Clinical features of Guillain-Barré syndrome patients with elevated serum creatine kinase levels.

BACKGROUND: It is not well defined whether Guillain-Barré syndrome (GBS) patients with elevated serum creatine kinase (CK) levels have characteristic clinical features and are related to the subgroups of GBS. METHODS: We retrospectively studied 51 consecutive patients with GBS, who visited our hospital, and compared clinical, laboratory and electrophysiological findings between patients with and without elevated CK levels. RESULTS: Of 51 patients, 14 patients (27%) showed an elevation of serum CK levels. When compared with patients with the normal CK levels, the ratios of male, antecedent infections, and anti-GM1 antibody positivity were significantly higher in patients with elevated CK levels. The ratios of hypoesthesia, cranial nerve involvement, and urinary retention were significantly less in patients with elevated CK levels. There were no significant differences in disability at peak between two groups. In the electrophysiological examination, sensory nerve abnormalities were not observed. Although some patients with elevated CK levels showed prolongation of distal motor latencies (DMLs) and increase of durations in the initial examination, development of the prolongation of DMLs and increase of durations was not observed in the follow-up examinations. The findings were consistent with acute motor axonal neuropathy (AMAN) with reversible conduction failure (RCF) but not acute inflammatory demyelinating polyneuropathy (AIDP). CONCLUSIONS: The results suggest that the GBS patients with elevated CK levels represent not a group of AIDP but a group of AMAN with axonal degeneration or RCF even though the initial electrophysiological examination shows AIDP pattern.

A Case of Type 2 Diabetes Mellitus with Lung Cancer Suffered from Euglycemic Diabetic Ketosis Accompanied by Adrenal Insufficiency after Immune Checkpoint Inhibitors.

A 74-year-old patient with type 2 diabetes mellitus received basal-bolus insulin, insulin secretagogues, and sodium glucose transporter 2 (SGLT2) inhibitors. After immune checkpoint inhibitor treatment for lung cancer, he suffered from depressed consciousness with a urinary ketone body (3+). When all hypoglycemic treatments were discontinued, his serum blood glucose remained at 121 mg/dL. He was diagnosed with euglycemic diabetic ketosis. Endocrine loading tests revealed isolated adrenocorticotropic hormone (ACTH) deficiency as an immune-related adverse event. It was suggested that euglycemic diabetic ketosis was induced by the self-suspension of insulin and insulin secretagogues, adrenal insufficiency, SGLT2 inhibitors, and carbohydrate intake shortage.

Serial MRI findings in a relapsing-remitting form of neuro-Behçet's disease: a case report.

We describe a case of neuro-Behcet's disease (NBD) characterized by recurrent attacks of neurologic deficit. T2-weighted images showed a high signal intensity lesion with extensive edema in the right thalamolenticular region, midbrain, and pons as well as the cerebral white matter. After a relapse of the disease, MRI demonstrated a high signal intensity in the left thalamus, internal capsule, and midbrain. These MRI abnormalities showed marked resolution with steroid treatment. We observed sequential MRI findings in a patient with a relapsing-remitting form of NBD who had parenchymal CNS involvement, and we examined the correlation among the MRI findings and clinical features during the clinical course.

[Herpes simplex encephalitis with expanded cerebral cortex lesions on T1-weighted MRI after clinical improvement: a case report].

We described a 58-year-old woman with herpes simplex encephalitis (HSE), who initially had fever and developed impaired consciousness. Cerebrospinal fluid (CSF) examination showed mononuclear pleocytosis and the existence of herpes simplex virus (HSV) DNA. The first T1-weighted MR image showed symmetrical swelling and low signal intensity lesions in the medial temporal lobes and hippocampus. T2-weighted MR image showed high signal intensity lesions in the medial temporal lobes, the amygdala, the hippocampus, the insula and the cingulate gyri bilaterally. After the treatment with intravenous acyclovir and betamethasone, impaired consciousness and recent memory disturbance gradually improved. On the second T1-weighted MR image examination, eighteen days after the onset, high signal intensity lesions were demonstrated in the right medial temporal lobe, the right hippocampus, the left insula and the bilateral cingulate gyri. Although the clinical symptoms had improved significantly over three months, the high signal intense lesions on T1-weighted MR images were also detected in the left medial temporal lobe, the right insula, and the straight gyrus. Brain CT did not demonstrate any abnormalities. The repeated CSF examinations showed negative HSV DNA and a decreased number of WBC. However, oligoclonal IgG bands were continuously positive. Myelin basic protein level and IgG index increased in parallel with the expansion of the cerebral lesions on T1-weighted MR images. In the present case, the abnormality of T1-weighted MRI was thought to indicate hemorrhagic inflammatory lesions that could not be detected by CT. The increased level of myelin basic protein, the elevated IgG index and the continuous positive oligoclonal IgG indicated continuous immunologic response against HSV in these lesions.

[Brain MRI of reversible, recurrent white matter lesions in a patient with a 35-year history of neuro-Behçet disease].

