Progression of bilateral internal carotid artery dissection during antiplatelet therapy.

Bilateral internal carotid artery dissection (ICAD) is a rare but important cause of stroke in young adults. Anticoagulant and/or antiplatelet agents are usually recommended for stroke prevention;however, such treatments remain highly controversial, and there are inadequate data to compare the efficacy of anticoagulation and antiplatelet therapy. We herein report the case of 30-year-old man presenting with progressive bilateral ICAD during antiplatelet treatment. This report suggests the possibility that intramural hematomas are enlarged by antiplatelet and anticoagulant agents and draws attention to the medications associated with ICAD.

Eosinophilia with organ involvement in 3 siblings.

We describe 3 siblings who suffered from marked eosinophilia with organ involvement. One sibling, who experienced cervical lymphadenopathy and peripheral neuropathy with eosinophilia (5,834 cells/µL) following bronchial asthma, was diagnosed with Churg-Strauss syndrome (CSS) according to the criteria of the American College of Rheumatology. Another sibling, who suffered from severe asthma with persistent polyarthritis and eosinophilia (2,496 cells/µL), was also diagnosed with CSS according to the criteria of the Japanese Ministry of Health, Labour and Welfare. The remaining sibling, who had eosinophilic pleuritis with peripheral blood eosinophilia (699 cells/µL), did not fulfill the widely used criteria for CSS or hypereosinophilic syndrome (HES) ; however, he fit the newly proposed criteria for HES. Glucocorticoid treatment relieved their symptoms. Although the diagnoses and the criteria used for diagnosis differed between the siblings, all 3 patients showed common features such as eosinophilia with organ involvement that required treatment, indicating the possibility of familial eosinophilia (FE). Furthermore, the clinical features observed differed substantially from those of previously reported FE patients, therefore, these 3 siblings may be affected by a type of FE distinguishable from those previously described.

Morel's laminar sclerosis showing apraxia of speech: distribution of cortical lesions in an autopsy case.

A 57-year old man with chronic alcoholism presented with apraxia of speech and disturbance of consciousness. He had a history of gastrectomy and had been drinking alcohol. The symptoms improved with administration of thiamine, but he later developed diarrhea and delirium, and died approximately 40 days after the onset. Autopsy findings were consistent with Wernicke's encephalopathy and pellagra encephalopathy. Furthermore, laminar cortical necrosis with vacuoles and astrocytosis was found in the second and third layers of the bilateral frontal cortices, suggesting Morel's laminar sclerosis. The lesions were mainly located in the bilateral primary motor cortices. Involvement of the lower part of the left primary motor cortex may be associated with apraxia of speech in our case.

Lewy body variant of Alzheimer's disease or cerebral type lewy body disease? Two autopsy cases of presenile onset with minimal involvement of the brainstem.

Lewy bodies (LB) usually extend from the brainstem to the cerebrum in patients with Parkinson's disease. However, whether the patterns of progression of LB and neuronal loss in Parkinson's disease are identical to those in other Lewy body diseases (LBD) remains unclear. In addition, pathological data on the autonomic nervous system involvement in LBD are limited. We present here the clinicopathological characteristics of two autopsy cases with both Alzheimer's disease and dementia with Lewy bodies (DLB), possibly diagnosed as having Lewy body variant of Alzheimer's disease (LBV/AD). Our patients presented clinically with dementia without parkinsonism. Histopathologically, phosphorylated alpha-synuclein-positive LB and Lewy neurites were abundant in the limbic system, especially in the amygdala, and to a lesser degree, in the neocortex, including the primary motor cortex. The amygdala was also most severely affected by neuronal loss, and the other limbic areas and neocortex were affected to a lesser degree. Despite the existence of a small number of LB and many Lewy neurites, neurons in the brainstem nuclei were relatively well preserved. The Braak stages of concurrent neurofibrillary changes and senile plaques were stage V and C, respectively, in both cases. Tyrosine hydroxylase-positive nerve fibers were relatively well spared in one case examined compared with Parkinson's disease cases. Furthermore, many Lewy neurites immunopositive for phosphorylated a-synuclein were found in the nerve fascicles of the epicardium in one case examined and in Parkinson's disease cases to a lesser degree. These findings suggest that: (i) in at least some LBV/AD cases, the amygdala develops neuronal loss and Lewy-related pathology prior to the brainstem nuclei; and (ii) the depletion of nerves in the heart tissue of LBV/AD is not necessarily complete despite the development of Lewy-related pathology.

Reverse 'see-saw' relationship between Graves' disease and myasthenia gravis; clinical and immunological studies.

Although the association of two distinct autoimmune diseases, Graves' disease (GD) and myasthenia gravis (MG), is rare, the relationships of clinical and immunological activities between the two diseases remain unknown. In the present study, we investigated whether there exist any relationships between clinical and immunological activities of GD and MG as well as any common characteristics of their HLA antigens in five patients with concomitant association with GD and MG. The present study clearly showed positive relationships between the clinical activities of GD and MG in all five cases. Except for two cases, one with undetectable acetylcholine receptor antibody and another with few sample number, there were positive relationships between two circulating auto-antibodies against TSH receptor and acetylcholine receptor as well as their immunological and clinical activities in the remaining three cases. Furthermore, the present serological HLA typing study revealed that all five cases had common HLA-DQ3. Therefore, our study clearly demonstrates a reverse 'see-saw' relationship between GD and MG based on their clinical and immunological features, and suggests that HLA-DQ3 may play a potential pathogenic role in the concomitant development of the two diseases.