CSF Protein Concentration Research Articles

Contribution of capsular and clonal types and β-lactam resistance to the severity of experimental pneumococcal meningitis

We used a rabbit model to assess the effects of capsular serotype, genetic background and β-lactam resistance on the course and severity of experimental meningitis. Meningitis was induced by five pneumococcal strains belonging to five different clones with known invasive potential: two serotype 3 strains (ST260 3 and Netherlands 3-31 clones) and three serotype 23F strains with different β-lactam susceptibility patterns (Spain 23F-1 clone, Tennessee 23F-4 clone and a double locus variant of the Tennessee 23F-4 clone). Major differences in secondary bacteremia and mortality rates were observed between serotypes 3 and 23F, as were divergences in the CSF lactate, protein and lipoteichoic-teichoic acid concentrations. Minor differences in the CSF-induced inflammatory response were found among strains belonging to the same serotype. Our results suggest that capsular serotype might be the main factor determining the course and severity of pneumococcal meningitis and genetic background contributes to a lesser extent. The acquisition of β-lactam resistance does not reduce the virulence of the invasive clones. Since five strains belonging to two serotypes were studied, our findings have to be confirmed with other pneumococcal serotypes.

CSF flow cytometry greatly improves diagnostic accuracy in CNS hematologic malignancies

To assess the diagnostic accuracy of flow cytometric immunophenotyping in comparison with classic cytomorphology for diagnosing CNS localizations of hematologic malignancies, and to evaluate the implications of CSF pleocytosis and protein content in this context. We reviewed the results of diagnostic evaluations of all CSF samples analyzed for localization of a hematologic malignancy between 2001 and 2004 at our center. A total of 1,054 samples from 219 patients were available for analysis. Sixty patients had a CSF localization diagnosed by positive flow cytometry, cytomorphology, or both. The first sample was positive by flow cytometry in 44 (73%) patients, by cytomorphology in 19 (32%). Four first samples were positive by cytomorphology but negative by flow cytometry. Patients with positive cytomorphology had more frequent clinical symptomatology (95% vs 58%) and CSF pleocytosis (84% vs 25%), and tended to a poorer progression-free survival than patients with positive flow cytometry only. OR for CNS localization in case of CSF pleocytosis was 10.1 (95% CI 4.9 to 20.8); OR for CNS localization in case of elevated protein content was 2.9 (95% CI 1.5 to 5.4). Nevertheless, 26 of 137 (19%) patients with normal cell count and protein concentration had a CNS localization. The diagnostic value of flow cytometry is more than twice that of cytomorphology. However, cytomorphologic examination of the CSF has additional diagnostic and possibly prognostic value, and should still be performed in conjunction with flow cytometry.

Dixièmes Journées des Maladies du Système Nerveux Périphérique Diagnostic des polyneuropathies axonales chroniques : les polyradiculonévrites chroniques méconnues

Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune disease that target myelin sheats of peripheral nerves. Its diagnosis is often difficult to make, and a number of cases are probably not identified because of the clinical and electrophysiological heterogeneity. Typical cases associate progressive or relapsing-remitting motor and sensory deficit with increased CSF protein content and electrophysiological features of demyelination. In some cases electrophysiological studies fail to show evidence of demyelination, conventional electrophysiological diagnostic criteria are not filled yet the patient may respond to immunomodulatory treatments. In such cases, presence of clinical characteristics suggestive of CIDP (that means not compatible with a length-dependent axonal process) are critical justifying fully investigations including sural nerve biopsy. The main clinical characteristic are: a symmetric proximal and distal motor weakness predominantly affecting the lower limbs, a diffuse areflexia, a sensory deficit characterized by a preferential involvement of large fibers, an evolution which may be either chronic progressive or recurrent. Usual therapeutic agents (corticosteroids, intravenous immunoglobulins, plasma exchanges) seem to have the same efficacy whatever the electrophysiologic profile.