Neuro-Behçet disease (NBD) can be categorized clinically as the acute type--characterized by meningoencephalitis--and the chronic progressive type- characterized by slowly progressive dementia, ataxia, and dysarthria. We describe a 35-year clinical course of NBD that was characterized by slowly progressive ataxia and dysarthria despite continued corticosteroid treatment. Because of difficulties in swallowing, which interrupted oral corticosteroid therapy, this case was characterized by recurrent manifestations of neurological symptoms and abnormal MRI findings. Resumption of corticosteroid therapy was effective. The patient was a 77-year-old woman who had presented with oral ulceration and dysarthria at the age of 42. She suffered from Entero-Behçet disease at the age of 52 and was treated with corticosteroids for 7 years. Oral corticosteroid therapy was resumed at the age of 64, but her neurological deficit slowly progressed and she developed paraplegia with dysphagia and dysarthria. Corticosteroids treatment was interrupted when she was 76; one year later, she was hospitalized in a state of somnolence. Brain MRI scans revealed new lesions with gadolinium enhancement. We diagnosed acute exacerbation of NBD attacks on the basis of positive findings for HLA-B51, protein elevation, and IL-6 in the cerebrospinal fluid. Corticosteroid treatment was effective. She became alert, and her MRI findings were no longer abnormal. Corticosteroids administration was continued via percutaneous endoscopic gastrostomy. Our case suggested that even if neurological exacertion is not obvious during the clinical course, immunosuppressive therapies should be continued for patients with chronic NBD to prevent acute aggravation.

Isolated adrenocorticotropic hormone deficiency with transient thyroiditis inducing an adrenal crisis.

OBJECTIVE: It was the aim of this study to describe a patient with isolated adrenocorticotropic hormone deficiency presenting with a variety of involuntary movements who developed an adrenal crisis due to transient thyroiditis. CLINICAL PRESENTATION AND INTERVENTION: A 61-year-old man was hospitalized with a variety of involuntary movements that were suspected manifestations of metabolic encephalopathy. After admission, his general status rapidly deteriorated to a life-threatening condition that included a degree of hyponatremia. The hyponatremia and metabolic encephalopathy provided clues toward a definitive diagnosis. After corticosteroid and sodium supplementation improved the status of the patient, endocrinological examinations revealed that he suffered from isolated adrenocorticotropic hormone deficiency followed by transient thyroiditis that induced an adrenal crisis. CONCLUSION: This case emphasizes the importance of considering hypoadrenalism when encountering hyponatremia or metabolic encephalopathy of unknown etiology.

The relationship of psychological factors to the prognosis of hyperthyroidism in antithyroid drug-treated patients with Graves' disease.

OBJECTIVE: The relationship between emotional stress and the onset of hyperthyroidism has been well investigated, but the relationship between psychological factors and prognosis of antithyroid drug-treated hyperthyroidism is not well known. This study has examined not only emotional stresses but also patients' personality traits using specific tests. DESIGN: A prospective cohort study. SUBJECTS: Sixty-nine patients with hyperthyroid Graves' disease in the euthyroid state after 2-5 years of antithyroid drug therapy and 32 healthy subjects as the control group. MEASUREMENTS: Patients responded to three types of questionnaires, including the Minnesota Multiphasic Personality Inventory for personality traits, the Natsume's Stress Inventory for major life events, and the Hayashi's Daily Life Stress Inventory for daily life stresses. RESULTS: In the Graves' disease patients, stress scores of life events correlated significantly with serum TSH receptor antibody activity (r = 0.424, P < 0.001) and thyroid volume (r = 0.480, P < 0.001). When the patients were divided according to prognosis (41 with relapse and 28 with remission), four personality traits including hypochondriasis, depression, paranoia and psychasthenia (mental fatigue) were significantly (P = 0.0146, 0.0052, 0.0125, and 0.0186, respectively) more common in the relapsed Graves' disease group than those of the remitted group. Six personality traits of conversion hysteria, psychopathic deviation, masculinity and feminity, schizophrenia, hypomania, and social introversion were not significantly different between the two groups. The scores of daily hassles (problems of daily life) were also significantly (P = 0.0124) greater in the relapsed Graves' disease group than in the remitted group. The scale scores of depression and psychasthenia showed a positive correlation with scores of daily hassles (r = 0.535, P < 0.0001; r = 0.580, P < 0.0001, respectively), while an inverse correlation with scores of daily uplifts (r = -0.373, P = 0.0332; r = -0.322, P = -0.0120, respectively). CONCLUSIONS: The results suggest that major life events, personality traits of hypochondriasis and depression, paranoia, mental fatigue, and daily problems aggravate the prognosis of antithyroid drug-treated hyperthyroidism. Escape from life events is virtually impossible; thus coping strategies suggested by the physician may be useful in improving prognosis in Graves' disease.

Serum concentrations of granulocyte colony-stimulating factor (G-CSF) in antithyroid drug-induced agranulocytosis.

Granulocyte colony-stimulating factor (G-CSF) levels in serum were determined by a highly-sensitive chemiluminescent enzyme immunoassay (limit of detection, 0.5 pg/ml) in 54 patients with Graves' disease including 6 patients complicated with methimazole-induced agranulocytosis. Serum G-CSF levels in patients with Graves' disease were not different from normal subjects and did not correlate with serum FT4 level or circulating neutrophil counts. Before the onset of agranulocytosis, there was no difference in serum G-CSF level between the patients complicated with agranulocytosis and the uncomplicated patients. When circulating neutrophil counts decreased to less than 0.5 x 10(9)/L, serum G-CSF level elevated with the mean of 106.8 +/- 82.2 (SD) pg/ml, but the level did not correlate with the duration of agranulocytosis. Interestingly, maximum serum G-CSF level during the treatment with recombinant human G-CSF (100 microg/day) was related to bone marrow finding at the onset of agranulocytosis and correlated with the duration of agranulocytosis (r = 0.824, p < 0.05). In conclusion, measuring serum G-CSF levels with a highly-sensitive chemiluminescent enzyme immunoassay revealed that 1) thyrotoxicosis does not affect serum G-CSF level, 2) serum G-CSF level during antithyroid drug treatment does not play an important role in development of agranulocytosis, 3) the maximum serum G-CSF level in the course of agranulocytosis is related to the responsiveness of bone marrow to G-CSF and the recovery time from agranulocytosis.