To identify the factors that could predict the functional outcome in patients with the axonal type of Guillain-Barre syndrome (GBS).
Two hundred and two GBS patients admitted to our university hospital between 2003 and 2014 were reviewed retrospectively. We defined a good outcome as being "able to walk independently at 1 month after onset" and a poor outcome as being "unable to walk independently at 1 month after onset". We evaluated the factors that differed between the good and poor outcome groups.
Twenty-four patients were classified into the acute motor axonal neuropathy type. There was a statistically significant difference between the good and poor outcome groups in terms of the GBS disability score at admission, and GBS disability score and Medical Research Council sum score at 1 month after admission. In an electrophysiologic analysis, the good outcome group showed greater amplitude of median, ulnar, deep peroneal, and posterior tibial nerve compound muscle action potentials (CMAP) and greater amplitude of median, ulnar, and superficial peroneal sensory nerve action potentials (SNAP) than the poor outcome group.
A lower GBS disability score at admission, high amplitude of median, ulnar, deep peroneal, and posterior tibial CMAPs, and high amplitude of median, ulnar, and superficial peroneal SNAPs were associated with being able to walk at 1 month in patients with axonal GBS.
Citations
Acute transverse myelitis (ATM) is an upper motor neuron disease of the spinal cord, and concomitant association of peripheral polyneuropathy, particularly the axonal type, is rarely reported in children. Our cases presented with ATM complicated with axonal type polyneuropathy. Axonal type polyneuropathy may be caused by acute motor-sensory axonal neuropathy (AMSAN) or critical illness polyneuropathy and myopathy (CIPNM). These cases emphasize the need for nerve and muscle biopsies to make the differential diagnosis between AMSAN and CIPNM in patients with ATM complicated with axonal polyneuropathy.
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Idiopathic CD4+ T-lymphocytopenia is a rare immune disorder characterized by an unexplained deficit of CD4+ T cells and results in various opportunistic infections. Herein, we report a case of new onset weakness in a 10-year-old boy secondary to motor axonal neuropathy associated with idiopathic CD4+ T-lymphocytopenia. The patient was referred to rehabilitation for an evaluation of progressive weakness involving all four limbs. A subsequent nerve conduction study and needle electromyography identified motor axonal neuropathy. At that time, laboratory studies specific to the differential diagnosis of motor axonal neuropathy were performed; however, the abnormality noted was a decreased CD4+ T-lymphocyte count. Motor axonal neuropathy represents an uncommon manifestation of idiopathic CD4+ T-lymphocytopenia and is probably associated with an underlying immune process.
Citations
Objective: To assess the axonopathy and demyelination neuropathy according to the electrophysiologic severity in diabetic neuropathy.
Method: Electrophysiologic data of 246 patients who had been diagnosed with diabetic neuropathy was obtained and classified into suspected, possible, and definite groups by the criteria of our laboratory. Nerve conduction study was performed in the median, ulnar motor and sensory nerves, peroneal and tibial motor nerves, and sural nerve. Statistics were done with the results from the median motor and sensory, tibial motor and sural nerves. According to the severity of diabetic neuropathy, correlation and Chi-square analysis between amplitudes and latencies were performed.
Results: Frequencies of diabetic neuropathy according to
electrophysiologic severity were as follows: 24 cases of suspected, 141 cases of possible, and 81 cases of definite neuropathy. The correlation ratios between amplitude and latency were 0.41∼0.79 (p＜0.05) in the definite group of all the nerves examined, and below 0.3 in the suspected and possible groups. By Chi-square analysis, amplitude reduction was the predominant finding in the suspected and possible groups.
Conclusion: In the early stage of diabetic neuropathy, axonopathy might be the preceding pathogenesis, while with progression of diabetic neuropathy, axonopathy and demyelination may coexist. (J Korean Acad Rehab Med 2002; 26: 50-54)
Guillain-Barre syndrme (GBS) has several subtypes that are divided by clinical, electro- physiological, and pathological findings. A novel form of GBS, that is termed acute motor axonal neuropathy (AMAN), is characterized by the selective involvement of motor fibers, and is associated with anti-GM1 antibodies.
A 8-year-old male patient were developed ascending, symmetrical paralysis, and areflexia, but no sensory disturbance. Elevated titers of serum IgG anti-GM1 antibodies were detected. His thoracolumbar spine magnetic resonance imaging (MRI) revealed thickening of cauda equina and enhancement of anterior nerve roots of T12-L1 spinal level after Gd-DTPA infusion. Electrophysiological diagnosis was acute motor axonal neuropathy (AMAN). We report this case with review of the literature.
The study of blink reflexes was carried out to demonstrate the correlations, if there were, between the stage of diffuse axonal injury(DAI) and the abnormality of blink reflexes. The blink reflex was recorded in 20 healthy adult subjects and 22 patients with DAI who were classified according to Adams' classification(DAI I; 7, DAI II; 9 and DAI III; 6). The latencies and amplitudes of R1 and R2 in patients with DAI were compared with those of healthy subjects.
The results were as follows; 1) In 20 subjects of patient group, the latencies of R1 were all within a normal range. In 2 subjects, the difference in latencies between the two sides was above 1.4 msec. 2) In 15 subjects, R2 was absent or delayed, and reduced in the size of amplitude in all. Nine were affected bilaterally, and 4 were unilaterally. 3) Seventy one percent of patients in each stage represented abnormal findings. 4) There were no correlations between the DAI stage and the blink reflex.
This study demonstrated that the polysynaptic R2 was more profoundly suppressed than the oligosynaptic R1 in a diffuse axonal injury because of a loss or decrease of suprapontine facillitation, which influenced the trigeminal spinal complex and the interneuron of lateral reticular formation.
We herein report a case of infantile neuroaxonal dystrophy(INAD) with protracted course. The 3 year old patient suffered from ataxia, gait disturbance, oculomotor disturbance, psychomotor regression and bilateral pyramidal tract signs since the age of two. Similar neurological symptoms occurred in his elder brother, beginning at the age of one, who eventually died at the age of four. Magnetic Resonance Imaging(MRI) of the patient showed progressive atrophy of cerebral cortex and cerebellum with diffusely increased T2 signal in bilateral cerebellar hemisphere. The patient's brother revealed similar findings. MRI of the suspected cases may facilitate early diagnosis of INAD, and since it is a well-established autosomal recessive neurodegenerative disaese, early and appropriate genetic counseling of the parents is required.
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