La méningite post-traumatique : incidence, microbiologie et pronostic

The aim of our study was to search for the incidence, the responsible organisms and the favoring causes of death of post-traumatic meningitis (PTM). This retrospective study was conducted over a seven-year period (January 1st, 1996 - December 31, 2002) in the ICU and the neurosurgery department of the Habib-Bourguiba University Hospital, Sfax, Tunisia. Over the study period, 38 patients presented PTM (0.96% of patients hospitalized for head injury), 92% of them had received antibiotic prophylaxis on admission. Mean time between head injury and the diagnosis of PTM was 9+/- 8 days (range: 2-34 days). The most common isolated organisms were multidrug resistant A. baumanii, and K. pneumoniae and reduced susceptibility S. pneumoniae. Factors predictive of prognosis in the 14 days following the diagnosis of meningitis were Glasgow coma score (GCS) on the day of diagnosis of PTM, absence of nuchal rigidity, CSF protein, CSF/blood glucose ratio, and S. pneumoniae as the causal agent of PTM. Antibioprophylaxis in patients with head trauma must be avoided to prevent the emergence of multidrug resistant bacteria when PTM occurs. GCS on the day of diagnosis of PTM, CSF protein concentration, CSF/blood glucose ratio, and S. pneumoniae as the causal agent of PTM are predictive factors of mortality of patients with PTM.

Clinical analysis of 16 cases of cryptococcus neofornans meningitis

目的 探讨部分新型隐球菌性脑膜炎的临床特点及诊断.方法采用回顾性的分析方法,对16例患者进行分析.结果16例患者均无明显恶液质表现,早期脑脊液表现为压力升高,轻度蛋白升高,需多次腰穿检查脑脊液才发现病原菌.结论新型隐球菌性脑膜炎早期症状不典型,不易发现病原菌,应多次作脑脊液检查以明确诊断。

Characteristics of cisternal cerebrospinal fluid associated with intracranial meningiomas in dogs: 56 cases (1985–2004)

To determine CSF characteristics associated with intracranial meningiomas in dogs. Retrospective case series. 56 dogs with intracranial meningiomas. Medical records of dogs with a histopathologic diagnosis of intracranial meningioma, in which CSF analysis had been performed, were reviewed. Information concerning total nucleated cell counts (TNCCs) and differential nucleated cell counts, RBC counts, and total protein concentration in CSF; seizure history and glucocorticoid administration; and location of meningiomas was recorded. TNCCs < 5 cells/microL were detected in 41 of 56 (73%) dogs; 5 of 56 (9%) dogs had TNCCs > 50 cells/microL. Analysis of CSF revealed predominantly neutrophilic pleocytosis in < 20% of dogs. There was a significant association between meningioma location (caudal portion of the cranial fossa or middle and rostral portion of the cranial fossae) and increased TNCCs (> or = 5 cells/microL). Results were significantly different from those routinely reported in the veterinary literature. Neutrophilic pleocytosis, especially with TNCCs > 50 cells/microL, was not typical in CSF samples from dogs with intracranial meningiomas. Neutrophilic pleocytosis may not be detected in CSF samples from dogs with meningiomas located within the middle or rostral portion of the cranial fossae.

CSF findings in 250 patients with serologically confirmed West Nile virus meningitis and encephalitis

To provide a large, comprehensive evaluation of the CSF findings in patients with serologically confirmed West Nile virus (WNV), CNS disease, and their correlation with outcome. CSF samples from 334 WNV-infected hospitalized patients were analyzed. Information was available and extracted from the medical records of 250 of these patients, and CSF parameters correlated with clinical and epidemiologic features of disease (e.g., patient age, sex, outcome). Patients with meningitis had a mean of 226 cells/mm3, and those with encephalitis had a mean of 227 cells/mm3. Three percent of meningitis patients and 5% of encephalitis patients had fewer than 5 cells/mm3, and approximately 8% of both groups had more than 500 cells/mm3. Patients with meningitis had a mean of 41% neutrophils, and those with encephalitis had 45%. Forty-five percent of meningitis patients and 37% of encephalitis patients had at least 50% neutrophils in their initial CSF specimen. Neither the mean percent neutrophils nor their distribution differed significantly between groups. Forty-seven percent of encephalitis patients and 16% of meningitis patients had CSF protein of 100 mg/dL or greater (p < 0.01). Although specific CSF parameters, including nucleated cell count and protein concentration, correlated significantly with outcome, multivariate analysis suggested that their total predictive value was modest. Age was an additional predictor of outcome independent of CSF variables in all patients. Serologically confirmed West Nile virus meningitis and encephalitis produce similar degrees of CSF pleocytosis and are frequently associated with substantial CSF neutrophilia. Patients with encephalitis have higher CSF protein concentrations and are more likely to have adverse outcomes, including admission to long-term care facilities or even death after their acute illness. CSF findings were only a modest predictor of disease outcome, with patient age adding important independent prognostic information.

