1/72. Giant negative T waves in guillain-barre syndrome.A guillain-barre syndrome patient showed giant negative T waves on electrocardiography at the height of the disease, with large left ventricular hypokinesis on echocardiography and extensive defects on 123I-meta-iodobenzylguanidine myocardial scintigraphy. Gamma-globulin improved the neurological symptoms, and the above abnormalities resolved. We speculate that cardiac sympathetic nerve endings were transiently damaged, with consequent myocardial injury, due to norepinephrine toxicity.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/72. 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 = 3keywords = nerve (Clic here for more details about this article) |
3/72. 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 = 3keywords = nerve (Clic here for more details about this article) |
4/72. Cramping pain and prolonged elevation of serum creatine kinase levels in a patient with guillain-barre syndrome following campylobacter jejuni enteritis.We describe a patient with guillain-barre syndrome (GBS) following Campylobacter jejuni enteritis, accompanied with severe cramping pain and a marked increase in serum creatine kinase (CK) levels. Both conditions became evident three weeks after the onset of GBS and continued for longer than one month. In this patient, it is possible that rapid extensive denervation due to severe axonal degeneration of motor nerve terminals might have caused hyperexcitability in regional muscles, leading to recurrent muscle cramps and persistent release of muscular CK.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
5/72. 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 = 1keywords = nerve (Clic here for more details about this article) |
6/72. 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 = 1keywords = nerve (Clic here for more details about this article) |
7/72. guillain-barre syndrome following hand-foot-and-mouth disease.We describe a patient who developed guillain-barre syndrome (GBS) following hand-foot-and-mouth disease (HFMD) which is known to be caused by enterovirus infection. A 35-year-old man developed acute paraparesis and dysesthesia in the four limbs following typical skin eruption of HFMD. Electrophysiologic studies showed peripheral nerve demyelination predominant in the distal terminals. HFMD is a rare cause of meningitis, encephalitis, and polio-like myelitis, but GBS following HFMD has never been described.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/72. Inexcitability of nerves in a fulminant case of guillain-barre syndrome.A 45-year-old woman presented with a recent sensorimotor deficiency in all 4 limbs, and the next day she was totally paralyzed. A slight motor improvement began on day 27. The cerebrospinal fluid had normal cellularity, but the protein varied from 90 mg/dL on the first day to 800 mg/dL on day 15, and then 290 mg/dL on day 33. Electrophysiologic studies performed on days 15 and 23 revealed a universal peripheral nerve inexcitability. A superficial peroneal nerve biopsy was performed on day 23. Nine fascicles were examined on semi-thin sections and myelinated fiber damage varied greatly from one fascicle to another. At ultrastructural examination, certain axons were severely damaged, but the others were quite well preserved and were naked or wrapped in a myelin sheath presenting a multivesicular degeneration. A few fibers had a better-preserved myelin sheath that was sometimes dissociated by elongated processes from an invading histiocyte. Six cases of fulminant guillain-barre syndrome with inexcitability of nerves and ultrastructural examination of nerve fragments have been reported. Electrophysiologic study is often ambiguous and cannot determine the precise origin of such an axonal degeneration. Therefore, ultrastructural analysis of a nerve biopsy is mandatory in this setting.- - - - - - - - - - ranking = 9keywords = nerve (Clic here for more details about this article) |
9/72. Immune response to gangliosides in a case of guillain-barre syndrome after varicella.An 8 year old girl was admitted to hospital with the typical clinical features of guillain-barre syndrome (GBS) after recovering from varicella. Onset of the disease was just two weeks after the onset of varicella in her young sister. Examination of cerebrospinal fluid and nerve conduction studies showed typical findings of GBS. Although serum from both the patient and sister were analysed for autoantibodies to gangliosides and myelin p0 protein, IgM anti-GM1 antibody and anti-GD1b antibody were only detected in the patient. HLA DR haplotypes were quite different between the two subjects. This suggests that these autoantibodies may play an important role in the pathogenesis of GBS after varicella zoster virus infection.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/72. guillain-barre syndrome presenting with severe pain: report of one case.Most neurologists are familiar with pain in guillain-barre syndrome but some pediatricians may still miss or underestimate it. We report a 10-year-old girl who presented with headache and numbness in lower extremities. On examination, she could walk and showed hyporeflexia, mild muscle weakness, prominent neck rigidity and right facial nerve palsy. headache and pain in neck, low back and calves were so severe that she could not fall asleep and the response to analgesic was very poor. On 8th day of illness, she needed mechanical ventilation and suffered tetraplegia. Symptoms of autonomic dysfunction including hypertension and sinus tachycardia appeared thereafter and lasted for a week. After receiving a high dose of intravenous immunoglobulin (2 g/kg in 2 days), her condition improved gradually although joint pain persisted for about three weeks. She could walk unaided by day 40 and run by day 70, and recovered completely 6 months later. We emphasize that pain in GBS needs proper evaluation and management in children as well as in adults.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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