Onset and course of chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is clinically heterogeneous. Our purpose was to determine whether initial progression time, clinical features, and distribution of nerve conduction slowing at presentation correlate with clinical course and prognosis. We examined how findings at presentation related to clinical course during an average follow-up time of 4.0 (range 1.0-9.0) years in 44 patients with CIDP. We calculated terminal latency index (TLI), a measure of differential slowing in distal relative to more proximal nerve segments. Patients with acute or subacute onset (progression over less than 8 weeks) had a higher remission rate (P = 0.012) than patients with chronic onset (progression over more than 8 weeks). Patients with proximal weakness had a higher remission rate than patients with the distal phenotype (P < 0.001). All 5 patients with a relapsing course had subacute onset. They had lower TLIs, suggesting a more distal pattern of demyelination, than patients with a monophasic or chronic course. In conclusion, subacute onset and presence of proximal weakness are good prognostic signs that correlate with a high rate of recovery to normal in CIDP. Distal accentuation of conduction slowing at presentation correlates with subacute onset and a relapsing course.     

Multifocal acquired demyelinating sensory and motor neuropathy: report of a case and review of the literature

Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory loss, and conduction block and other features of demyelination in nerve conduction studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In classic CIDP, there are symmetric proximal and distal weakness, sensory deficit in both upper and lower extremities and reduced deep tendon reflex. In MMN, limb weakness without sensory loss is asymmetric in the distribution of individual peripheral nerves and the weakness typically begins in the distal upper extremities. We report one patient with chronic progression of asymmetric numbness and weakness in four extremities. MADSAM neuropathy was diagnosed after extensive clinical and laboratory evaluations. It is very important to distinguish between CIDP, MADSAM neuropathy, and MMN by clinical, laboratory, and histological features because of different effective therapeutic strategies.     

Motor chronic inflammatory demyelinating polyneuropathy (CIDP) in 17 patients: Clinical characteristics,electrophysiological study,and response to treatment

Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM‐CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred‐CIDP) if ≥2 sensory nerve action potential amplitudes were abnormal. Among the 700 patients with CIDP, 17 (2%) were included (PM‐CIDP n = 7, MPred‐CIDP n = 10); 71% were male, median age at onset was 48 years (range: 13‐76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three‐quarters (75%) responded to intravenous immunoglobulins (IVIg) and four of five patients to corticosteroids including three of three patients with MPred‐CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F‐wave abnormalities (88%). During follow up, 4 of 10 MPred‐CIDP patients developed mild sensory symptoms; none with PM‐CIDP did so. Patients with PM‐CIDP had poorer outcome (median ONLS: 4; range: 22‐5) compared to MPred‐CIDP (2, range: 0‐4; P = .03) at last follow up. This study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F‐wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred‐CIDP.     

Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide clinical range, and is characterised by multifocal demyelination that can involve the distal nerve terminals, intermediate nerve segments, and nerve roots. OBJECTIVE: To investigate whether the distribution patterns of demyelination along the course of the nerve correlate with clinical profiles in patients with CIDP. METHODS: Motor nerve conduction studies were carried out on 42 consecutive patients. According to the physiological criteria for demyelination, the presence of a demyelinative lesion was determined in the distal nerve segments (distal pattern) or intermediate nerve segments (intermediate pattern), or in both (diffuse pattern). The serum concentration of tumour necrosis factor (TNF)-alpha was measured by immunoassay. RESULTS: Patients were classified as having a distal (n=10), intermediate (n=13), or diffuse (n=15) pattern, or were unclassified (n=4). Patients with the distal or diffuse pattern had common clinical features such as subacute onset, symmetric symptoms, and weakness involving proximal as well as distal muscles. Patients with the distal pattern had a good response to treatment and a monophasic remitting course, but the diffuse pattern was associated with a treatment dependent relapsing course, reflecting longer disease activity. The serum TNF-alpha concentrations increased only in the "diffuse" subgroup of patients, and this might be associated with breakdown of the blood-nerve barrier and therefore, involvement of the intermediate segments. The intermediate pattern was characterised by a chronic course, asymmetric symptoms, less severe disability, and refractoriness to treatments. CONCLUSIONS: CIDP consists of subtypes with varying predilections for lesions along the course of the nerve. The distribution patterns of conduction abnormalities may be useful in the prediction of outcome of patients with CIDP.     