Demyelinating motor Guillain–Barré syndrome following rubella

A 20-year-old man developed weakness without sensory complaints 10 days after rubella. He had fever, typical rubella rash, and suboccipital lymphadenopathy during an epidemic outbreak in his school. Examination showed symmetric weakness of forearm and intrinsic hand muscles, tibioperoneal muscles, and triceps surae (grade 4/5, Medical Research Council scale) and hyperactive tendon reflexes. He could not get up from squatting or walk on heels and toes. Cranial nerves and sensory examination were normal. Serum creatine kinase was normal. Serologic tests revealed immunoglobulin (Ig) G and IgM antirubella. CSF protein content was increased (0.8 g/L). Electrophysiologic examination showed partial motor conduction blocks (CBs) in eight nerves, prolonged distal motor latencies in tibial nerves, and normal sensory conductions even across the sites of CB (figure). EMG of distal muscles of limbs showed reduced recruitment with high frequency discharging motor units. Brain and spinal MRI and median and tibial somatosensory evoked potentials were normal. A diagnosis of Guillain–Barre syndrome (GBS) was made, and the patient was treated with four plasmaphereses with complete recovery in 6 months. On serial electrophysiologic examination, CBs disappeared in 8 to 16 weeks, and abnormal temporal dispersion occurred in four nerves, indicating that demyelination was the pathophysiologic basis (see figure). Sensory conductions remained normal, and fibrillation potentials were not detected through …

Hashimoto's Encephalopathy Presenting as an Acute Medical Emergency

Three cases are described of a reversible encephalopathy, all presenting with marked neurological disturbance. In all three, the diagnosis was not clear at the time of presentation but eventually it was felt all of the cases were consistent with Hashimoto's encephalopathy. The diagnosis of Hashimoto's encephalopathy should be considered in any case of unexplained encephalopathy. Common features are high anti-thyroid peroxidase antibody titres, an abnormal EEG and an elevated CSF protein concentration. The encephalopathy is independent of thyroid hormonal status. Treatment with corticosteroids leads to a prompt resolution of symptoms and long-term low dose steroid therapy prevents further neurological recurrence.

Demyelinating motor guillain‐barré syndrome following rubella

A 20‐year‐old man developed weakness without sensory complaints ten days after rubella. Examination showed limb weakness and brisk tendon reflexes but no sensory abnormalities. Laboratory investigations revealed IgG and IgM anti‐Rubella and increased CSF protein content (0,8 g/L). Electrophysiological examination showed partial motor conduction blocks in eight nerves and normal sensory conductions even across the sites of CB. Brain and spinal cord MRI and SEPs were normal. The patient was treated with four plasmaphereses and fully recovered in six months. Conduction blocks gradually improved with increasing duration and abnormal temporal dispersion in proximal CMAPs. GBS has been rarely reported after rubella. Anti‐myelin basic protein antibodies have been found in a patient with a relapsing motor neuropathy following rubella vaccination. As antibodies cross‐reacted with a viral protein, molecular mimicry has been proposed as a pathophysiological mechanism. In our patient we did not find anti‐MBP antibodies and antibodies to‐glycolipids (GM1, GM2, GA1, GD1a, GD1b, GQ1b, sulfatides, galactocerebroside) were also negative. Indirect immunofluorescence after incubation of patient's serum on rabbit sciatic nerve and human sural nerve and roots was negative. Our patient confirms the occurrence of GBS following Rubella and shows some uncommon features: 1) hyperactive deep tendon reflexes; 2) demyelination selectively involving motor fibres; and 3) widespread early conduction blocks in intermediate nerve segments.

Prognostic scoring system for primary CNS lymphomas: the International Extranodal Lymphoma Study Group experience.

