Masayuki Sugie

Cerebral Venous Thromboembolism in Antiphospholipid Syndrome Successfully Treated with the Combined Use of an Anti-Xa Inhibitor and Corticosteroid.

We herein report a case presenting with cerebral venous sinus thrombosis (CVST) associated with primary antiphospholipid syndrome (APS). The patient developed recurrent CVST followed by a hemorrhagic ischemic stroke despite the use of warfarin during the appropriate therapeutic window. Thus, we substituted warfarin to rivaroxaban with prednisolone and obtained a good clinical course. In addition to the effect of prednisolone of inhibiting elevated lupus anticoagulants and the recurrence of arterial thrombosis, rivaroxaban may prevent CVST and inhibit hypercoagulability induced by corticosteroids. The combination of an anti-Xa inhibitor and corticosteroid may be an alternative treatment for CVST and arterial thrombus with warfarin-resistant APS.

Primary central nervous system lymphoma initially mimicking lymphomatosis cerebri: An autopsy case report

A 59‐year‐old immunocompetent man was admitted to our hospital because of progressive dementia with concomitant bilateral uveitis. The first brain MRI revealed diffuse hyperintense lesions in the cerebral white matter of both hemispheres on a T2‐weighted image and fluid‐attenuated inversion recovery image. However, another MRI taken more than 1 month later revealed enhanced cohesive mass lesions in the bilateral thalami, in addition to the white matter lesions. The white matter lesions were slightly hyperintense on a diffusion‐weighted image and apparent diffusion coefficient map image, suggesting vasogenic edema. One year after the onset of uveitis, he died of respiratory failure. Pathological diagnosis was diffuse large B‐cell lymphoma with perivascular proliferation and diffuse scattered infiltration in the cerebrum and brainstem. Microscopically, cohesive mass lesions in the bilateral thalami were a massive cluster of lymphoma cells. This is a case of primary CNS lymphoma (PCNSL) mimicking ‘lymphomatosis cerebri (LC)’ at first but later exhibiting typical mass lesions, giving rise to the possibility that cases of LC might unmask features of regular lymphomas in their later course more often than believed thus far.

Severe cortical involvement in MV2 Creutzfeldt-Jakob disease: an autopsy case report.

MV2 type sporadic Creutzfeldt–Jakob disease (sCJD) is reported to have a long duration and marked involvement of the cerebral deep gray matter. We describe an autopsied long‐surviving sCJD case of MV2. In the early stages, the patient exhibited memory impairment, attention deficit and semantic memory disorder. Diffusion‐weighted MRI showed abnormal hyperintensity signals along the cerebral cortex, sparing the thalami and basal ganglia. Pathological observations included: severe spongiosis throughout the cerebral cortex, several kuru plaques and plaque‐like PrP deposits in the cerebellum, with only minimal degeneration in the thalami and basal ganglia. Our case suggests that MV2 has a wide clinicopathological spectrum, which ranges from “VV2” to “MM2” type.

Increased norepinephrine-associated adrenomedullary inclusions in Parkinson's disease

The association with Parkinsons disease (PD) of adrenomedullary inclusions, known as ‘hyaline globules’ or ‘adrenal bodies’, has been reported for over 35 years. However, the common perception has been that adrenomedullary chromaffin cells cannot be recognized as pathological cells in PD. In the present study, we discovered that the number of adrenomedullary inclusions per unit area of the adrenal medulla was larger in PD and other Lewy body disorders (LBD) than in other neurological diseases and controls without any autonomic dysfunctions, and correlated with the duration of LBD. We also showed that the cells with adrenomedullary inclusions are all norepinephrine‐secreting chromaffin cells. This was detected by PAS reaction following peroxidase immunohistochemistry of four proteins: chromogranin A, phenylethanolamine N‐methyltransferase, S‐100 protein and neurofilament protein. We also proved that the components of adrenomedullary inclusions are immunocytochemically different from those of Lewy bodies and Lewy‐related neurites, as adrenomedullary inclusions were immunonegative to ubiquitin and α‐synuclein as well as to the above four proteins. Therefore, contrary to current opinion, the norepinephrine‐secreting adrenomedullary chromaffin cell is indeed another type of pathological cell in PD and other LBD.

