1/61. Familial guillain-barre syndrome.There are few reports of guillain-barre syndrome (GBS) occurring in families. We have encountered a mother, who developed acute inflammatory demyelinating polyradiculoneuropathy at age 35 years, whose son developed the bulbar form of GBS 7 years later. Both shared HLA DR2.- - - - - - - - - - ranking = 1keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/61. Lumbosacral acute demyelinating polyneuropathy following hepatitis b vaccination.We report a patient who presented with an acute inflammatory demyelinating polyneuropathy, that followed the second injection of a hepatitis b vaccination, and characterized by motor and sensory deficit restricted to lower limbs and perineum, and persistent bladder dysfunction. The relationship between the preceding event and neurological disease is discussed.- - - - - - - - - - ranking = 0.54850163157609keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/61. Acute sensory neuropathy in an adolescent girl following BCG vaccination.A 13-year-old girl developed a sensory neuropathy following bacille Calmette-Guerin (BCG) vaccination, consistent with acute inflammatory demyelinating polyradiculoneuropathy or acute sensory axonal neuropathy.- - - - - - - - - - ranking = 1keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
4/61. filtration of cerebrospinal fluid for acute demyelinating neuropathy in systemic lupus erythematosus.We report a patient with systemic lupus erythematosus complicated by an acute demyelinating neuropathy. Conventional therapy with intravenous immunoglobulins and immunoadsorption complemented by pulse methylprednisolone and cyclophosphamide failed. Institution of filtration of the cerebrospinal fluid was followed by a rapid improvement of the paresis.- - - - - - - - - - ranking = 0.10020780056919keywords = demyelinating (Clic here for more details about this article) |
5/61. guillain-barre syndrome occurring in two women after ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus.We report two women who presented with a guillain-barre syndrome just after a ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus. One had characteristic clinical signs and the other had major motor involvement. At neurophysiologic investigations, one had typical demyelinating neuropathy whereas the second had mainly axonal degeneration. At ultrastructural examination of a peripheral nerve biopsy, features of macrophage-associated demyelination were present in both nerve specimens, thus confirming the diagnosis of acute inflammatory demyelinating polyneuropathy, i.e., guillain-barre syndrome. Prominent axonal involvement was also present in the motor nerves of the second patient. insulin therapy had to be permanently continued and these two cases are quite different from the transient diabetes sometimes observed in certain cases of guillain-barre syndrome. Both the latter and insulin-dependent diabetes mellitus probably have auto-immune mechanisms. It is likely that in our two patients both auto-immune diseases were triggered by a common event. Such cases of guillain-barre syndrome have to be distinguished from other acute diabetic neuropathies.- - - - - - - - - - ranking = 0.48837695123457keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/61. Acute axonal form of guillain-barre syndrome in a multiple sclerosis patient: chance association or linked disorders?multiple sclerosis (MS) is characterized by inflammation, demyelination and gliosis, involving the central nervous system (CNS) and commonly sparing the peripheral nervous system (PNS). Coexistence of CNS and PNS chronic demyelination has been rarely demonstrated in chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) and in MS, but the occurrence of acute polyradiculoneuropathy in a patient with MS is even more unusual. We describe the case of a woman with relapsing-remitting MS who presented with an acute severe tetraparesis. cerebrospinal fluid (CSF) examination together with neurophysiological data and sural nerve biopsy study demonstrated an axonal form of guillain-barre syndrome (GBS). It remains unresolved if the association of an axonal form of GBS and MS is fortuitous or, on the contrary, is indicative of the coexistence in some individuals of common pathogenetic mechanisms.