Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represents an important acquired condition characterized by progressive, symmetrical, proximal and distal weakness. CIDP is characterized by sensory loss and weakness, areflexia, elevated CSF protein and electrodiagnostic evidence of multifocal demyelination with or without superimposed axonal degeneration. Some reports are made that an antecedent illness in the weeks preceding the onset of symptoms such as upper respiratory syndrome or flu-like illness, gastrointestinal syndrome etc., but intestinal pseudoobstruction as the main clinical feature in CIDP is an uncommon finding. The clinical course is variable. The condition is responsive to immunosuppressive therapy, especially prednisone and plasma exchange. We report a case of intestinal pseudoobstruction secondary to CIDP diagnosed by clinical features, electrodiagnostic study and nerve biopsy pathology.
Axons ; Biopsy ; Demyelinating Diseases ; Intestinal Pseudo-Obstruction* ; Pathology ; Plasma Exchange ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Prednisone

Marchiafava-Bignami disease (MBD) is a fatal disorder characterized by demyelination of the corpus callosum. MRI, suggestive of corpus callosum demyelination with associated white matter involvement in both cerebral hemispheres, indicates a diagnosis of MBD. In this case, MR diffusion-weighted findings taken at an acute stage of MBD revealed lesions not only in the corpus callosum but also in the cerebral cortex. Lower apparent diffusion coefficient values of the corpus callosum and cortical lesions were associated with poor clinical outcome.
Middle Aged ; Male ; Humans ; Demyelinating Diseases/*pathology ; Corpus Callosum/*pathology ; Brain/*pathology ; Alcoholism/complications

BACKGROUND: Diabetic neuropathy is one of the most common neuropathies. Although pathologic studies show both segmental demyelination and axonal loss in diabetic neuropathy, the relative importance of segmental demyelination is debated. Conduction block (CB) is a physiologic hallmark of segmental demyelination. If segmental demyelination were a main pathology of diabetic neuropathy, CB should be common. We undertook this study to determine the prevalence of CB in diabetic patients. METHODS: Fifty-two consecutive diabetic patients (M=30, F=22) were referred to EMG laboratory and underwent routine nerve conduction studies (NCS). CB was defined by two methods. One was > 20% drop in peak-to-peak amplitude and < 15% change in negative-peak duration between proximal and distal stimulation sites. The other was > 50% drop in the amplitude and area. Clinical findings, electrophysiological data, and effectiveness of immunomodulating therapy for some patients with CB were reviewed. RESULTS: A total 326 nerves were studied. The criteria for 20% and 50% CB were met in 35 nerves in 19 patients and 7 nerves in 6 patients, respectively (prevalence=10.7%, 2.1%, respectively). Some patients with CB were treated with IVIG or steroid and had a good response. CONCLUSIONS: CB in diabetic neuropathy is not a common finding. The rarity of CB in diabetic neuropathy suggests that segmental demyelination is not a prominent part of the underlying pathology. The presence of CB and good responsiveness to immunomodulating therapy in diabetic neuropathy also suggest alternative or additional causes for neuropathy, such as chronic inflammatory demyelinating polyneuropathy.
Axons ; Demyelinating Diseases* ; Diabetes Mellitus ; Diabetic Neuropathies* ; Humans ; Immunoglobulins, Intravenous ; Neural Conduction ; Pathology ; Polyneuropathies ; Prevalence

Due to unknown underlying biochemical disorders, the delineation of Dejerine-sottas disease has been subject to recent controversy. This is a case of a 9 year-old Korean female with the clinical manifestations of sporadic occurence, chronic severe and symmetrical motor sensory polyneuropathy, thickened palpable peripheral nerves, facial diplegia, areflexia and abnormal pupillary reactivity to light. The electrophysiological studies are indicative of chronic demyelination neuropathy showing markedly slowed motor NCV, low and dispersed CMAPs and extreme dispersion of a SNAP. The pathology of the sural nerve reveals prominant hypomyelination and onion bulbs characterized by whorling concentric proliferations of the cytoplasmic processes of Schwann cells. The nosological problems of hypertrophic neuropathy in childhood are discussed.
Axons/pathology ; Case Report ; Child ; Demyelinating Diseases/pathology ; Facial Paralysis/*pathology ; Female ; Hereditary Motor and Sensory Neuropathies/*pathology ; Human ; Sural Nerve/pathology