Sensory CIDP presenting as cryptogenic sensory polyneuropathy

The objective of this study was to report that patients with chronic inflammatory demyelinating polyneuropathy (CIDP) can present with a clinical picture of cryptogenic sensory neuropathy. Patients with distal sensory neuropathy and electrodiagnostic studies that are minimally abnormal or consistent with an axonal pathology are usually diagnosed as having cryptogenic sensory neuropathy if no cause for neuropathy can be found. Some of these patients, however, may have sensory CIDP. We reviewed the records of eight patients with CIDP, diagnosed by sural nerve biopsy, who presented with sensory neuropathy and electrodiagnostic studies that were minimally abnormal or revealed changes consistent with axonal neuropathy. All patients reported distal numbness and paresthesias and, on examination, had predominantly large fiber distal sensory loss and normal muscle strength. In most patients, deep tendon reflexes were reduced or absent. Sural nerve biopsies in all patients were consistent with chronic myelinopathy, with quantitative teased fiber analysis revealing segmental remyelination in 13-40% of the fibers. The four patients who received IVIg therapy had improved sensation and gait. Of the remaining four patients, one is being followed, one had spontaneous remission, one was lost to follow-up, and one, with contraindications to therapy, reported disease progression. Sensory CIDP may present as cryptogenic sensory polyneuropathy with normal or axonal electrophysiologic features. Sural nerve biopsy should be considered in patients with progressive, predominantly large fiber sensory neuropathy of otherwise unknown etiology, as they may have sensory CIDP that responds to therapy.     

New trends in neuropathy practice: clinical approach to CIDP]

Our recent study showed that the overall prevalence of CIDP was estimated as 2.2 per 100,000 population in Aomori Prefecture, in Northan Honshu of Japan. In our series of more than 80 cases with CIDP, a chronic acquired inflammatory demyelinating polyneuropathy, nearly 30% showed clear laterality of weakness, and electrophysiologic laterality or multifocality was apparent in almost all cases. Nearly 90% of patients were able to walk without walking aids or other assistance. Sixty% showed distal dominant muscular weakness. In 12 patients with age of onset under 15, pes cavus deformity was seen in 5. Two thirds complained numbness in the extremities during progressive phase. Four cases initially showed severe sensory ataxia associated with motor conduction block. It should be, thus, reminded that clinical spectrum of CIDP is enormously wide: chronic acquired demyelinating multiple mononeuropathy showing asymmetric involvement (Lewis-Summer syndrome) should be put on one side of the clinical presentation of CIDP. Multifocal motor neuropathy (MMN) is, on the other hand, an unique syndrome mimicking amyotrophic lateral sclerosis (ALS). There may be, however, true association syndrome of CIDP and ALS presenting both peripheral nerve demyelination and pyramidal sign with progressive bulbar involvement. Recently, several atypical varieties of CIDP showing only one-limb involvement, upper limb weakness rather than lower limb power loss, or proximal weakness, etc ... have been reported in the literature. To realize such clinical variations of chronic acquired demyelinating neuropathy is important for early diagnosis and commencement of treatment of CIDP. Clinical guideline for suspicion of CIDP could be useful for general physicians and neurologists unfamiliar to peripheral neuropathies.     