- - - - - - - - - - ranking = 1.1809185695898keywords = polyradiculoneuropathy, inflammatory demyelinating, chronic inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/61. Event-related potentials in patients with total locked-in state due to fulminant guillain-barre syndrome.A series of electrophysiological investigations were performed over a 6-month period in two patients affected by fulminant Guillain-Barre polyradiculoneuropathy, who developed an ascending paralysis leading, within 72 h, to flaccid quadriplegia, internal and external ophthalmoplegia, absence of all brainstem reflexes and no respiratory effort: the clinical state resembled brain death. brain CTs were normal and spinal fluid examination revealed albuminocytological dissociation. All motor nerves tested were unexcitable, whereas sensory responses were markedly abnormal but present. Sequential EEG recordings revealed normal, partially reactive alpha rhythm in both patients. In one patient, normal auditory event-related potentials (ERPs: peak N1, P2, N2, P3, evoked in an 'oddball' paradigm) and CNV-like potentials could be recorded not earlier than the 20th day into the illness. In earlier recordings, N1 and P2 peaks as well as mismatch negativity (MMN) were present over the frontal and central scalp electrodes. This patient has now partially recovered motor functions and no cognitive defects are present, but he has little recollection of the events occurring in the first 2 weeks spent in the ICU, when he was completely paralyzed. The other patient generated normal N1 and P2 ERP peaks, but no N2, P3 and MMN were detected in a series of recordings. He died without having ever regained appropriate behavioral responses. The ERP abnormalities observed raise the matter of the origin of cognitive dysfunction in patients with severe and prolonged de-efferentation/de-afferentation. ERPs allow monitoring the level of alertness and attention and appear more specific than EEG in identifying a state of awareness in patients in which communication is severely impaired as a consequence of neurological disorders.- - - - - - - - - - ranking = 0.53166460887926keywords = polyradiculoneuropathy (Clic here for more details about this article) |
8/61. Inflammatory demyelinating polyradiculoneuropathy associated with interstitial lung disease.A 58-year-old woman presented with inflammatory demyelinating polyradiculoneuropathy accompanied by sensory and motor disturbance and interstitial lung disease. Corticosteroid therapy led to a marked amelioration of both the neuropathy and the lung disease. We suggest that a demyelinating neuropathy is associated with an interstitial lung disease.- - - - - - - - - - ranking = 3.2268662360862keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
9/61. guillain-barre syndrome: perspectives with infants and children.An acute flaccid paraparesis or ascending quadriparesis in an infant or child constitutes a very important pediatric neurology emergency. The guillain-barre syndrome (GBS) is the most frequent cause. This is primarily an autoimmune, post-infectious, demyelinating, peripheral nervous system process. A small percentage of children develop a primary axonal process not unlike that identified more commonly in china. Because of the potential for acute respiratory compromise, any child suspected of having GBS needs immediate hospitalization. The major considerations in differential diagnosis include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis. On occasion, some younger children present with an acute severe pain syndrome that may mask as a pseudo-encephalopathy. Another clinical variant is the Miller-Fisher syndrome characterized by ataxia, ophthalmoparesis, and areflexia. This is associated with a high frequency of the anti-GQ-1-b antibodies. Although most children with GBS have a relatively benign clinical course, some become very ill and require intubation with intensive care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk. To date, no well-controlled study has been completed analyzing the relative merits of the two most commonly used therapies, namely plasmapheresis or intravenously administered immunoglobulin.- - - - - - - - - - ranking = 0.020041560113838keywords = demyelinating (Clic here for more details about this article) |
10/61. pregnancy, anaesthesia and Guillain Barre syndrome.Two case histories of pregnant women with Guillain Barre syndrome (acute demyelinating polyradiculoneuritis) are reported. The first required anaesthesia during the second trimester for a minor surgical procedure. The second woman was admitted to the intensive care Unit in the first trimester and was ventilated for 18 weeks. Both babies were carried to term and delivered by Caesarean section. A review of the management of Guillain Barre syndrome in pregnancy discusses anaesthetic management, intensive care and the use of plasmapheresis and gamma-globulins. The care of pregnant women recovered from Guillain Barre syndrome is also discussed.- - - - - - - - - - ranking = 0.020041560113838keywords = demyelinating (Clic here for more details about this article) |
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