To identify survival predictors and to design a prognostic score useful for distinguishing risk groups in immunocompetent patients with primary CNS lymphomas (PCNSL). The prognostic role of patient-, lymphoma-, and treatment-related variables was analyzed in a multicenter series of 378 PCNSL patients treated at 23 cancer centers from five different countries. Age more than 60 years, performance status (PS) more than 1, elevated lactate dehydrogenase (LDH) serum level, high CSF protein concentration, and involvement of deep regions of the brain (periventricular regions, basal ganglia, brainstem, and/or cerebellum) were significantly and independently associated with a worse survival. These five variables were used to design a prognostic score. Each variable was assigned a value of either 0, if favorable, or 1, if unfavorable. The values were then added together to arrive at a final score, which was tested in 105 assessable patients for which complete data of all five variables were available. The 2-year overall survival (OS) +/- SD was 80% +/- 8%, 48% +/- 7%, and 15% +/- 7% (P =.00001) for patients with zero to one, two to three, and four to five unfavorable features, respectively. The prognostic role of this score was confirmed by limiting analysis to assessable patients treated with high-dose methotrexate-based chemotherapy (2-year OS +/- SD: 85% +/- 8%, 57% +/- 8%, and 24% +/- 11%; P =.0004). Age, PS, LDH serum level, CSF protein concentration, and involvement of deep structures of the brain were independent predictors of survival. A prognostic score including these five parameters seems advisable in distinguishing different risk groups in PCNSL patients. The proposed score and its relevance in therapeutic decision deserve to be validated in further studies.

Non-obstructive hydrocephalus associated with intracranial schwannomas: hyperproteinorrhachia as an etiopathological factor?

This series illustrates the association of communicating hydrocephalus with intracranial non-obstructive schwannomas. This association has commonly been observed, however it has only been reported once previously. Moreover, in all the patients we present, hyperproteinorrhachia was a common denominator. This finding may therefore be the underlying mechanism for hydrocephalus. Seven patients presenting with intracranial schwannomas along with non-obstructive hydrocephalus and hyperproteinorrhachia are reported. Six had a vestibular schwannoma and presented with a unilateral deafness and various degrees of gait disturbance, urinary incontinence and neuropsychological impairment. Due to their advanced ages, these patients underwent a ventriculo-peritoneal shunt, and their symptoms related to hydrocephalus resolved. One patient that suffered from hemifacial dysesthesia and memory deficits presented with a non-obstructive trigeminal schwannoma. In this case the tumour was removed and the hydrocephalus was consequently reversed, and the CSF protein content normalized. The constant finding of hyperproteinorrhachia in all these patients suggests that a high CSF protein content may be the underlying cause of hydrocephalus through a speculative mechanism of decreased CSF resorption.

AUTOMIC FAILURE AND NORMAL PRESSURE HYDROCEPHALUS IN A PATIENT WITH CHRONIC DEMYELINATING INFLAMMATORY NEUROPATHY

A 75‐year‐old man with HCV hepatitis developed at the age of 70 presented with rest and action tremor localized at both hands and progressive cognitive impairment with memory loss. Four years later he begun to complain of progressive fatigue, occasional falls, numbness at the extremities and orthostatic hypotension. One month after admission, he rapidly worsened with inability to walk, mainly because of autonomic failure. Neurological examination revealed gait disturbances, including a wide base of support and short stride, slurred speech, reduction of upward gaze, rest and action tremor at both hands, intrinsic hand muscle and anterior tibialis muscle wasting and weakness on both sides, absent deep tendon reflexes, loss of vibration sense at lower limbs, and bilateral pes cavus. Routine laboratory studies, autoantibodies, thyroid function, neoplastic markers and immunoelectrophoresis were normal. Cryoglobulins were absent, whereas CSF protein content was increased (142 mg/dl). Autonomic nervous system investigation detected severe orthostatic hypotension. Nerve conduction studies showed absent sensory potentials and a marked reduction of compound motor action potential amplitudes and of motor conduction velocities. A sural nerve biopsy revealed remarkable onion bulb‐like changes, endoneurial and perivascular infiltrations of inflammatory cells. Psychometric tests showed mild cognitive impairment. Brain MRI was consistent with normotensive hydrocephalus. The findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, autonomic nervous system involvement and normal pressure hydrocephalus. A condition of multiple system atrophy (MSA) might be taken into account, even if somatic peripheral nerve involvement may rarely occur in MSA. Moreover the normal pressure hydrocephalus could be due to the high protein content in CSF (Fukatsu R et al., 1997).

INCREASED CSF PROTEINS IN CMT1A: ROLE OF ROOT HYPERTROPHY OR SUPERIMPOSED CIDP?

