Guillain-Barre Syndrome Presenting with Hyperreflexia – A Rare Case Report.

Guillian-Barre syndrome (GBS) is an acquired autoimmune polyradiculopathy. Areflexia and progressive flaccid weakness are essential for its diagnosis. Here we report a case of 25 year old female presenting with acute onset flaccid quadriparesis. The diagnosis of GBS was made on history and clinical findings supported by cerebro-spinal fluid (CSF) analysis and nerve conduction studies (NCS). The hallmark of this case was the presence of hyperreflexia in all four limbs without involvement of higher functions, sensory, autonomic and bulbar dysfunction. To diagnose this rare entity, a high index of suspicion is needed.

Electrophysiological changes in acute guillain-barre syndrome.

Aim: To study the various electrophysiological changes in the motor conduction, sensory conduction and F wave latencies of acute Guillain-Barre Syndrome patients. Methods: Sixteen patients with acute GBS were included in this study. They were subjected to various nerve conduction studies (NCS) following standardized procedures. The mean values obtained for the various nerve conduction parameters were compared against the corresponding standardized values using Student’s t-test. P value less that 0.05 was considered significant. Results: The results of NCS in GBS patients were as follows: 1.The motor nerve conduction velocity was significantly lower and the motor nerve conduction latency was significantly prolonged. 2. The sensory nerve action potential conduction velocity and amplitude remained normal in most of these individuals. 3. F wave latency was significantly prolonged. Conclusion: Acute Guillain-Barre Syndrome patients manifest with abnormal motor nerve conduction parameters and F wave latency. Electrophysiological studies would help the researchers to diagnose the disease at an early stage.

MRI diagnosis of Guillain Barre syndrome / 中华神经医学杂志

Objective To analyze the characteristics and limitations of MRI diagnosis of Guillian Barre syndrome. Methods Fifteen patients with Guillian Barre syndrome (14 in acute stage, 1 in chronic stage) were performed MRI plain scans and contrast-enhanced (CE) T1-weighted images (T1WI) with fat saturation to observe the relation between MRI manifestations and clinical characteristic in peripheral nerves in the vertebral canal. Results Eight patients in acute stage appeared cauda equina nerve thickening to a varying degree by MRI plain scan and the involved nerves showed isointensity on T1WI and isointensity or slight hypointensity on T2WI. In the chronic patient, the cauda equina and the spinal nerve at all the levels of the spinal cord showed obvious thickening. CE T1WI indicated partial spinal and canda equina nerves thickening to a varying degree below T8 level in 14 acute stage patients with 2 accompanied by partial cervical nerves thickening and 2 by partial cranial nerves thickening, in the chronic patient, T1WI showed enhancement and thickening of all the spinal nerves, cauda equina and partial cranial nerves. Every patient had a symptom of weakness in lower limb, including 9 paralyses in both limbs. Enhanced MRI displayed pathologic changes in the cauda equina in all the patients, while 7 complained of weakness in both upper limbs with only 3 (43%) defined on MRI and 6 suffered from functional disorder of the cranial nerves with 3 (50%) defined on MRI. Conclusions MRI is a sensitive method to demonstrate the pathological changes of the cauda equina in GBs;however, under-diagnosis can often happen in involvements of the cervical and cranial nerves.

Rehabilitation of Bickerstaff's Brainstem Encephalitis with Guillain-Barre Syndrome: A case report

We reported a 32-year-old man diagnosed as Bickerstaff's brainstem encephalitis with Guillian-Barre syndrome. After plasmapheresis, his consciousness and respiratory function and motor strength improved. He was discharged without rehabilitation treatment and could perform activities of daily living independently on wheel chair level. For reducing cocontraction of lower extremity muscles, neuromuscular reeducation using EMG biofeedback was performed after admission. After a month of treatment, gait pattern was improved. He received rehabilitative managements such as pool therapy with gait training and improved to 4/5 grade at proximal lower extremities, but the endurance and the quality for his walking was poor because of the cocontraction of muscles in lower extremities. EMG biofeedback for the neuromuscular reeducation leading to each muscle's isolated movements was done. After 2-month rehabilitation, he could walk over 20 meters even level independently without walking aids. This case could be a good model for the effective neuromuscular reeducation.