Acute-onset painful upper limb multifocal demyelinating motor neuropathy

We report three patients who presented with acute onset of shoulder and upper arm pain followed within a few days by predominantly distal upper limb weakness. Nerve conduction studies showed severe and unequivocal focal motor conduction block in the forearm and/or upper arm along with slowing of motor conduction and prolonged F wave responses. Only very mild changes in sensory nerve conduction were found. One patient made partial clinical improvement after 17 months, and there was a significant improvement in the degree of motor conduction block and the motor conduction velocities. A second patient remained unchanged after 5 months. Idiopathic brachial neuritis (IBN) typically presents acutely with brachialgia and acute or subacute non-progressive weakness. Multifocal motor conduction block in nerves in the arm or forearm has not been described in patients with IBN. Multifocal motor conduction block restricted to the upper limbs has been described in focal chronic inflammatory demyelinating polyneuropathy (CIDP) and in multifocal motor neuropathy with multifocal motor conduction block (MMNCB). However, both these conditions have hitherto usually been described as largely painless chronic progressive disorders with a subacute onset. Our patients, with features overlapping MMNCB/CIDP and IBN, represent an as yet unreported clinical variant. Received: 9 February 2000 / Received in revised form: 18 May 2000 / Accepted: 28 June 2000     

Intravenous immunoglobulin as first treatment in diabetics with concomitant distal symmetric axonal polyneuropathy and CIDP

The authors investigated the impact of IVIg as first line treatment of diabetic patients suffering from chronic inflammatory demyelinating polyneuropathy (CIDP) concomitant with distal symmetric axonal polyneuropathy. Nine patients with these clinical and electrophysiological features were treated with IVIg (0.4 g/Kg/day for 5 days). Clinical and electrophysiological evaluations were performed before and after treatment. Following IVIg treatment there was no significant improvement in clinical deficit. However, there was a significant and persistent decrease in the Rankin scale score and an improvement in the demyelinating feature on nerve conduction studies. Our findings suggest that IVIg had small but detectable beneficial effects on diabetic patients with CIDP and a high degree of axonal damage. Received: 10 August 2001, Received in revised form: 5 November 2001, Accepted: 7 November 2001     

Long-term effects of intravenous immunoglobulin in CIDP.

OBJECTIVE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating disease of the peripheral nervous system characterized by muscle weakness, areflexia or hyporeflexia, and sensory disturbances. Although short-term efficacy of intravenous immunoglobulin (IVIg) has been demonstrated in randomized-controlled trials, the data pertaining to long-term outcome in CIDP are limited. Consequently, the aim of the present study was to assess the long-term effects of IVIg on neurophysiological parameters in CIDP. METHODS: Neurophysiological records from 11 CIDP patients, treated with IVIg for 12 months, were reviewed. Nerve conduction studies were assessed at baseline, 1-year, and last follow-up. RESULTS: There was a significant reduction in the frequency of conduction blocks (pre-treatment nerve segments affected 61%; last follow-up 39%, P<0.01) and a reduction in ongoing axonal loss (pre-treatment regions with spontaneous activity, 47%; post-treatment 29%, P<0.01) with IVIg treatment. Further, there was significant improvement in sensory nerve conduction studies with IVIg treatment (sensory amplitudes reduced pre-treatment, 90% nerves tested; post-treatment, 62%, P<0.01). CONCLUSIONS: The present study suggests that long-term IVIg maintenance therapy improves neurophysiological parameters in CIDP. However, CIDP patients remain IVIg dependent and new conduction blocks may develop. SIGNIFICANCE: The present study suggests that long-term IVIg maintenance therapy improves neurophysiological parameters in CIDP, possibly by reducing the immune response and thereby fostering nerve healing.     