A 45‐year‐old male patient came to our observation with a clinical picture of slowly progressive wasting and weakness of distal limb muscles, mild distal sensory to all modalities, with pes cavus and hammer toes. His parents were consanguineous and both were affected by Charcot‐Marie‐Tooth disease (CMT). In the patient, disease onset occurred at age 11. Disease course was slowly progressive, but a somewhat sudden worsening had occurred three years before; muscle wasting and weakness were slightly asymmetrical. A previous spinal tap had demonstrated very high levels of CSF proteins (about 300 mg/dl). When admitted to our hospital, a repeat CSF examination revealed even higher protein content (380 mg/dl), raising the hypothesis of CIDP (chronic inflammatory demyelinating polyradiculoneuropathy) superimposed on CMT. Nerve conduction studies showed a diffuse and uniform nerve conduction slowing (10–12 m/s in limb nerves). Molecular analyses demonstrated the common 17p11.2 duplication of CMT1A. We performed nerve biopsy looking for signs of nerve inflammation, and MR scan of lumbar roots searching for evidence of blood‐root barrier disruption and root inflammation (root enhancement). We administered to the patient two courses of high dose intravenous immunoglobulin (IVIg), followed by steroid treatment for some weeks: there was a questionable clinical improvement and CSF protein content slightly decreased (288 mg/dl). Nerve biopsy showed the typical features of CMT1A with diffuse onion‐bulb formations, but neither focal abnormality nor inflammatory infiltrates were observed. MR scan demonstrated very mild enhancement of lumbar nerve roots after gadolinium administration, but the most impressive finding was a striking hypertrophy of the lumbar roots, which were overall much thicker than the cord itself and filled almost completely the spinal canal. Although superimposition of CIDP cannot be excluded, root hypertrophy causing leakage of the blood‐root barrier or even block of the CSF circulation appears the most likely explanation of the incredible rise of CSF protein content in this case.Partly supported by a grant from the Italian Ministry of Health.

Psychotic Symptoms and Emotional Distress in Patients with Guillain-Barré Syndrome

Psychological disturbances in 49 most severely compromised Guillain-Barré syndrome patients were prospectively studied by a semistructured interview and assessed by repeat psychiatric examination during the patients’ stay in the neuro-intensive care unit (ICU). Additional information was obtained from attending physicians, nurses and relatives. Anxiety (82%), acute stress disorder, depressive episodes (67%) and brief reactive psychosis (25%) were observed, with oneiroid psychosis (14%) among the latter. Psychotic episodes were strongly associated (p < 0.001) with severe tetraparesis, artificial ventilation and multiple cranial nerve dysfunction. CSF protein concentration was also correlated with the occurrence of psychotic symptoms. Patients themselves experienced loss of communication to be the most difficult condition to cope with. Fifty-five percent explicitly felt reassured by the environment of the ICU and 90% described contact with relatives to be most helpful. Our data suggest that motor deprivation and loss of communication are the conditions most closely connected with the occurrence of psychotic symptoms. Therapeutically, continuous psychosocial support and psychopharmacological measures may both be valuable tools to ameliorate distress.

Chronic inflammatory demyelinative polyneuropathy.

Abstract Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired polyneuropathy presumably of immunological origin. It is characterized by a progressive or a relapsing course with predominant motor deficit. The diagnosis rests on the association of non-length-dependent predominantly motor deficit following a progressive or a relapsing course associated with increased CSF protein content. The demonstration of asymmetrical demyelinating features on nerve conduction studies is needed for diagnosis. The outcome depends on the amplitude of axon loss associated with demyelination. CIDP must be differentiated from acquired demyelinative neuropathies associated with monoclonal gammopathies. CIDP responds well to treatment with corticosteroids, intravenous immunoglobulins, and plasma exchanges, at least initially.

Use of 14–3–3 and other brain-specific proteins in CSF in the diagnosis of variant Creutzfeldt-Jakob disease

OBJECTIVESThe detection of the protein 14–3–3 in the CSF has been shown to be a reliable and sensitive marker for sporadic Creutzfeldt-Jakob disease (CJD). Other brain-specific proteins such as neuron...

Both total and phosphorylated tau are increased in Alzheimer's disease

BACKROUNDPathological tau protein concentrations in CSF are found in both Alzheimer's disease (AD) and frontotemporal dementia (FTD), but studies on brain tissue have suggested that the tau pathology in AD...