Balo's concentric sclerosis is regarded as a rare variant of multiple sclerosis. Traditionally, Balo's concentric sclerosis was a post-mortem diagnosis, but the recent introduction of brain magnetic resonance imaging (MRI) scans may allow noninvasive access without biopsy. Brain MRI findings of Balo's concentric sclerosis is characteristic concentric configuration of alternating bands of white matter of different pathology, with relatively preserved myelination alternating with regions of demyelination in the cerebral white matter. We report a case of Balo's concentric sclerosis with recurrent optic neuritis.
Biopsy ; Brain ; Demyelinating Diseases ; Diagnosis ; Diffuse Cerebral Sclerosis of Schilder* ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Myelin Sheath ; Optic Neuritis* ; Pathology

BACKGROUND: Guillain-Barre syndrome (GBS) is subclassified into acute inflammatory demyelinating polyneuropathy, acute motor or motor-sensory axonal neuropathy, and the other variants. While most studies from Western countries report demyelinating type as predominant form of GBS, some reports from Asian countries suggest the opposite result. Because they are not easily discriminated clinically, electrophysiological study is the most practical method for their subclassification. METHODS: Among the patients who had been admitted to our hospital with typical clinical features of GBS over the past 4 years, we analyzed 32 patients, who had undergone at least 2 serial nerve conduction studies (NCS) throughout the course. In each case, conventional NCS in at least 3 limbs were performed and the criteria of Hadden et al., and 2 other criteria were applied. RESULTS: Using the criteria of Hadden et al., 91% of the patients were classified as primary demyelination, and none as primary axonal pathology. Among the cases of primary demyelination, 21% showed electrophysiological features of axonal GBS at least in one occasion during the serial studies. CONCLUSIONS: Our study suggests that the primary demyelinating type is by far more predominant in our population. Secondary changes of electrophysiological findings in some of our cases suggest that primary demyelination could be misinterpreted as primary axonal pathology without timely serial studies. For the epidemiologic study of larger scale in conjunction with clinical features and immunologic investigations, the protocol and criteria of consensus is necessary, with the qualified normal limit values of each laboratory.
Asian Continental Ancestry Group ; Axons ; Classification* ; Consensus ; Demyelinating Diseases ; Epidemiologic Studies ; Extremities ; Guillain-Barre Syndrome* ; Humans ; Neural Conduction ; Pathology

White matter damage, characterized by demyelination due to the damage of oligodendrocyte precursor cells (OPCs), is the most common type of brain damage in preterm infants. Survivors are often subject to long-term neurodevelopmental sequelae because of the lack of effective treatment. In recent years, it has been found that cell transplantation has the potential for the treatment of white matter damage. OPCs are frequently used cells in cell transplantation therapy. With abilities of migration and myelinization, OPCs are the best seed cells for the treatment of white matter damage. Several studies have found that OPCs may not only replace impaired cells to reconstruct the structure and function of white matter, but also inhibit neuronal apoptosis, promote the proliferation of endogenous neural stem cells, and enhance the repairment of the blood-brain barrier. However, the clinical application of OPC transplantation therapy faces many challenges, such as the effectiveness, risk of tumorigenesis and immune rejection. With reference to these studies, this article reviewed the development of myelination, the obtainment of OPCs, the therapeutic mechanism as well as application research, and analyzed the current challenges of OPC transplantation, in order to provide a new direction for clinical treatment of white matter damage in preterm infants.
Cell Separation ; Demyelinating Diseases ; therapy ; Humans ; Infant, Newborn ; Infant, Premature ; Oligodendrocyte Precursor Cells ; transplantation ; White Matter ; pathology

We reviewed dinical, histological and ultrastructural findings of 124 cases of sural nerve biopsy specimens to delineate the trends of peripheral nerve diseases in our institute. Eighty-one were men and 43 were women. We categorized them into five groups: specific diagnosis (66 cases, 53.2%), axonal degeneration type (47 cases, 37.9%), demyelinating type (4 cases, 3.2%), mixed axonal degeneration-demyelinating type (6 cases, 4.8%) and normal (1 case, 0.9%). Cases with specific diagnosis included 21 inflammatory demyelinating polyneuropathy (15 chronic inflammatory demyelinating polyradiculoneuropathy, 6 Guillain-Barre disease), 13 hereditary motor and sensory neuropathy (7 Charcot-Marie-Tooth type I, 6 Charcot-Marie-Tooth type II), 10 vasculitis, 6 toxic neuropathy, 4 leprosy, 3 diabetic neuropathy, 2 alcoholic neuropathy, 1 Fabry's disease and other specific diseases (5 cases). In our cases, the proportion of specific diagnoses was higher, while the proportion of demyelinating peripheral neuropathies and normal were lower than those of Western series. The results of this study indicate that 1) a dose clinicopathologic correlation is important to make a precise diagnosis of peripheral nerve biopsy, 2) Biopsy under strict indication may reduce unnecessary histologic examination, 3) There is no difference in disease pattern of peripheral neuropathy between Western people and Koreans.
Adult ; Biopsy ; Charcot-Marie-Tooth Disease/pathology ; Demyelinating Diseases/pathology ; Fabry Disease/pathology ; Female ; Hereditary Motor and Sensory Neuropathies/pathology ; Human ; Korea ; Leprosy/pathology ; Male ; Microscopy, Electron ; Nerve Fibers, Myelinated/pathology ; Peripheral Nerves/ultrastructure ; Peripheral Nerves/pathology ; Peripheral Nervous System Diseases/pathology* ; Peripheral Nervous System Diseases/microbiology ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/pathology ; Sural Nerve/ultrastructure ; Sural Nerve/pathology*

