An infantile case of cerebral infarction associated with thrombocytosis]

Cerebral infarction in children is often caused by intracranial vascular disorder, cardiac disease, head injury, or infection, and is rarely induced by blood disease. In this paper, we describe an infantile case of cerebral infarction associated with thrombocytosis. A female infant of eight months of age developed left hemiparesis after a slight head injury. Her CT and MRI demonstrated a cerebral infarction located from the right internal capsule to the right corona radiata. Laboratory findings revealed iron-deficiency anemia and thrombocytosis with a platelet count 107.5 x 10(4)/mm3. Although she had no disorder that had caused iron deficiency, serum Fe value of the patient was low with a count of 18 micrograms/dl. Her bone marrow was normal except for a slight increase in the number of megakaryocytes. One month later, her anemia was improved by means of oral iron replacement. However, her platelet count remained at more than 100 x 10(4)/mm3 as it had been before. Her condition of left-sided hemiparesis gradually improved by a program of rehabilitation, and did not recur after aspirin administration. Although the main cause of her thrombocytosis that led to a transient cerebrovascular accident is obscure, it is postulated that her iron deficiency anemia induced secondary thrombocytosis, or else the patient had essential thrombocytosis.     

A case of brain infarction with nephrotic syndrome]

A 47-year-old man lost his consciousness and brought to our hospital by ambulance. On admission, he had aphasia and upper right limb paresis. Diffusion weighted MR image of the brain on admission showed multiple high intensity areas in the left middle cerebral artery (MCA) territory. Brain angiography performed on the 2nd hospital day revealed the left MCA severe stenosis. We started intravenous antithrombotic therapy on the 1st day. The left carotid angiography on 12th day demonstrated that the left MCA stenosis was improved. He had medical history of hypertension, diabetes mellitus and gout. But he had only slight atherosclerosis, and had no arrhythmia and patent foramen ovale. Blood chemistry test showed marked hypoproteinemia and hyperlipidemia, and urine examination showed proteinuria. He was diagnosed as nephrotic syndrome for the first time. Nephrotic syndrome brought hypercoagulability, so we suspected that nephrotic syndrome concerned with brain infarction.     

A case of Turner syndrome complicated with brain infarction]

A 21 year-old female college student with a history of Turner syndrome at age 9, and 6-year growth hormone replacement therapy noticed weakness of right extremities when she got up on March 26, 2001. On admission, she showed right hemiparesis (4+/5) and hypesthesia on the right of body. The hemiparesis progressed (3-/5) in spite of antithrombotic therapy. Brain MRI revealed a high intensity lesion with a diameter of 1.5 cm in the posterior limb of the left internal capsule and putamen on DWI and T2WI. MR angiography and TC-CFI revealed no stenosis in her left middle cerebral artery, but > 50% stenosis in the horizontal portion (M1) of her right middle cerebral artery. Branch lesions were presumed to exist in the left M1. Non-atherosclerotic angiopathy, coagulopathy, and other conventional risk factors of brain infarction were not found. Pathogenesis of Turner syndrome might have played a role in the development of brain infarction in this patient.     

A case of hemodynamic brain infarction with postprandial hypotension]

A 76-year-old man with left internal carotid artery occlusion developed a progressing right hemiparesis. Brain MRI presented reinfarctions in the left anterior border zone and terminal zone in the left deep white matter. Ambulatory blood pressure monitoring showed a decrease in systolic blood pressure by more than 20 mmHg one hour after starting meals, which is considered as postprandial hypotension. The recurrent stroke occurred probably by a hemodynamic mechanism with the presence of internal carotid artery occlusion and postprandial hypotension. Administration of voglibose, an alpha-glucosidase inhibitor, improved postprandial hypotension. In patients with severe carotid or intracranial artery disease, the postprandial hypotension should be carefully monitored for prevention of hemodynamic brain ischemia.     

A case of brain stem infarction with bilateral hearing loss]

The study case was a 66-year-old man who had bilateral neurosensory hearing impairment due to brain stem infarctions. He noticed mild hearing loss, frequent vertigo and tinnitus. About one month later, his hearing took a sudden turn for the worse, and he suffered from dysarthria, dysphagea and abasia. Neurological examination revealed pseudobulber palsy, left hemiparesis, cerebeller ataxia, disturbance of pain and temperature sensation on the right face and left side of the body. Brain stem auditory evoked potentials (BAEPs) showed a delayed small wave V with the abscence of previous waves on the right side and no significant waves on the left side. Brain magnetic resonance images (MRI) revealed infarctions in the bilateral middle cerebellar peduncles, including in the right lateral portions of pons, and the right lower pontine base. We believe that not only peripheral, but also central auditory pathways adjacent to infarctions were damaged. Magnetic resonance angiography (MRA) showed severe stenosis or occlusion of left vertebral artery and basilar artery. We concluded that hypoperfusion of the vertebrobasilar artery territories caused ischemia of the cochlear nerve and the auditory tracts in the brain stem, which resulted in bilateral hearing loss.     