IELSG prognostic score for primary central nervous system lymphomas (PCNSL): analysis of an international series of 378 immunocompetent patients

METHODS Median age was 61 ys (12-85); ECOG-PS >1= 222 (65%). High LDH serum level in 69/195 (36%), ocular involvement in 22/170 (13%), meningeal spread in 38/241 (16%), elevated CSF protein concentration in 82/134 (61%), involvement of deep structures of the brain (periventricular regions, basal ganglia, stem brain, and/or cerebellum) in 136 (36%) cases. Treatment was chemotherapy (CHT) in 32 (8%) cases, radiotherapy (RT) in 98 (26%), RT-CHT in 36 (9%), CHT-RT in 197 (53%), none in 7 (2%), data were not available in 8 (2%). RESULTS Age £60 ys (2-yr OS: 46±3% vs. 29±3%, log-rank test, p=0.00006), PS <2 (50±5% vs. 31±3%; p=0.00001), normal LDH serum level (49±4% vs. 29±5%; p=0.008), normal CSF protein level (61±7% vs. 39±5%; p=0.003), and absence of involvement of deep regions of the brain (42±3% vs. 28±4%; p=0.0006) were significantly and independently (Cox analysis) associated with a better outcome. These 5 variables were used to design a prognostic score, considering “0” the favorable feature and “1” the unfavorable one and summing the 5 results. This score was tested in 105 assessable patients for whom complete data of all the 5 variables were available. The 2-yr OS was 80±8%, 48±7% and 15±7% (p=0.00001), respectively for patients with 0‐1, 2‐3 and 4‐5 unfavorable features. This prognostic score was tested separately on the subset of 75 assessable patients treated with HD-MTX-based CHT±RT achieving similar results, with a 2-yr OS of 85±8%, 57±8% and 24±11% (p=0.0004), respectively for patients with 0‐1, 2‐3 and 4‐5 unfavorable features. CONCLUSIONS Age, PS, LDH serum level, CSF protein concentration, and involvement of deep structures of the brain were independent predictors of survival. The combined analysis of these 5 variables resulted in a prognostic score useful to distinguishing different risk groups, even in patients treated with HD-MTX-based CHT±RT. The independent role of these 5 variables and the clinical relevance of the proposed prognostic score deserve to be assessed in further studies. This score could become useful in stratifying patients and comparing results in future prospective trials.

Chronic Progressive Inflammatory Demyelinating Polyneuropathy In Childhood: Clinical And Electrophysiological Features

Chronic inflammatory demyelinating neuropathy (CIDP) occurring in childhood is rare and the diagnosis in indolently progressive cases may be difficult. We report 7 children (age 8–12 years) with insidiously progressive weakness and atrophy (in 3 cases) predominantly involving the lower limbs and mimicking a genetically determined neuropathy. Family history was negative in all cases. Numbness and paresthesias were present in 1/7 and mild sensory signs in 3/7 patients. Motor conductions were slowed in at least two nerves in 6/7, distal motor latencies prolonged in 4/7 and F waves delayed in 6/7 children, but these findings, although characteristic of a demyelinating neuropathy, did not help in the differential diagnosis with an inherited disorder. The following features documenting focal and non‐uniform nerve conduction abnormalities indicated an acquired demyelinating disorder: 1) abnormal terminal latency index in at least one nerve in 5/7 children, 2) conduction block (reduction of proximal CMAP area&gt; 50%) in at least one nerve in 5/7 children, 3)&gt; 10 m/s conduction velocity difference among nerves of upper limbs or nerves of lower limbs in 4/7 children, 4) abnormal median sensory in presence of normal sural sensory conduction in 3/7 children. CSF protein concentration was increased in 5/7 (59–120 mg/dl). One patient had a self‐limited course and recovered spontaneously in 10 months. Three children had a monophasic course, improved with steroids and had little or no residual disability. The remaining three with the longest disease duration before diagnosis (&gt;2 years) improved with steroids but showed some residual distal weakness and atrophy and two developed foot deformities. Three children had at least one relapse at steroid tapering or discontinuation. Because of therapeutic implication the possibility of a CIDP occurring in childhood should always be kept in mind. An extensive electrophysiological study searching for indicators of non‐uniform nerve conduction abnormalities may suggest the diagnosis in most cases. When the diagnosis is doubtful and genetic studies are negative we recommend a therapeutic trial of steroids for at least four weeks.