Brain ; pathology ; Demyelinating Diseases ; complications ; diagnosis ; pathology ; Encephalomyelitis ; complications ; diagnosis ; pathology ; Fatal Outcome ; Humans ; Leukoencephalitis, Acute Hemorrhagic ; complications ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Male ; Young Adult

OBJECTIVETo explore the possibility of peripheral nerve damage induced by antiepileptic drugs (AEDs) in different age rats and its pathogenesis.

METHODSAdult (2-month-old) and infant (7-day-old) rats were divided into 8 groups (n = 16 in each) and treated with the following 7 AEDs respectively: phenytoin [PHT, 62.5 mg/(kgxd)], phenobarbital [PB, 30.0 mg/(kgxd)], sodium valproate [VPA, 312.5 mg/(kgxd)], clonazepam [CZP, 1.25 mg /(kgxd)], carbamazepine [CBZ, 187.5 mg/(kgxd)], topiramate [TPM, 40 mg/(kgxd)], oxcarbazepine [OXC, 312.5 mg/(kgxd)], remaining one group was used as control. Four weeks later, 8 rats were sacrificed randomly from each group and serum, sciatic nerves and spinal cord samples were collected. The rest half rats were sacrificed 4 week after AEDs withdrawal. Histological observations were performed on the sciatic nerves samples, including teased fibers, semi-thin sections and electron microscopy. The activity of total antioxidant capacity (T-AOC), superoxide dismutase (SOD) and glutathione peroxidase (GSH-PX) in serum and sciatic nerves were detected respectively. Expression of apoptosis-related proteins Bcl-2 and Bax was detected by immunohistochemistry. Neurons apoptosis in the anterior horns of spinal cord were detected by TUNEL.

RESULTS(1) Except for TPM group, various incidence (7.2% - 20.2%) of teased fibers abnormalities were observed in all the other different age groups. PHT group showed the most serious changes followed by PB (adult) or VPA (infant), CBZ, CZP and OXC groups. The predominant abnormality of teased fibers was demyelination. (2) There was no significant difference in the incidence of pathologic changes in teased fibers between adult and infant groups. Four weeks after AEDs withdrawal, recovery of pathologic changes in teased fibers in infant groups was much better than adult. (3) Significantly increased expression of Bax protein and ratio of Bax/Bcl-2 was only found in infant rats treated with PB, CNP or VPA compared with control (P < 0.05), the results of TUNEL was in accordance with immunohistochemistry. (4) Compared with control, the activity of T-AOC and SOD decreased in both infant and adult rats treated with PHT, CZP, CBZ and OXC, and the reduction of SOD activity in serum and sciatic nerves samples was also found in PB groups. Serum activity of GSH-PX was decreased in both age groups treated with PHT, PB, VPA, CZP, CBZ and OXC. The reduction of GSH-PX activity in sciatic nerves samples was remarkably in both adult and infant rats treated with PHT, PB, CBZ, OXC as well as the infant rats treated with CZP.

CONCLUSIONSSix AEDs (PHT, PB, CBZ, VPA, CZP, OXC) showed the potential to cause peripheral nerves damage. Demyelination was the predominant pathologic change. Both adult and infant rats had the same susceptibility. Recovery of pathologic changes in teased fibers in both age groups was slow, but infant rats were prone to revive more quickly. There was no significant correlation between spinal cord neuron apoptosis and peripheral nerves damages in rats treated with AEDs. Breakdown of oxidation-antioxidation balance was closely related to development of peripheral nerves damages caused by most AEDs.

Animals ; Anticonvulsants ; adverse effects ; Demyelinating Diseases ; chemically induced ; pathology ; Oxidative Stress ; Peripheral Nerves ; drug effects ; Rats ; Rats, Sprague-Dawley ; Sciatic Nerve ; pathology ; Spinal Cord ; pathology