1/56. Primary polyarteritis nodosa presenting as acute symmetric quadriplegia.We report a case of peripheral neuropathy presenting as acute symmetric areflexic quadriplegia in the setting of a well-defined clinical, histopathologic, and angiographic diagnosis of classic polyarteritis nodosa. While it is usually easy to recognize the typical clinical presentation of necrotizing angiopathy-induced peripheral neuropathy as a mononeuritis multiplex or a distal polyneuropathy in association with a collagen vascular disease, clinicians must be equally sensitive to a number of more challenging possibilities. Acute quadriplegia similar to that seen in guillain-barre syndrome can be secondary to primary classic polyarteritis nodosa and the former may be the chief or even the sole manifestation of the latter.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
2/56. Reversible tetraplegia due to polyneuropathy in a diabetic patient with hyperosmolar non-ketotic coma.critical illness polyneuromypathy has not previously been reported as a complication of diabetic coma. We describe a patient with hyperosmolar non-ketotic coma (HONK) complicating gram-negative sepsis in whom persistent coma and profound tetraplegia caused considerable concern. Although, initially, it was feared that the patient had suffered a central neurological complication such as stroke or cerebral oedema, a diagnosis of critical illness motor syndrome (CIMS) was subsequently confirmed neurophysiologically. Profound limb weakness associated with HONK is not necessarily due to a catastrophic cerebral event, rather it may be a result of CIMS, which has an excellent prognosis for full neurological recovery.- - - - - - - - - - ranking = 1.3333333333333keywords = neuropathy (Clic here for more details about this article) |
3/56. 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 = 0.33333333333333keywords = neuropathy (Clic here for more details about this article) |
4/56. Flaccid quadriplegia from tonsillar herniation in pneumococcal meningitis.A young woman with fulminant pyogenic meningitis became quadriplegic, areflexic and flaccid due to herniation of the cerebellar tonsils and compression of the upper cervical cord. This state of spinal shock was associated with absent F-waves. intracranial pressure was greatly elevated and there was an uncertain relationship of tonsillar descent to a preceding lumbar puncture. Partial recovery occurred over 2 years. Tonsillar herniation can cause flaccid quadriplegia that may be mistaken for critical illness polyneuropathy. This case demonstrates cervicomedullary infarction from compression, a mechanism that is more likely than the sometimes proposed infectious vasculitis of the upper cord.- - - - - - - - - - ranking = 0.33333333333333keywords = neuropathy (Clic here for more details about this article) |
5/56. Progressive infantile axonal polyneuropathy.polyneuropathies are relatively uncommon in early infancy and the majority of affected children are found to have hypomyelinating neuropathies. Axonal sensorimotor neuropathies have been described in childhood but the majority of affected children present at or after 6 months of age, have nonprogressive courses, and achieve the ability to walk, albeit late. Here we present three infants with infantile progressive axonal polyneuropathy from two families with nonconsanguineous parents. Each child presented shortly after the neonatal period and with rapid progression to quadriplegia. Involvement of the lower cranial nerves, phrenic nerves, or both was present in each child. electrophysiology was diagnostic in each child. While the diagnosis of spinal muscular atrophy was considered in each case, clinical presentation, biopsies, and genetic testing were inconsistent with this diagnosis. Recognition of this early form of progressive axonal neuropathy is important as respiratory compromise occurred early and the condition showed familial inheritance in two of our patients.- - - - - - - - - - ranking = 2keywords = neuropathy (Clic here for more details about this article) |
6/56. Fulminant peripheral neuropathy with severe quadriparesis associated with vincristine therapy.OBJECTIVE: To report a case of fulminant neuropathy with severe quadriparesis associated with vincristine chemotherapy. CASE SUMMARY: A 48-year-old white man with acute lymphoblastic leukemia was started on an induction chemotherapeutic regimen that included intravenous vincristine. He received a total of 6 mg of vincristine over two weeks during induction chemotherapy. Over the next two weeks, he developed a fulminant peripheral neuropathy with severe quadriparesis. DISCUSSION: Although commonly associated with peripheral neuropathy, vincristine neurotoxicity only rarely involves instances of fulminant peripheral neuropathy with severe quadriparesis. guillain-barre syndrome is also associated with leukemia and may present as a fulminant peripheral neuropathy with severe quadriparesis. CONCLUSIONS: Fulminant neuropathy with severe quadriparesis occurring in patients with leukemia being treated with vincristine (and who do not have coexistent charcot-marie-tooth disease) is more likely due to guillain-barre syndrome than to vincristine neurotoxicity.- - - - - - - - - - ranking = 3.3333333333333keywords = neuropathy (Clic here for more details about this article) |
7/56. Pathogenesis and recovery of tetraplegia after electrical injury.The site of neurological damage causing paralysis after electrical trauma remains to be clarified. A patient is described who developed a flaccid tetraplegia after a high voltage electrical injury. The findings on initial examination and neurophysiological investigation showed a very severe generalised sensory-motor polyneuropathy. His subsequent follow up over 60 months showed a remarkable degree of reinnervation and the unmasking of a myelopathy. The degree of reinnervation noted suggests an axonopathy that left the other elements of the peripheral nerves relatively spared. These findings provide the most convincing evidence to date that a generalised polyneuropathy can follow electrical injury and that it results from non-thermal mechanisms such as electroporation.- - - - - - - - - - ranking = 0.66666666666667keywords = neuropathy (Clic here for more details about this article) |
8/56. Refractory heterotopic ossification with complications.BACKGROUND: Heterotopic ossification (HO) following spinal cord injury can lead to various complications, including venous thrombosis, autonomic dysreflexia, and pressure ulcers. We report refractory, complicated HO in a 19-year-old man with C8 incomplete tetraplegia. He first presented at 9 weeks postinjury with fever and swelling of his right leg. Ultrasound indicated a deep venous thrombosis (DVT). Persistent symptoms prompted triple-phase bone scan and magnetic resonance imaging (MRI), which revealed HO compressing the right external iliac vein and no evidence of DVT. The HO was complicated by hypercoagulability. CLINICAL COURSE: The HO was refractory to oral indomethacin and etidronate; therefore, intravenous etidronate was instituted, resulting in only a transient decrease in alkaline phosphatase. Local irradiation of the right hip did not decrease the activity of HO. The patient was discharged on oral etidronate, indomethacin, and warfarin. This complicated case raises issues regarding early diagnosis and aggressive treatment of HO, as well as treatment of associated hypercoagulability.- - - - - - - - - - ranking = 0.00020509272771491keywords = deep (Clic here for more details about this article) |
9/56. Severe axonal polyneuropathy after a FK506 overdosage in a lung transplant recipient.FK506-induced polyneuropathies are rarely encountered. We report a case of axonal sensorimotor polyneuropathy in a lung transplant recipient that occurred during a FK506 overdosage. Onset was acute in the form of severe areflexic tetraparesis and resolution was observed after reduction of dosage. Because of increasing use of FK506 in solid organ transplantation, caution should be paid with FK506 dosage monitoring in cases of peripheral nervous system symptoms.- - - - - - - - - - ranking = 1.6666666666667keywords = neuropathy (Clic here for more details about this article) |
10/56. Motor dominant neuropathy in sjogren's syndrome: report of two cases.Most of the peripheral neuropathies in sjogren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of guillain-barre syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating polyradiculoneuropathy. These patients received i.v. immunoglobulin therapy. To our knowledge, this is the first report indicating that i.v. immunoglobulin has beneficial effects on motor dominant neuropathy in SS.- - - - - - - - - - ranking = 2.6666666666667keywords = neuropathy (Clic here for more details about this article) |
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