Autoimmune polyendocrinopathy syndrome is a heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected. We present a case of autoimmune polyendocrinopathy syndrome type 2 in a 42-year-old woman with Guillain-Barre syndrome and scleroderma. This combination of syndromes has not been reported and warrants further investigation.
Adult ; Female ; Guillain-Barre Syndrome ; complications ; diagnosis ; Humans ; Polyendocrinopathies, Autoimmune ; diagnosis ; Scleroderma, Diffuse ; complications ; diagnosis

A 59-year-old man was admitted with numbness, pain, and a tingling sensation in both lower legs. He was initially diagnosed with diabetic peripheral neuropathy based on a symptom questionnaire and a quantitative sensory test. Despite symptomatic treatment of diabetic neuropathy, he complained of worsening sensory symptoms and additional motor weakness in both lower extremities. As the motor weakness of both extremities became more aggravated over time, brain and spine imaging tests and a nerve conduction test were performed. The nerve conduction study revealed motor and sensory axonal neuropathy. In his cerebrospinal analysis, albumino-cytologic dissociation, which is compatible to the Gillian-Barre syndrome, was found. Cerebrospinal fluid analysis showed albumino-cytologic dissociation. He was treated with intravenous immunoglobulin and his neurologic deficits were gradually improved.
Diabetic Neuropathies/*complications/*diagnosis ; Electromyography ; Guillain-Barre Syndrome/*complications/*diagnosis ; Humans ; Male ; Middle Aged ; Neural Conduction ; Neurologic Examination

We report here a case of acute hepatitis A, which was complicated by Guillain-Barr? syndrome (GBS). A 32-year old male admitted to our hospital with the symptoms of acute hepatitis and was diagnosed to have acute hepatitis A with positive IgM anti hepatitis A virus antibody. On 9th day after the onset of jaundice, acute progressive, ascending, symmetric motor paralysis were developed and eventually respiratory failure ensued. Cerebrospinal fluid analysis showed albumino-cytologic dissociation and nerve conduction velocity test suggested a polyradiculopathy. He was diagnosed to have GBS and treated with intravenous immunoglobulin and required a ventilatory support. After 90 hospital days, he recovered in ambulatory condition with the aid of crutches. The clinical course, prognosis and the outcome of neuropathic symptoms of GBS following acute hepatitis A were relatively poor in our case.
Acute Disease ; Adult ; Guillain-Barre Syndrome/*diagnosis/etiology/therapy ; Hepatitis A/complications/*diagnosis ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Male

OBJECTIVEThere is no report on Bickerstaff's brainstem encephalitis (BBE) patients in China. We here report the first case of BBE in China.

METHODSClinical features, results of electromyography, electroencephalography (EEG), magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) examination were studied to clarify the characteristics of this syndrome.

RESULTSA 44-year-old man presented himself at our inpatient department with somnolence and dizziness as his initial symptoms. He developed multiple cranial nerves paralysis especially internal and external ophthalmoplegia, ataxia and tetraparesis within 1 week. His condition rapidly deteriorated, and he experienced coma. Electromyography showed indications of peripheral nerve dysfunction, electroencephalography revealed loss of basic rhythm, MRI demonstrated high-intensity abnormalities on T(2)-weighted images of medulla oblongata, and CSF albuminocytological dissociation was defined abnormally as high protein. Ten months later, he almost completely recovered.

CONCLUSIONBBE, fisher syndrome (FS) and Guillain-Barré syndrome (GBS) are similar clinically; BBE and FS were proposed to be the variant of GBS.

Adult ; Brain ; pathology ; Coma ; Diagnosis, Differential ; Electroencephalography ; Electromyography ; Encephalitis ; complications ; Guillain-Barre Syndrome ; complications ; Humans ; Magnetic Resonance Imaging ; Male ; Medulla Oblongata ; pathology ; Time Factors