Clinical evaluation for 30 cases of therapeutic plasma exchange / 대한내과학회지

BACKGROUND: Therapeutic plasma exchange is the therapeutic procedure to improve clinical course by removing various antibodies, immune complexes, toxic materials or by replacing insufficient plasma elements. But there are many debates about its effects although many immunologic diseases improve their clinical courses by plasma exange. The aim of this study is to examine clinical aspects of plasma exchange. METHODS: We investigated retrospectively 30 patients who had been done a total of 128 plasma exchange procedures during the last 5 years at Korea University Anam Hospital. We judged clinical improvement according to diseases, replacing solutions and examined the changes of blood chemistries, side effects etc. RESULTS: There were 14 males and 16 females. The patients' age ranged from 8 to 76 years old and mean age was 45 years old. Total 30 patients had been carried out a total of 128 plasma exchange procedures and the mean was 4.3 times. The frequency of plasma exchange was from once up to 12 times and five times is the most frequent number carried out (27%). The most frequent diseases were multiple myeloma and myasthenia gravis which were 17% respectively and other diseases in the decreasing order of frequency were hemolytic uremic syndrome (HUS), Guillian-Barre syndrome, fulminant hepatitis etc. Double lumen catheter into femoral vein(73%) was most frequently used for the vascular access. The clinical improvement was obtained in 50% of total cases and the clinical results in myasthenia gravis and Guillian-Barre syndrome were excellent. There were 8 patients(27%) who died during treatment period, but the cause of death was not related to plasma exchange procedure itself. As a replacement solution, 5% albumin alone(23%) was used most frequently and the others were fresh frozen plasma(FFP) alone(20%), FFP plus Hartman's soultion plus albumin(20%), FFP plus albumin(17%) etc. Serum total calcium concentration was significantly decreased from 8.5 to 7.9 mg/dL(p=0.017) and platelet was also decreased from 154,666/mm3 to 100,222/mm3(p=0.02) after plasma exchange but the other chemistries were not changed. The frequent side effects were nausea, vomiting, fever, chill, hypotension, itching sensation and muscle cramps. CONCLUSION: The plasma exchange is one of the useful therapeutic procedures in some immunologic diseases especially for the myasthenia gravis and Guillian-Barre syndrome. It is a relatively safe procedure, but gastrointestinal side effects such as nausea, vomiting in early period may be a problem. And the careful monitoring of serum calcium concentration, platelet count before and after plasma exchange is required.

Report of Nationwide Epidemiology of Aseptic Meningitis Outbreak in 1993 in Korea

PURPOSE: There has been no nationwide report pertaining to the epidemiology of aseptic meningitis, although a great numer of patients have been diagnosed of the illness. Therefore, we report an explosive outbreak of aseptic meningitis occured in a nationwide scale in 1993. METHODS: Aseptic meningitis epidemiology surveillence was performed retrospectively on the patients diagnosed of aseptic meinigitis from January 1993 to December 1993 at 60 hospitals (the third graded medical centers and comparable training hospitals) located in 9 districts. The data pertaining to the patients were obtained through the questionnaire answered by the pediatricians or pediatric houseofficers. Monthly and geographical distributions, age and sex distributions, clinical manifestations, laboratory data, complications, and the causative virus of aseptic meningitis were described. RESULTS: 1) The total number of cases reported in 1993 aseptic meningitis epidemiology surveillence was 5,090. Geographically the greatest number of patients was reported from Seoul (2,693), followed by Kyungnam (851), Chunbook (497), Kyungi (492), Chungnam (189), Kangwon (133), Kyungbook (127), Chunnam (82), and Cheju (26). 2) Monthly distribution revealed that the number of patients was increased from April to August and 95.4% (4,858 cases) of the totoal were diagnosed from May to July. The median dates for the diagnosis of aseptic meningitis in various districts were May 8 in Cheju, followed by Chunbook, Kyungnam, Chunnam, Kyungbook, Chungnam, Kyungi, Seoul in June and July 3 in Kangwon. 3) Average age of the study population was 4.7+/-2.9 years and male to female ratio 2.1:1. 4) Subjective symptoms consisted of headache, abdominal pain, poor appetite, irritability, etc, and physical findings fever, local neurological signs, etc in order of frequency. Duration of temperature elevation was 6.1+/-3.7 days in average and 22 cases had no temperature elevation at all. 5) Initial CSF findings revealed pressure of 132 66 mmH2O, leukocytes 594+/-890/mm3 with PMNL 25.6+/-24.7% and lymphocytes 72.3+/-26.1%, protein 47.0+/-37.6 mg/dl, and glucose 60.3+/-14.9 mg/dl. In 35.7% of the patients, CSF leukocytes were more than 500/mm3 and in 19.9% were greater than 1000/mm3. Thirty five patients did not have pleocytosis in the initial CSF examination. 6) The associated illnesses were pharyngotonsillitis (1,285 cases, 31.3%), pneumonia (104 cases, 2.7%), sinusitis (74 cases, 1.9%), etc. 7) While almost all the patients (99.0%) were reported to be improved without complications, 48 cases had complications with 7 encephalitis, 2 neurogenic bladder, 2 Guillian-Barr syndrome, 5 paralysis, and 32 unspecified. Six cases were reported to be deceased. 8) Virus was isolated from 25 out of 55 cases from Seoul and Kyungnam areas on whom virus isolation was tried, and all were echovirus 9. CONCLUSIONS: Aseptic meningitis outbreak in 1993 was nationwide, geographically begun in the southern part of the peninsula spreading rapidly northward, and seasonally occurred from April to August with the peak in June. The average age was 4.7+/-.9 years and male to female ratio 2.1:1. Clinical manifestations and laboratory findings of the study patients were comparable to the reported previoulsy. The causative virus for the nationwide aseptic meningitis outbreak in 1993 was echovirus 9.