Two cases with chronic inflammatory demyelinating polyneuropathy showing high signal and enlargement of the brachial plexus in short TI inversion recovery of MRI]

We reported valuable MRI findings of the brachial plexus seen in two cases with chronic inflammatory demyelinating polyneuropathy (CIDP). Case 1 was a 44-year-old man who developed slowly progressive weakness and atrophy of the extremities with no sensory disturbances. Studies of CSF showed a normal level of protein and no increase of cell counts but nerve conduction studies demonstrated a significant conduction block between the axilla and the elbow in the right ulnar nerve. Case 2 was a 34-year-old male who had been suffering from distal limb weakness and sensory disturbance. Protein content in CSF was markedly elevated without pleocytosis, and nerve conduction studies revealed a conduction block between the elbow and the wrist in the right ulnar nerve. He received corticosteroid therapy, resulting in a good recovery. Brachial plexus in both cases showed enlargement with marked high signal on short TI inversion recovery (STIR) of MRI. STIR is a fat suppressed T2 weighted image and this technique is known to be useful to identify the morphology of peripheral nerve tissues. CIDP is one form of hypertrophic neuritis and the MRI findings seen in these two cases strongly support the diagnosis of CIDP.     

CIDP: DESCRIPTION OF FOUR PARTICULAR CASES

Chronic inflammatory demyelinating polineuropathy (CIDP) is an acquired polineuropathy, clinically heterogeneous, and is associated with demyelination of spinal roots and peripheral nerves with a monophasic, progressive or relapsing course.
We describe four patients affected by CIDP with different clinical pictures.
The first patient, a 54-year-old man, complained of a 10 year paresthesia, with a slight wasting and weakening of the right leg, where he showed the absence of deep tendon reflexes and a reduction of the vibration sense.
The second patient, a 21-year-old woman, had distal paresthesia and mild weakness restricted to the left hand. Deep tendon reflexes were reduced and vibration sense was normal.
The third patient, a 41-year-old man, with a 5 year history of muscle cramps more frequent in the upper limbs, showed mild weakness of the right forearm, absence of distal deep tendon reflexes, and a reduction of vibration sense in the lower limbs.
The last patient, a 20-year-old man, showed a marked increase of CPK level, a severe and progressive proximal and distal hypotrophy in the upper and lower limbs, and respiratory distress. Deep tendon reflexes were absent.
Nerve conduction studies showed a reduction of sensory and motor velocity, which was not homogeneous among the patients and among the explored nerves of each patients. Proximal motor conduction blocks were present only in three cases. CSF parameters were normal and antibodies anti nerve were absent in all patients.
The first three patients were treated with IVIg with a clear clinical and electrophysiological improvement, the last patient showed a moderate improvement, only after cyclosporin A treatment .
These four cases confirm that CIDP is clinically heterogeneous with a variable spectrum of symptoms and severity, from a minimal clinical evidence to a severe clinical picture as in our fourth patient.     

Treatment with interferon-alpha 2a in a patient with chronic inflammatory demyelinating polyneuropathy]

We report an adult patient with refractory chronic inflammatory demyelinating polyneuropathy (CIDP) who resisted conventional immunological therapies but could be successfully treated with interferon (IFN)-alpha 2a. A 56-year-old man was admitted to our hospital with progressive distal dominant muscle weakness and sensory disturbance of all extremities. He was diagnosed as CIDP based on laboratory examinations which revealed conduction block on peripheral nerve conduction studies and demyelinating findings on sural nerve biopsy. He was given several immunological treatments, including immunoadsorption therapy (IAT), oral corticosteroid, immunosuppressive agents such as cyclophosphamide and mizoribine, but without satisfactory efficacy although transient improvement of muscle weakness was observed for two weeks after IAT. A session of IAT every four weeks was therefore continued to maintain his symptoms for nine months. He was then treated with IFN-alpha 2a intramuscular injections every other day. Four weeks after starting of IFN-alpha 2a therapy, the worsening of his symptoms stopped and his muscle strength improved gradually to normal level without IAT, suggesting that IFN-alpha 2a may be effective in some patients with refractory CIDP. Further studies are needed to confirm the efficacy of IFN-alpha 2a for CIDP.     

Minimal and asymptomatic chronic inflammatory demyelinating polyneuropathy.

