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A 16-year-old male with language disorders, such as motor aphasia or mutism, was hospitalized on day 4 after the onset of fever. Magnetic resonance imaging (MRI) on admission revealed lesions of the corpus callosum and brain white matter. Brain single photon emission computed tomography (99mTc-ethyl cysteinate dimer) on day 7 shows hypoperfusion (with right dominance) of bilateral upper parietal region. His condition improved gradually with symptomatic treatments alone, and he was discharged on day 13. The lesions on the MRI disappeared by day 15. Although this case might have suffered from leukoencephalopathy, clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) type II was suspected from the reversible splenial lesion. Except for the elevation (640 times) of mycoplasma pneumonia antibody titer (particle agglutination) in the serum, the blood tests and cerebrospinal fluid findings showed no significant abnormalities. We then considered this encephalopathy was related to mycoplasma pneumonia infection. Since no symptoms of mycoplasma infection except for neurologic symptoms were observed, indirect mechanism, such as immune-mediated reactions, is suggested to cause encephalopathy in this case.

A 31-year-old woman was admitted to the hospital after several episodes of transient numbness in her fingers and dysarthria. No neurological symptoms and signs were observed in this patient on admission; however, blood tests revealed elevated amylase and immunoglobulin G levels, an increased erythrocyte sedimentation rate, and positive anti-Sjögren's-syndrome (SjS)-related antigen A antibodies. The cerebrospinal fluid examination revealed a slight increase in the cell count and protein content. Diffusion-weighted and fluid-attenuated inversion recovery images on magnetic resonance imaging showed high-signal-intensity lesions in the cerebral white matter and basal ganglia supplied by the left middle cerebral artery. A part of the lesions was enhanced by the administration of gadolinium contrast medium. Magnetic resonance angiography suggested stenosis in the left anterior, middle, and posterior cerebral arteries. The patient was thus diagnosed with acute cerebral infarction and was then treated with argatroban, edaravone, and clopidogrel. Furthermore, Schirmer's test, sialography, and salivary gland biopsy results led to the diagnosis of SjS. A nerve conduction study suggested concomitant mild polyneuritis or mononeuritis multiplex. Collectively, we speculate that cerebral arterial stenosis associated with SjS leads to transient ischemic attack-like episodes, transient neurological symptoms and signs, and cerebral infarction. (Received June 13, 2018; Accepted September 11, 2018; Published December 1, 2018).

We observed a 41-year-old woman with severe central pontine myelinolysis (CPM) and unusually extensive extrapontine myelinolysis (EPM), but without evidence of hyponatremia. Increased alcohol consumption in prior months was the main cause of her CPM/EPM. However, in general, EPM is a rare accompaniment in alcoholic patients with CPM without hyponatremia. With regard to our patient, the EPM was unusually widespread; magnetic resonance imaging (MRI) of her brain showed multiple hyperintense lesions on T2-weighted images distributed symmetrically in bilateral caudate nuclei, lentiform nuclei and thalami. Serial follow-up MRI revealed almost complete resolution of EPM after methylprednisolone pulse therapy. By contrast, marked cavitary hypointensity in the pons remained, but complete remission of neurological symptoms was achieved.

A patient with amyopathic dermatomyositis associated with fatal rapidly progressive interstitial pneumonitis resistant to therapy is described. Pathologic examination of a transbronchial lung biopsy specimen showed diffuse alveolar damage and nonspecific interstitial pneumonia-organizing pneumonia-like findings. Bronchoalveolar lavage fluid contained many CD8+ lymphocytes, considered to be cytotoxic T cells. Analysis of bronchoalveolar lavage fluid in this case may provide prognostically and pathogenetically important information.

CNS involvement is rare in Churg-Strauss syndrome (CSS); only approximately 6 to 8% of patients with CSS have CNS complications.1,2 To date, only a few cases of CNS vasculitis in CSS have been described in detail. They usually consist of reports about cerebral infarctions, intracerebral hemorrhages, and subarachnoid hemorrhages.1–6 Herein, we describe a patient with CSS presenting as multifocal enhancing cortical lesions on MRI. ### Case report. A 62-year-old woman with a history of bronchial asthma for approximately 10 years was admitted to our hospital due to left-sided weakness and visual disturbance. The symptoms developed over 1 week, and were accompanied by multiple skin rashes, occasional fresh hematochezia, and worsening shortness of breath due to frequent attacks of asthma. Her examination was remarkable for erythematous maculopapules distributed over her abdomen and extensor surface of the lower extremities (figure, A), slight tachypnea with a room air saturation of 93%, and expiratory wheezing with inspiratory crackles at bilateral lung bases. Neurologic examination disclosed mild left hemiparesis and binocular blurred vision, especially in the central visual field. Figure Cutaneous lesions and brain MRI findings Multiple erythematous maculopapules are evident on the extensor surface of the lower extremities (A). Biopsy specimens obtained from the skin lesions demonstrated multiple foci of granulomatous angiitis (B) and dense perivascular eosinophilic infiltrates (C) in …

We present distinctive MRI findings in an adult female patient with possible Sydenham's chorea. T2-weighted MRI showed bilaterally symmetric, diffusely homogenous, and clearly demarcated hyperintensities selectively involving the entire striatum with swelling of the bilateral caudate heads. The MRI features may reflect the pathogenetic mechanisms of Sydenham's chorea associated with a specific autoimmune response to the basal ganglia.

We describe a case of bacteremic, leukopenic pneumococcal pneumonia with respiratory failure, accompanied by diabetic ketoacidosis and hypothermia. Pulmonary leukostasis may play a role in the pathogenesis of the acute respiratory distress syndrome (ARDS) in pneumococcal pneumonia. The patient recovered with mechanical ventilation, intravenous antibiotics, pulse-steroid therapy, and continuous hemodiafiltration (CHDF). In particular, administration of steroid and the use of CHDF may improve the status of pulmonary leukostasis in leukopenic pneumococcal infection.

In order to clarify endocrine abnormalities due to hypothalamic involvement in multiple sclerosis (MS), serum prolactin levels were measured in 27 patients with MS and 22 healthy subjects. The presence of hypothalamic lesions was also studied by magnetic resonance imaging (MRI). Serum prolactin levels were found to be significantly higher in MS patients than in healthy controls in both sexes. Although only one patient had galactorrhea, one-third of the MS patients had mild to moderate hyperprolactinemia, which was a 4-13-fold increase over the mean value of healthy subjects. The results of thyrotropin-releasing hormone, sulpiride, L-DOPA and bromocriptine loading tests suggested a hypothalamic dysfunction, rather than pituitary prolactinoma in MS patients. Four of eight patients with hyperprolactinema had diencephalic hypothalamic lesion(s) contiguous with the third ventricle on the brain MRI, while none of the normoprolactinemic patients had any lesions in the diencephalon. All relapsing-remitting patients with hyperprolactinemia showed a rise in prolactin levels in the acute stage of the relapse and a decrease during the recovering stage and the following remission phase. Our findings suggest that latent hyperprolactinemia due to hypothalamic dysfunction occurs frequently in MS patients in relapse. The increase of serum prolactin is considered to be a sensitive indicator for hypothalamic lesions in MS.