Acute motor and sensory axonal neuropathy (AMSAN) are recently described subtypes of Guillain-Barre syndrome characterized by acute onset of distal weakness, loss of deep tendon reflexes, and sensory symptoms. A 21-yr-old male was transferred to our hospital due to respiration difficulties and progressive weakness. In laboratory findings, immunoglobulin M antibodies against hepatitis A were detected in blood and cerebrospinal fluid. The findings of motor nerve conduction studies showed markedly reduced amplitudes of compound muscle action potentials in bilateral peroneal, and posterior tibial nerves, without evidence of demyelination. Based on clinical features, laboratory findings, and electrophysiologic investigation, the patient was diagnosed the AMSAN following acute hepatitis A viral infection. The patient was treated with intravenous immunoglobulin and recovered slowly. Clinicians should consider this rare but a serious case of AMSAN following acute hepatitis A infection.
Acute Disease ; Electromyography ; Guillain-Barre Syndrome/*diagnosis/drug therapy/etiology ; Hepatitis A/complications/*diagnosis ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Male ; Young Adult

Biomarkers ; blood ; Brain ; diagnostic imaging ; pathology ; Brain Edema ; etiology ; pathology ; Child ; Diagnosis, Differential ; Electroencephalography ; Guillain-Barre Syndrome ; complications ; diagnosis ; therapy ; Humans ; Magnetic Resonance Imaging ; Male ; Posterior Leukoencephalopathy Syndrome ; complications ; diagnosis ; therapy ; Radiography

INTRODUCTIONSevere Guillain-Barré syndrome (GBS) causes ventilatory insufficiency and the need for prolonged artificial ventilation. Under circumstances where medical care for patients with severe GBS is required in a resource-limited institution, identifying initial clinical presentations in GBS patients that can predict respiratory insufficiency and the need for prolonged mechanical ventilation (> 15 days) may be helpful for advanced care planning.

METHODSThe medical records of patients diagnosed with GBS in a tertiary care and medical teaching hospital from January 2001 to December 2010 were retrospectively reviewed. The demographic data and clinical presentations of the patients were summarised using descriptive statistics. Clinical predictors of respiratory insufficiency and the need for prolonged mechanical ventilation (> 15 days) were identified using univariate logistic regression analysis.

RESULTSA total of 55 patients with GBS were included in this study. Mechanical ventilation was needed in 28 (50.9%) patients. Significant clinical predictors for respiratory insufficiency were bulbar muscle weakness (odds ratio [OR] 5.08, 95% confidence interval [CI] 1.31-21.60, p = 0.007) and time to peak limb weakness ≤ 5 days (OR 0.75, 95% CI 0.62-0.91, p < 0.001). Bulbar muscle weakness (p = 0.006) and time to peak limb weakness ≤ 5 days (p < 0.001) were also found to be significantly associated with the need for prolonged mechanical ventilation (> 15 days).

CONCLUSIONBulbar weakness and time to peak limb weakness ≤ 5 days were able to predict respiratory insufficiency and the need for prolonged mechanical ventilation in patients with GBS.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Guillain-Barre Syndrome ; diagnosis ; therapy ; Humans ; Male ; Middle Aged ; Muscle Weakness ; complications ; Odds Ratio ; Regression Analysis ; Respiration, Artificial ; Respiratory Insufficiency ; therapy ; Retrospective Studies ; Tertiary Care Centers ; Young Adult

Autochthonous hepatitis E virus (HEV) is an emerging pathogen in developed countries, and several cases of acute HEV infection have been reported in South Korea. However, there have been no reports on HEV-associated Guillain-Barré syndrome (GBS) in Korea. We recently experienced the case of a 58-year-old Korean male with acute HEV infection after ingesting raw deer meat. Persistent cholestasis was resolved by the administration of prednisolone. At 2.5 months after the clinical presentation of HEV infection, the patient developed weakness of the lower limbs, and was diagnosed with GBS associated with acute hepatitis E. To our knowledge, this is the second report on supportive steroid therapy for persistent cholestasis due to hepatitis E, and the first report of GBS in a Korean patient with acute HEV infection.
Acute Disease ; Alanine Transaminase/blood ; Antibodies, Viral/blood ; Aspartate Aminotransferases/blood ; Bilirubin/analysis ; Cholestasis/*drug therapy ; Guillain-Barre Syndrome/complications/*diagnosis ; Hepatitis E/*diagnosis/etiology ; Hepatitis E virus/immunology ; Humans ; Immunoglobulin M/blood ; Liver/pathology ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Republic of Korea ; Steroids/*therapeutic use