Infectious aneurysm of the intracavernous carotid artery occurring concomitantly with sphenoid sinusitis; an autopsy case report

A previously healthy 56-year-old woman presented with right-sided ophthalmic pain and diplopia following headache and fever. A neurological examination revealed 3rd and 6th right cranial nerve palsies. Brain magnetic resonance imaging (MRI) and 3D-computed tomography (CT) angiography (CTA) showed right-sided sphenoid sinusitis, cavernous sinus thrombophlebitis, and aneurysms in the right intracavernous carotid artery and in a portion of internal carotid-posterior communicating artery. We diagnosed her condition as cavernous sinus syndrome with an infectious aneurysm secondary to sphenoiditis; therefore, broad spectrum antibiotics were administered. However, 7 days after admission, she died of massive epistaxis. Macroscopically, coagulated blood was observed at the surface of the sphenoid sinus, suggesting bleeding in the cavernous sinus. A histopathological examination revealed severe infiltration of the inflammatory cells into the cavernous sinus and sphenoid mucosa. Rupture of the aneurysm in the cavernous sinus was also observed. However, no pathogenic organism was identified. We thought that the sphenoid sinusitis had spread through the venous flow into the cavernous, and the infectious aneurysm developed due to infiltration of inflammatory cells into the arterial wall. This is the first detailed clinico-pathological study of an infectious aneurysm in the intracavernous internal carotid artery occurring concomitantly with sphenoiditis.

perceiving "ghost" images: a unique case of visual allesthesia with hemianopsia in mitochondrial disease

A 49-year-old man with mitochondrial disease presented with visual allesthesia, a rare and puzzling phenomenon. He was admitted for treatment because of convulsions. After the convulsions ceased, he exhibited left homonymous hemianopsia. Brain diffusion-weighted magnetic resonance imaging (MRI) showed a high-intensity area in the right occipital lobe. Both the hemianopsia and the MRI activation in this area disappeared by day 36 of hospitalization. On the morning of day 57, right homonymous hemianopsia emerged in a singular manner. The patient perceived an illusory object (a bottle placed by the bedside) in his left visual field, while the real object was in his blind right field. This case of visual allesthesia was accompanied by palinopsia, ie, perseveration of the image of the bottle. Diffusion-weighted MRI showed a new, high-intensity area in the left occipital lobe. We believe the visual allesthesia resulted from transfer of cortical information obtained by blindsight between hemispheres as a result of epileptic excitation.

Revisiting Clinical Utility of Chest Radiography and Electrocardiogram to Determine Ischemic Stroke Subtypes: Special Reference on Vascular Pedicle Width and Maximal P-Wave Duration

Backgrounds: It is often difficult to diagnose stroke subtypes at admission, particularly in sinus rhythm cases. Vascular pedicle width (VPW) on chest X-ray (CXR) and maximal P-wave duration (P-max) on electrocardiogram (ECG) are again realized as useful parameters reflecting intravascular volume and atrial conduction status, respectively. We investigated the utility of VPW and P-max as a tool for differentiating ischemic stroke subtypes. Methods: We studied 343 acute stroke patients showing sinus rhythm on admission. Dividing the patients into cardioembolic (CE) stroke (n = 57) and non-CE (n = 286) groups, we compared clinical backgrounds including VPW on CXR, and P-max in lead II and premature atrial contraction (PAC) on 12-leads ECG. Then, we investigated the independent factors for CE. Results: Independent factors associated with CE were VPW (≥59.3 mm) (p < 0.001; odds ratio (OR), 10.12; 95% confidence interval (CI), 4.13-24.8), P-max in lead II (≥120 ms) (p < 0.001; OR, 8.61; 95% CI, 3.96-18.7), PAC (p = 0.002; OR, 7.35; 95% CI, 2.14-25.3) and D-dimer level (≥1.11 µg/ml) (p = 0.016; OR, 2.57; 95% CI, 1.20-5.51). Conclusions: VPW, P-max, PAC and D-dimer are useful parameters for diagnosing CE stroke in patients with sinus rhythm at admission.

Early clinical and radiological diagnosis of sporadic Creuzfeldt-Jakob’s disease (sCJD) — a case study of pathologically-proved sCJD MV2 —

Background Early clinical and radiological features of sCJD with long clinical illness have not been clarified yet. Subject An exceptioal case of pathlogically-proved sCJD MV2 exhibiting severe status spongiosus in the entire cerebral cortex and kuru-plaque with only mild degeneration in the thalami and basal ganglia. Total clinical illness was about four years. Method We compared clinical and radiological features chronologically. Results At the initial radiological study, about half a year after the initial symptom (forgetfulness), diffusion weighted magnetic resonance images (DW-MRI) revealed abnormal high signal along the occipito-parietal lobe cortex with

A 30-year-old male treated by neurosurgery against temporal lobe epilepsy.

The male patient was delivered by vacuum extraction at birth and he did not cry for a while. He had experienced fibrile convulsion at the age of 1 and there was nothing of note in his family history. Since the age of 7 years, the patient had sometimes noticed a nasty smell in his chest. At the age of 10 years, he experienced a fit of unconsciousness and after that he was medicated with anticonvulsive drugs until the age 18 years. Soon after stopping this medication, he experienced a sudden attack of complex partial seizures. Neuroradiological examination revealed hippocampal atrophy (Fig. 1), suggesting temporal lobe epilepsy. When he was 30 years old, he underwent neurosurgical treatment for intractable epilepsy, including hippocampal resection and lateral temporal lobe resection.