1/64. AAEM case report 4: guillain-barre syndrome. American association of Electrodiagnostic medicine.A 57-year-old woman developed rapidly progressive, symmetric, extremity weakness, facial diplegia, ophthalmoplegia, respiratory insufficiency, and sensory ataxia over a 3-week period. Electrodiagnostic studies were performed on days 6, 13, and 50 following the onset of weakness. Motor nerve conduction abnormalities were the predominant findings. Prolonged motor distal latencies, prolonged or absent F waves, and partial motor conduction blocks were present and form the diagnostic features of an acquired, demyelinating polyneuropathy. Abnormalities in sensory nerve conductions and blink reflexes were also present. guillain-barre syndrome was diagnosed prompting the initiation of therapeutic plasma exchange. The patient's clinical status continued to worsen over the next 10 days before stabilizing. Considerable improvement in extremity strength, ocular motility, and respiratory function occurred in the subsequent weeks. Well-planned and well-executed electrodiagnostic studies generate key adjunctive data to the clinical diagnosis of guillain-barre syndrome.- - - - - - - - - - ranking = 1keywords = ophthalmoplegia, ataxia (Clic here for more details about this article) |
2/64. polyradiculopathy in sarcoidosis.We present three new and 14 retrospective cases of polyradiculopathy in sarcoidosis. Of these, 71% had weakness and 59% areflexia of the lower extremities, and 35% had sphincter dysfunction. Cases often were associated with central nervous system sarcoidosis. All cases involved thoracolumbar or lumbosacral roots, except a single case of cervical polyradiculopathy. Of 14 treated patients, nine improved with corticosteroids, laminectomy, or both. polyradiculopathy complicating sarcoidosis: (1) is uncommon; (2) primarily involves thoracic and lumbar roots; (3) may arise from contiguous, hematogenous, or gravitational nerve root sleeve seeding; (4) may be asymptomatic; and (5) may improve with corticosteroids. Differential diagnosis of weakness in patients with sarcoidosis should include nerve root involvement from the primary process by direct sarcoid involvement.- - - - - - - - - - ranking = 5.344542093392keywords = areflexia (Clic here for more details about this article) |
3/64. pain and the guillain-barre syndrome in children under 6 years old.During a 15-year period, 29 children, under the age of 6 years, with acute guillain-barre syndrome were seen at our institution. A review of their charts revealed that pain was a symptom in all patients and was present on admission in 79% of cases. pain was often the most important symptom and led to misdiagnosis in 20 patients (69%). In 11 of these children, symptoms were present for more than a week before the correct diagnosis was made. The most common pain syndrome was back and lower limb pain, present in 83% of patients. Pediatricians should consider guillain-barre syndrome in their differential diagnosis when faced with a child who has lower limb pain and areflexia.- - - - - - - - - - ranking = 5.344542093392keywords = areflexia (Clic here for more details about this article) |
4/64. Bilateral ballism in a patient with overlapping Fisher's and Guillain-Barre syndromes.A 29 year old woman developed diplopia and ataxic gait. Neurological examination showed total ophthalmoplegia, cerebellar ataxia, and areflexia. Moreover, there was muscle weakness in all four limbs. An overlap of Fisher's and Guillain-Barre syndromes was dignosed. On day 5 she suddenly developed involuntary flinging movements that affected the face and four limbs. Surface EMG showed 1.5-2 Hz rhythmic grouping discharges. The involuntary movements were considered ballism. This is the first report of a patient with guillain-barre syndrome and a related disorder who showed ballism.- - - - - - - - - - ranking = 6.344542093392keywords = areflexia, ophthalmoplegia, ataxia (Clic here for more details about this article) |
5/64. Polyneuritis cranialis with contrast enhancement of cranial nerves on magnetic resonance imaging.The disorder of multiple cranial nerve palsies without spinal cord involvement is referred to as polyneuritis cranialis (PC) and is rare. It is thought to be an acute post-infective polyneuropathy or a variant of guillain-barre syndrome. Electrophysiological evidence of demyelination has been reported, but no radiological abnormalities of the affected cranial nerves have been noted. We report a case of PC where contrast enhanced magnetic resonance imaging (MRI) showed enhancement of the peripheral segments of the oculomotor and abducens nerves. This case illustrates the utility of MRI in the assessment of cranial nerve palsies.- - - - - - - - - - ranking = 0.0065175223003735keywords = variant (Clic here for more details about this article) |
6/64. Polyneuritis cranialis: clinical and electrophysiological findings.A 13 year old boy, developed bilateral facial weakness, dysphonia and dysphagia acutely after a febrile illness. Neurological examination and MRI of the brain were normal. The CSF protein level increased. Blink reflex monitoring during clinical recovery was consistent with demyelination of the lower cranial nerves innervating the branchial arch musculature, a rare variant of guillain-barre syndrome.- - - - - - - - - - ranking = 0.0065175223003735keywords = variant (Clic here for more details about this article) |