OBJECTIVES: Show the chronic inflammatory demyelinating polyneuropathy (CIDP) is not only clinically heterogeneous but extremely variable in severity. METHODS: Three patients were referred for mild distal paresthesiae lasting more than 6 months and one for inguinal and thigh pain later ascribed to coxarthrosis. Strength was normal in all patients and tactile sensation reduced distally only in one. Tendon jerks were absent, except the knee jerks in one patient, reduced in lower limbs in two and normal in one. RESULTS: Electrophysiology showed a demyelinating neuropathy without motor conduction block. CSF protein content was increased in all patients. Nerve biopsies showed de-remyelination with varying degrees of axonal loss. Genetic studies excluded a demyelinating neuropathy associated with duplication or deletion of the 17p.11.2 segment. CONCLUSIONS: CIDP patients with pure sensory clinical presentation have been described but are generally more severely impaired. However, because of the mildness of symptoms and the unequivocal electrophysiological involvement of motor fibers, we think that in these cases the term minimal CIDP is more appropriate than sensory CIDP. These cases represent the most benign end of the CIDP spectrum. In our series minimal or even asymptomatic CIDP encompasses 8% of cases.     

The spectrum of chronic inflammatory demyelinating polyneuropathy

Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee 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. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, 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.     

Diagnostic utility of somatosensory evoked potentials in chronic polyradiculopathy without electrodiagnostic signs of peripheral demyelination

Introduction: Diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) remains uncertain when nerve conduction studies (NCS) fail to show demyelination. Methods: We conducted a retrospective study of patients who presented with clinical criteria of CIDP in whom electrodiagnostic (EDx) criteria of definite or probable CIDP were missing [axonal sensorimotor neuropathy (n = 23), normal EDx with pure sensory presentation (n = 3)]. All patients received immunomodulatory treatment. Twenty‐six patients were evaluated with somatosensory evoked potentials (SSEPs), MRI of spinal roots, cerebrospinal fluid analysis, and/or nerve biopsy. Diagnosis of CIDP was considered to be confirmed in patients who responded to immunotherapy. Results: Twenty‐two of 26 patients (85%) had SSEPs reflecting abnormal proximal conduction in sensory fibers, including 14 who had only clinical and SSEP data in favor of CIDP. SSEPs were abnormal in 16 of 20 responders (80%) to immunotherapy. Conclusion: SSEP recording contributes to the diagnosis of CIDP when nerve conduction studies fail to detect peripheral demyelination. Muscle Nerve 53 : 78–83, 2016     

A Case of Cauda Equina Syndrome in Early-Onset Chronic Inflammatory Demyelinating Polyneuropathy Clinically Similar to Charcot-Marie-Tooth Disease Type 1

To present a case of cauda equina syndrome (CES) caused by chronic inflammatory demyelinating polyneuropathy (CIDP) which seemed clinically similar to Charcot-Marie-Tooth disease type1 (CMT1). CIDP is an immune-mediated polyneuropathy, either progressive or relapsing-remitting. It is a non-hereditary disorder characterized by symmetrical motor and sensory deficits. Rarely, spinal nerve roots can be involved, leading to CES by hypertrophic cauda equina. A 34-year-old man presented with low back pain, radicular pain, bilateral lower-extremity weakness, urinary incontinence, and constipation. He had had musculoskeletal deformities, such as hammertoes and pes cavus, since age 10. Lumbar spine magnetic resonance imaging showed diffuse thickening of the cauda equina. Electrophysiological testing showed increased distal latency, conduction blocks, temporal dispersion, and severe nerve conduction velocity slowing (3 m/s). We were not able to find genetic mutations at the PMP 22, MPZ, PRX, and EGR2 genes. The pathologic findings of the sural nerve biopsy revealed thinly myelinated nerve fibers with Schwann cells proliferation. We performed a decompressive laminectomy, intravenous IgG (IV-IgG) and oral steroid. At 1 week after surgery, most of his symptoms showed marked improvements except foot deformities. There was no relapse or aggravation of disease for 3 years. We diagnosed the case as an early-onset CIDP with cauda equine syndrome, whose initial clinical findings were similar to those of CMT1, and successfully managed with decompressive laminectomy, IV-IgG and oral steroid.     

