1/41. Neurogenic stunned myocardium in guillain-barre syndrome.Neurogenic stunned myocardium (NSM), a syndrome of reversible left ventricular dysfunction best described after subarachnoid hemorrhage, has not been associated with peripheral neuropathy. We describe a woman with guillain-barre syndrome in whom a syndrome compatible with NSM developed in the setting of a physiologically documented increase in sympathetic cardiovascular tone. This case supports the presumed unifying role of excessive sympathetic nervous system activation in the pathogenesis of NSM.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/41. Transient absence of F-waves in acute myelopathy: a potential source of diagnostic error.BACKGROUND: The frequent absence of F-waves in lesions of the nerve roots and proximal nerve is well known, with absence of F-waves occasionally the only electrophysiologic manifestation of early guillain-barre syndrome. It is less well known that acute central nervous system lesions can cause disappearance of F-waves. CASE DESCRIPTION: A 25 year old woman presented with quadriparesis and sensory loss progressive over several days. Hyporeflexia and hypotonia were present. Imaging studies were initially negative. Electrophysiologic testing was normal apart from the diffuse absence of F-waves. This led to strong consideration of the diagnosis of guillain-barre syndrome, and treatment for this diagnosis. However, imaging studies ultimately revealed the diagnosis to be transverse myelitis. F responses normalized 6 weeks after the initial study. CONCLUSIONS: F responses are significantly modulated by central nervous system factors. The relevant experimental and clinical literature is reviewed. The relevance of this to the diagnosis of guillain-barre syndrome has not been previously emphasized, but our experience confirms that the absence of F-waves in a patient with acute weakness accompanied by hyporeflexia and hypotonia does not distinguish between peripheral nerve and central nervous system lesions.- - - - - - - - - - ranking = 4.5024922170287keywords = nervous system, central nervous system (Clic here for more details about this article) |
3/41. guillain-barre syndrome in a patient with non-Hodgkin's lymphoma.We describe a case of guillain-barre syndrome (GBS) in a patient with non-Hodgkin's lymphoma (NHL). A 21-year-old woman with a newly diagnosed stage IV high-grade lymphoma (precursor T-cell NHL according to the R.E.A.L. classification) developed flaccid quadriparesis and bilateral facial diplegia after three weeks of treatment with vincristine, daunorubicin, L-asparaginase and prednisolone. The clinical course and neurological examination were consistent with GBS. Despite treatment with intravenous immunoglobulins her neurological symptoms progressed. plasmapheresis was therefore initiated followed by intravenous immunoglobulins. After partial remission of neurologic symptoms, induction chemotherapy with cyclophosphamide and cytarabine was continued without any further complication. Three months later, the lymphoma was in complete remission. GBS has been described in Hodgkin's disease and after bone marrow transplantation but is rare in NHL. In patients with NHL who develop neurological symptoms, drug toxicity and nervous system infiltration are the leading cause of neuropathology, but GBS should be considered in the differential diagnosis.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/41. 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 = 2.5008307390096keywords = nervous system, central nervous system (Clic here for more details about this article) |
5/41. 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 = 0.15917415184264keywords = brain (Clic here for more details about this article) |
6/41. 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 = 1keywords = nervous system (Clic here for more details about this article) |
7/41. Fulminant guillain-barre syndrome mimicking cerebral death: case report and literature review.A 45-year-old woman was admitted to the intensive care unit (ICU) for respiratory arrest. One day prior to admission, she had been nauseated and in a state of total exhaustion. On the night of admission she was unresponsive and developed gasping respiration. The patient was comatose with absent brainstem reflexes and appeared brain dead. blood chemistry findings and brain magnetic resonance imaging were normal. Electroencephalogram revealed an alpha rhythmical activity unresponsive to painful or visual stimuli. The cerebrospinal fluid showed an albuminocytological dissociation. guillain-barre syndrome (GBS) was suspected. The electrophysiological evaluation revealed an inexcitability of all nerves. The pathological findings of the sural nerve biopsy indicated an axonal degeneration secondary to severe demyelination. GBS can very rarely present with coma and absent brainstem reflexes. This case illustrates the importance of electrophysiological tests and laboratory and imaging studies in patients with suspected brain death where a cause is not clearly determined.- - - - - - - - - - ranking = 0.3979353796066keywords = brain (Clic here for more details about this article) |
8/41. A case of central nervous system vasculitis related to an episode of guillain-barre syndrome.The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune guillain-barre syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next month after her discharge to a rehabilitation centre.- - - - - - - - - - ranking = 6.0033229560383keywords = nervous system, central nervous system (Clic here for more details about this article) |
9/41. Hyperreflexia in a patient with motor axonal guillain-barre syndrome.We report a patient who presented after an episode of diarrhoea with ascending, symmetrical weakness without sensory loss, and without sphincter or other autonomic dysfunction. On clinical examination there were no cranial nerve deficits. Hyperreflexia of tendon jerks without other upper motor neurone signs was found. Electrophysiological examination demonstrated acute distal symmetrical motor axonal polyneuropathy. No electrophysiological signs of peripheral nerve demyelination or central nervous system involvement were found. Albuminocytologic dissociation was present in the cerebrospinal fluid. Stool culture and serological tests were inconclusive. Our patient's clinical picture was, apart from hyperreflexia of tendon jerks throughout the disease, characteristic of guillain-barre syndrome. This is the first such patient reported in europe. The aetiology remained unclear. We suggest that selective axonal motor fibre affection, with possible mild pyramidal involvement, caused tendon jerk hyperreflexia.- - - - - - - - - - ranking = 1.5008307390096keywords = nervous system, central nervous system (Clic here for more details about this article) |
10/41. Overlapping guillain-barre syndrome and Bickerstaff's brainstem encephalitis associated with anti-GQ1b IgG antibody after herpes simplex virus infection.herpes simplex virus (HSV) is a rare, antecedent infectious agent in guillain-barre syndrome (GBS). We report a patient with overlapping GBS and Bickerstaff's brainstem encephalitis (BBE). The patient had a vesicular lesion on her nose. Antecedent HSV type 1 (HSV-1) infection was confirmed by isolation of the virus and detection of the presence of serum anti-HSV-1 IgM antibody during the acute phase. Her serum IgG had high anti-GQ1b antibody titer. External ophthalmoplegia has been noted in 2 of 4 reported cases of HSV-associated GBS. Herpetic brainstem encephalitis cases of poor prognosis are known, but only 2 cases of benign brainstem encephalitis secondary to HSV infection, in which there was acute ophthalmoplegia and clinical features consistent with those of BBE have been reported.- - - - - - - - - - ranking = 0.55710953144924keywords = brain (Clic here for more details about this article) |
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