Electrophysiological changes in the acute ?axonal? form of Guillain-Barre syndrome (original) (raw)
Related papers
Inexcitability of nerves in a fulminant case of Guillain-Barre syndrome
Journal of the Peripheral Nervous System, 2000
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-Barré 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.
Journal of the Peripheral Nervous System, 2009
We describe a clinicopathological study of a patient presenting with severe and electrophysiological axonal Guillain-Barré syndrome (GBS). An 83-year-old man had a 2-day history of distal acroparesthesias and ascending weakness culminating in quadriplegia, the patient dying 1 month after onset. On day 3, motor conduction velocity (MCV) and distal motor latency values were normal or minimally delayed; most F waves were present with latencies normal or barely delayed. Compound muscle action potential (CMAP) amplitudes were variably reduced. On day 10, there was reduction of CMAPs with relative preservation of MCV. On histological study, the density of myelinated fibers was normal in L5 ventral and dorsal roots, where outstanding lesions included dark fibers, scattered macrophage infiltration, and occasional images of de-remyelination or axonal degeneration. In the fifth spinal nerve, there was widespread loss of myelinated fibers with focal areas showing almost complete fiber loss and variable fascicular combination of extensive de-remyelination and axonal degeneration. Wallerian-like degeneration predominated in femoral and sciatic nerves. Peripheral neuron cell bodies showed central chromatolysis. We conclude that the pathological hallmark of this electrophysiological axonal GBS case is extensive but variable de-remyelination of proximal nerve trunks with superimposed nerve ischemia and axonal degeneration.
Changes in motor nerve excitability in acute phase Guillain‐Barré syndrome
Muscle & Nerve
Background: The most common subtypes of Guillain-Barré syndrome (GBS) are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). In the first days after the onset of weakness, standard nerve conduction studies (NCS) may not distinguish GBS subtypes. Reduced nerve excitability may be an early symptom of nerve dysfunction, which can be determined with the compound muscle action potential (CMAP) scan. The aim of this study was to explore whether early changes in motor nerve excitability in GBS patients are related to various subtypes. Methods: Prospective case-control study in 19 GBS patients from The Netherlands and 22 from Bangladesh. CMAP scans were performed within 2 days of hospital admission and NCS 7-14 days after onset of weakness. CMAP scans were also performed in age-and country-matched controls. Results: CMAP scan patterns of patients who were classified as AMAN were distinctly different compared to the CMAP scan patterns of the patients who were classified as AIDP. The most pronounced differences were found in the stimulus intensity parameters. Conclusions: CMAP scans made at hospital admission demonstrate several characteristics that can be used as an early indicator of GBS subtype.
Guillain-Barré syndrome. A clinical electrophysiological and biochemical study
Acta Neurologica Scandinavica, 1986
Fifty-six consecutive patients with Guillain-BarrC syndrome representing 49% of all cases of peripheral neuropathy (except those due to diabetes mellitus and leprosy) admitted to the Postgraduate Institute of Medical Education and Research, Chandigarh over a period of three years were studied. All patients developed weakness of limbs within one day to three weeks. Attenuation of deep tendon jerks (98%) paresthesia (66%), cranial nerve involvement (41 To) and antecedent infection (32%) were the common clinical features. The common patterns of motor weakness were predominantly proximal in all the four limbs (45%) or predominantly proximal in lower limbs along with distal muscles in upper limbs (29%). Electrodiagnostic studies revealed prolonged distal (motor) latency (82%), reduced motor nerve conduction velocity (740/0), sensory nerve conduction abnormality (85%) and evidence of denervation (41%). Evoked motor response of median, ulnar, common peroneal and tibia1 nerves exhibited significant increase in duration and reduction in amplitude. The maximum incidence of electrophysiological abnormality occurred between four to 12 weeks after the onset of neurological symptoms. Four patients died and 11 showed poor recovery. Long intervals (> 3 weeks) between peak deficit and onset of recovery and coexistence of reduced motor nerve conduction velocity with evidence of denervation on EMG were found to be associated with poor recovery.
Electrophysiological findings in early Guillain-Barré syndrome
Acta clinica Croatica, 2011
The aim of the study was to identify the most common electrophysiological abnormalities in early Guillain-Barré syndrome (GBS). Neurophysiological data on 51 GBS patients assessed within 12 days of symptom onset were reviewed. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) was present in 46 of 51 GBS patients. The following abnormalities were observed in our AIDP patients: absent H reflex in 90.7%, conduction block in the Erb-to-axilla segment in 78.6%, motor conduction velocity suggestive of demyelination in the Erb-to-axilla segment in 45.2%, prolonged F wave latency in 65.2%-73.8% of patients but only 20.0%-37.0% with prolonged F wave latency suggestive of demyelination, and reduced or absent sensory nerve action potential in 62% of patients. Abnormal values of terminal latencies, and motor and sensory conduction velocities in distal nerve segments suggestive of demyelination were recorded in less than 30% of patients. In conclusion, the most sensitive parameter i...