7/64. Recurrent guillain-barre syndrome. Clinical and laboratory features.The clinical and laboratory features of recurrent guillain-barre syndrome (RGBS) were reviewed in 12 patients in whom a total of 32 episodes fulfilled accepted criteria for guillain-barre syndrome (GBS). All patients were asymptomatic or only mildly symptomatic between attacks. In a given patient, the time to reach peak deficit from the onset of symptoms, the functional grade at peak deficit and the duration of the intervals between episodes varied considerably and unpredictably from one episode to the next. Analysis of these parameters across the entire group revealed no significant change as the number of attacks increased. The distribution of weakness varied between episodes with the possible exception of features of the Miller Fisher variant which were more constant. tremor was noted in two patients and enlarged nerves in one patient. There was no evident response to immunosuppressive therapy. Results of cerebrospinal fluid (CSF) analysis and nerve conduction studies during recurrences were those expected in typical monophasic GBS. On nerve biopsy, onion bulb formations were sometimes observed after several recurrences. The following characteristics of RGBS may be sufficiently distinctive from those of chronic relapsing polyneuropathy to justify their nosological separation: rapid onset of symptoms with subsequent complete or near complete recovery, high incidence of an antecedent illness, lack of an apparent response to immunosuppressive therapy and normal CSF protein levels at the onset of a recurrence.- - - - - - - - - - ranking = 0.0065175223003735keywords = variant (Clic here for more details about this article) |
8/64. Motor nerve inexcitability in guillain-barre syndrome. The spectrum of distal conduction block and axonal degeneration.We studied 34 patients with the guillain-barre syndrome (GBS) to clarify the clinical significance of inexcitable motor nerves and of low amplitude compound muscle action potentials (CMAPs). The patients were subdivided into two groups. Group 1 included eight patients who had electrically inexcitable motor nerves within 2 wks of the first symptom. (Two patients without extensive conduction studies had only one inexcitable motor nerve.) The outcome in this group at 1 yr varied from complete recovery (five patients) to severe motor sequelae (three patients). Group 2 included 26 patients who had two electrophysiological assessments, and in whom the serial changes in CMAP amplitudes were analysed and correlated to outcome. Fourteen of these 26 sets of serial studies were performed within 1 mth. Twelve of 26 patients in Group 2 showed decrease in the amplitude of CMAPs between serial studies; only six of these had a good outcome at 1 yr. Nine of 26 patients showed increase in CMAP amplitude between serial studies, of these eight had a good clinical outcome. Low-amplitude CMAPs or inexcitable motor nerves in the initial stages of GBS are due to distal pathology of the motor axons, either distal conduction block or axonal degeneration. The nature of these changes cannot be predicted by the results of the initial electrophysiological evaluation, including the presence or absence of active denervation. However, improvement of CMAP amplitude on sequential studies suggests a good outcome at 1 yr. We believe that, in the absence of a biological marker for GBS, individualization of an 'axonal variant' of the syndrome is not warranted at the present time.- - - - - - - - - - ranking = 0.0065175223003735keywords = variant (Clic here for more details about this article) |
9/64. Upper airway dysfunction--an unusual presentation of guillain-barre syndrome.We report the case of a 9-year-old boy who presented to the emergency department with dysphagia, respiratory distress, hoarseness, and generalized weakness. While in the ED, his respiratory status deteriorated, and he required intubation. At that time, he was found to have normal upper airway anatomy. He was admitted to the pediatric ICU and, within 24 hours, developed areflexia and muscle weakness; he was diagnosed with guillain-barre syndrome. Atypical presentations of guillain-barre syndrome are discussed, and the necessity of considering this diagnosis when evaluating patients with evidence of cranial nerve dysfunction or upper airway distress is emphasized.- - - - - - - - - - ranking = 5.344542093392keywords = areflexia (Clic here for more details about this article) |
10/64. Subacute inflammatory demyelinating polyneuropathy in children.Reported are five children with subacute demyelinating polyneuropathy. All patients had a monophasic disease, progressing over 4 to 8 weeks and characterized by predominantly motor features, areflexia, minimal or no cranial nerve abnormalities, no autonomic or respiratory involvement, elevated CSF protein, electrophysiologic evidence of demyelination, and good response to corticosteroids. A benign course with full recovery was the rule.- - - - - - - - - - ranking = 5.344542093392keywords = areflexia (Clic here for more details about this article) |
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