CIDP, Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features (feet & hands involvement) , 13 (15%) had exclusively sensory polyneuropathy ; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination (brain & spinal cord  involvement) , four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.

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