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> 50%) in at least one nerve in 5/7 children, 3)> 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 (>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.     

Diagnosis and management of immune-mediated neuropathies

Immune-mediate neuropathies, or inflammatory neuropathies are neuropathies due to the dysregulation of the immune system. The injury to peripheral nerves can be divided into two phases: an early stage of immune injury, and a later stage of structural damage. The overall effects are axonal degeneration or demyelination depending on the target of immunological attacks. According to time course, there are two major types: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Clinical manifestations of both diseases include progressive motor weakness and sensory disturbance with some variations among different patients. The major findings of nerve conduction studies on GBS patients are prolonged distal motor latencies and minimal F-wave latencies with variable reduction of nerve conduction velocities. In CIDP patients, slowed nerve conduction velocities are the usual findings in addition to prolongation of distal motor latencies and minimal F-wave latencies. Certain subtypes of immune-mediated neuropathies are associated with high titers of anti-gangliosdie antibodies. Patients with GBS and CIDP can benefit from immunotherapy. For GBS, plasma exchange and intravenous immunoglobulin (IVIG) are equally effective in reducing complications and neurological disability. Steroid of high dose is, however, harmful to GBS. Plasma exchange and IVIG can alleviate neurological deficits of CIDP with steroid to maintain the effects of plasma exchange and IVIG. In conclusion, careful clinical observations and judgment are the most important issue to manage patients with immune-mediated neuropathies.     

Chronic polyradiculoneuritis. 25 cases]

Twenty-five patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were studied in order to define the clinical, biological, electrophysiological and pathological features of this disease. There were 11 men and 14 women ranging in age from 15 to 82 years. The average follow-up was 43 months. Patients fulfilled the criteria laid down by Dyck et al. (1975), except that progression of weakness was at least 2 months and not 6. Fourteen patients had a progressive course and 11 a relapsing one. Weakness was almost constant (24/25), sensory impairment was present in 22/25 with deep sensation predominantly impaired. Areflexia was observed in all patients. A history of previous infection or other possible precipitating event was given by 7 patients. Cerebrospinal fluid examination showed a raised total protein count in 22 cases. Electrophysiological examination revealed a slowing down of nerve conduction velocities to an extent compatible with a demyelinating process in 23 cases; 2 patients only had prolongation of F-waves. Sural nerve biopsy was less informative: inflammatory process--the most specific finding--was observed in only 3 out of 20 biopsies. Six patients had benign forms or spontaneous remission, the others were treated with corticosteroids or with immunosuppressive drugs. Most patients (64%) recovered very well. Only one died during the time of study. Eight other patients were treated with high-dose intravenous immunoglobulins and 4 of them improved with administrations at regular intervals to maintain the benefits observed. The occurrence of CIDP in association with other conditions is reviewed and we discuss its nosological position among the acquired demyelinating neuropathies.     

An autopsy case of chronic inflammatory demyelinating polyradiculoneuropathy with respiratory failure

An 85-year-old Japanese woman presented with progressive symmetrical proximal and distal muscle weakness, numbness in the distal extremities, and sudden onset of hemifacial weakness. The results of laboratory studies fulfilled the American Academy of Neurology criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). She died of respiratory failure after two courses of intravenous immunoglobulin treatment, each resulting in transient improvement in strength and sensory symptoms. Autopsy revealed multifocal demyelinative or axonal lesions of varying severity affecting the cranial and peripheral nerves and including the phrenic nerve. These findings suggest that the clinical phenotype of CIDP depend on distribution and severity of the anatomical lesions.