[A case of eosinophilic myositis presenting with myocarditis and cardiac embolism].

We report the case of a 72-year-old male who presented with the complaints of muscular pain and weakness. The patient showed marked eosinophilia, elevated levels of myogenic enzymes and pathological abnormalities including eosinophil infiltration obtained from the muscle biopsy. Based on these findings, the patient was diagnosed with eosinophilic myositis. During follow-up, left ventricular wall motion abnormalities with transient electrocardiographic abnormalities were identified; these were believed to be concurrent with eosinophilic myocarditis. Further, notable complications included cardiogenic cerebral embolism. Eosinophilic myositis has been found to cause a wide spectrum of complications. Our findings indicate that in cases of suspected eosinophilic myositis, it is crucial to identify myocarditis immediately and to select an anticoagulant therapy to prevent cerebral embolism.

Successful treatment of granulomatous amoebic encephalitis with combination antimicrobial therapy.

Granulomatous amoebic encephalitis (GAE) is a rare but fatal infection. Due to its nonspecific symptoms and laboratory and neuroradiological findings, it is rarely diagnosed antemortem. We herein present the case of a 72-year-old Japanese woman who was diagnosed with GAE following the detection of a pathogen similar to Balamuthia mandrillaris under a microscopic examination of cerebrospinal fluid sediment and who achieved remission with combination antimicrobial therapy. There are no previous reports of pathogens similar to B. mandrillaris being detected in cerebrospinal fluid antemortem; therefore, this case may be used as a benchmark for further studies.

The spectrum of clinicopathological features in pure autonomic neuropathy.

We assessed the clinicopathological features of nine patients with pure autonomic neuropathy, that is, neuropathy without sensory or motor deficits. The duration from symptom onset to diagnosis ranged from 1 month to 13 years. Of eight patients in whom serum antiganglionic acetylcholine receptor antibody was determined, four were positive. All patients who tested positive for this antibody manifested widespread autonomic dysfunction, with the exception of one patient who only experienced orthostatic hypotension. However, patients who were negative for the antiganglionic acetylcholine receptor antibody presented with partial autonomic failure. One of these patients had diffuse parasympathetic failure and generalized hypohidrosis but no orthostatic hypotension, which is clinically compatible with postganglionic cholinergic dysautonomia. Electron microscopic examination revealed a variable degree of reduction in unmyelinated fibers. Compared with normal controls, the patients had a significantly increased density of collagen pockets (p < 0.05). Additionally, the percentage of Schwann cell subunits with axons (out of the total number of Schwann cell subunits associated with unmyelinated fibers) was significantly decreased (p < 0.01). The density of unmyelinated fibers tended to decrease with increasing time between the onset of autonomic symptoms and biopsy (p < 0.05). In conclusion, the clinical and pathological features of pure autonomic neuropathy vary in terms of progression, autonomic involvement, presence of the antiganglionic acetylcholine receptor antibody, and loss of unmyelinated fibers.

[A case of hypertrophic cranial pachcymeningitis in a patient with lymphocytic hypophysitis].

We report a 67-year-old man of lymphocytic hypophysitis complicated by hypertrophic cranial pachcymeningitis. The hypophysitis presenting with central diabetes insipidus caused isolated adrenocorticotropic hormone (ACTH) deficiency after three years and two months since the onset of his illness. Moreover he suffered from left cavernous sinus syndrome after four years. Brain gadolinium (Gd) enhanced MRI, which was obtained in 2001, showed only enhancement of the pituitary stalk. However, brain Gd enhanced MRI, performed four years later, showed not only a remarkable thickening and enhancement of the pituitary stalk but also abnormal enhancement of the lesion in the left cavernous sinus. In addition, it showed diffuse thickening and enhancement of the dura mater. This clinical course suggested that chronic inflammation of the pituitary had spread to the dura mater, a clinical condition differing from parasellar chronic inflammatory disease (PSCID). In addition, because diffuse thickening and enhancement of the dura mater was present, it was likely that lymphocytic hypophysitis was complicated by hypertrophic cranial pachcymeningitis due to autoimmune reactions. We should carefully observe cases of lymphocytic hypophysitis and assess change over time in the dura mater of the whole brain by Gd enhanced MRI.

[An autopsy case of purulent meningitis, presenting high signals in subarachnoid space and ventricles in diffusion weighted images (DWIs)].

A 79-year-old, bedridden woman with an untreated colon cancer, developed abrupt disturbance of consciousness and high fever. Brain MRI showed significant high signals in the subarachnoid space and ventricles in diffusion-weighted images (DWIs), and she died on the same day. At autopsy, much exudate was found over the base of the brain, leading to a diagnosis of purulent meningitis. On histological findings, inflammatory cell infiltration was significantly restricted within the subarachnoid space, but not into the brain parenchyma. This case report demonstrated that high signals in subarachnoid space and ventricles in DWIs may be very useful for diagnosis of purulent meningitis.