Early nodal changes in the acute motor axonal neuropathy pattern of the Guillain-Barré syndrome
Journal of Neurocytology, 1996
The axonal patterns of Guillain-Barr6 syndrome, associated in many cases with antecedent Campylobacter jejuni infection, are now recognized as frequent causes of acute flaccid paralysis in some regions of the world. This study examined ultrastructurally the PNS of seven cases of the acute motor axonal neuropathy form of Guillain-Barr6 syndrome. In this disorder previous studies of advanced cases have found Wallerian-like degeneration of motor fibres in the spinal roots and peripheral nerves, with little lymphocytic inflammation or demyelination. The present study was focused on identifying early changes and establishing the sequence of changes. By electron microscopy the earliest and mildest changes consisted of lengthening of the node of Ranvier with distortion of the paranodal myelin, and in some instances with breakdown of the outermost myelin terminal loops. At this stage many nodes had overlying macrophages which extended their processes through the Schwann cell basal lamina covering the node and apposed the axolemma. Macrophage processes then extended beneath the myelin terminal loops, and the whole macrophage entered the periaxonal space at the paranode. Macrophage processes dissected the axon from the adaxonal Schwann cell plasmalemma and the macrophages advanced into the internodal periaxonal space, where they typically surrounded a condensed-appearing axon. At this stage the adaxonal Schwann cell cytoplasm regularly degenerated and disappeared, so that the periaxonal space was bounded by the innermost myelin lamella, and the axolemma of many fibres could not be seen. The internodal myelin sheath and the abaxonal Schwann cell cytoplasm remained normal. This arrangement appeared to be stable for some time, but in many fibres the axon subsequently underwent Wallerian-like degeneration. By interfering with impulse conduction, these nodal and periaxonal changes may explain paralysis in some pathologically mild cases. In addition, at early stages, these changes may be reversible, thus explaining the rapid recovery of some patients who become paralysed with acute motor axonal neuropathy. These observations, taken together with previous studies, suggest that acute motor axonal neuropathy is an antibody-and complement-mediated disorder in which the relevant epitopes are present on the nodal and internodal axolemma. neuropathy (AMAN) (Goldstein et al.
Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy
Frontiers in neurology, 2017
Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy with acute onset and rapid clinical worsening; early diagnosis and immunomodulating therapy can ameliorate the course of disease. During the first days, however, nerve conduction studies (NCSs) are not always conclusive. Here, we describe a 73-year-old man presenting with progressive muscular weakness of the lower limbs, ascending to the upper limbs, accompanied by distal sensory disturbances. Neuroimaging of brain and spine and NCSs were unremarkable; cerebrospinal fluid analysis revealed no albuminocytologic dissociation. Based on typical clinical features, and on positivity for serum GD1b-IgM antibodies, GBS with proximal conduction failure at multiple radicular levels was postulated, and a standard regime of intravenous immunoglobulin was administered. Four weeks later, the patient presented with flaccid tetraparesis, areflexia, and reduction of position sense, tingling paresthesias, and initial respirato...
Electrodiagnostic and Clinical Aspects of Guillain-Barré Syndrome: An Analysis of 142 Cases
Journal of Clinical Neuromuscular Disease, 2008
Background: The incidence of Guillain-Barré syndrome (GBS) and its subtypes varies throughout the world. Objective and Methods: We present a retrospective analysis of 142 GBS cases, treated at our center, aimed at classifying GBS electrophysiologically, to study the sequential electrophysiological changes in cases with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), and to look for any clinical and cerebrospinal fluid parameters that can also help in distinguishing the subtypes. Results: One hundred twenty-one (85.2%) cases had AIDP, 15 (10.6%) had acute motor axonal neuropathy, and 6 (4.2%) were unclassifiable. Conclusions: Motor conduction blocks and temporal dispersion could be observed from days 3 and 5 onward, respectively. Progression of motor conduction slowing in AIDP was most impressive in the median nerves. Varying affection of deep tendon reflexes, cranial nerves, and cerebrospinal fluid albuminocytological dissociation can also help make a distinction between AIDP and acute motor axonal neuropathy. Sural sparing, a marker of demyelinating neuropathy, is more commonly seen in later than in early stages of AIDP.
Nerve Conduction Studies In Guillian Barre’ Syndrome
2013
BACKGROUND: NCS are objective methods for diagnosis, quantification and classification of poly neuropathies. Electrophysiology is most important to confirm GBS in all its forms. Aim: To study early characteristics of NCS in GBS. Research design: Cross sectional, analytical Material & Methods: NCS performed on 49 GBS participants were retrospectively analyzed. Different parameters motor, sensory, late waves studied & compared with literature. Statistics: Descriptive statistics, ANOVA, correlations. Results: Age range 1-82 yrs. 76% males, AIDP form (93.88%) predominates, CMAP median bilaterally moderately associated with muscle grades p value 0.005 & 0.006, CMAP & F disturbance severe and predominant feature. 41.86 % H- reflex un recordable. 34.69% showed sensory abnormalities. Age group "d 3 years showed similar pattern. 3 children showed in excitability. Abnormality in number of variables of nerves in combination is likely pattern. Conclusions: Early NCS pattern emerged similar...