An autopsy case of catastrophic antiphospholipid syndrome presenting with recurrent multiple cerebral infarction associated with lung cancer]

We reported an autopsy case of cerebral infarction with primary lung cancer. The patient was a 50-year-old man. Despite having been treated with warfarin potassium and ticlopidine hydrochloride, he relapsed cerebral infarction. His laboratory data on admission showed that lupus anticoagulant was positive, together with a high value of beta-thromboglobulin, thrombin-antithrombin III complex, markers of platelet and coagulation activation, CEA and CA 19-9. The autopsy finding revealed a primary papillary adenocarcinoma in the right lower lung, multiple cerebral infarction, renal infarction, pulmonary infarction and splenic infarction. The atherosclerotic changes were mild in the whole tissues and findings of vasculitis were not observed. Recurrence of cerebral infarction was effectively suppressed with the addition of steroid therapy to antithrombotic therapy. This case was considered as catastrophic antiphospholipid syndrome. It is necessary to differentiate antiphospholipid syndrome in case of the abnormal coagulation and fibrinolytic factors with recurrent cerebral infarction. Moreover, systemic examinations are important, because malignant tumor may exist on the background of the case.     

An operated case of bitemporal lobe epilepsy associated with old infarction in the left occipital lobe]

A 15-year-old female, who presented with bitemporal lobe epilepsy associated with old infarction in the left occipital lobe, was reported. MRI with fluid attenuated inversion recovery sequence demonstrates cortical atrophy with hyperintensity of the white matter in the left occipital lobe as well as volume loss and hyperintensity of the left hippocampus. Interictal positron emission tomography with [18F]fluorodeoxy glucose (FDG-PET) and single photon emission computed tomography with technetium-99m-ethyl-cysteinate dimer indicate hypometabolism and hypoperfusion in the left occipital lobe and the left temporal lobe, respectively. Scalp recorded EEG did not lateralize the side of the epileptogenic zone. Chronic subdural electrode recording demonstrated that the ictal onset zones were located in the bilateral side of the temporal lobe. Eighty-nine percent of 19 spontaneous seizures were left sided onset. The anterior temporal lobectomy with hippocampectomy was performed for the left side. Although temporal lobe epilepsy is sometimes a bilateral disease, unilateral lobectomy for a strong predominant side, based on the MRI and FDG-PET findings, is effective for some patients.     

Two cases of brain infarction associated with dissection of the thoracic aorta]

Case 1: a 57-year-old woman, who suddenly developed disturbance of consciousness, left spatial neglect, and left hemiparesis, was admitted to our hospital on the day 1. Brain CT scan on the day 2 revealed broad edematous infarction in her middle cerebral artery territory. The dissection of the thoracic aorta was observed with transesophageal echocardiography and brain embolism due to mural thrombus of the dissecting thoracic aorta was suspected. Case 2: a 67-year-old woman, who developed disturbance of consciousness, left spatial neglect, and left hemiparesis, was admitted to our hospital on the day 15. Brain CT scan on the day 15 showed non-edematous infarction in her middle cerebral artery territory. Contrast thoracic CT scan on the day 31 revealed the dissection of the thoracic aorta. No clinical exacerbation was observed, but brain CT scan on the day 94 showed broad infarction in her middle and anterior cerebral artery territories. It is considered that pseudolumen of the dissected thoracic aorta caused occlusion of her right internal carotid artery. Transesophageal echocardiography, or contrast thoracic CT scan should be considered for detection of the dissection of the thoracic aorta in the cases of cryptogenic stroke.     

A case of brain infarction associated with viral encephalitis: MRI and pathological findings

A 10-year-old girl had an infarction in the left brain during an acute viral meningoencephalitis. She initially showed seizure, unconsciousness and fever, and right hemiplegia gradually developed. She died at the 48th day of disease from respiratory disturbance and renal failure. Cranial MRI during the acute phase of the disease, when there was no clinical sign of right hemiplegia, showed a high intensity lesion in the left parietal and occipital areas on T-2 weight image. Autopsy disclosed the findings suggesting viral encephalitis, including multiple focal necrosis, perivascular round cell infiltration, proliferation of glial cells and spongy degeneration with some intranuclear inclusion bodies, and infarction in the left hemisphere. These findings suggest that T-2 high intensity lesion on MRI reflected infarction.     

An autopsy case of miliary brain metastases]

A 57-year-old man was admitted to our hospital with a diagnosis of psychiatric emergency. His symptoms were similar to encephalitis, metabolic encephalopathy or acute depressive psychosis because of poor focal neurological signs. Laboratory examinations, including routine hematological and biochemical investigations, serum vitamin B1 B12 levels, and cerebrospinal fluid obtained by lumbar puncture, were normal. Brain CT was also normal, therefore it was difficult to make a diagnosis. But, we could clinically diagnose him as having pulmonary adenocarcinoma with numerous metastatic nodules of the brain. Because miliary lesions in the cerebral hemispheres, brainstem and cerebellum were disclosed on brain MRI. Furthermore, chest CT revealed the lung tumor in the left S8 area. In addition, laboratory examination showed a rise of tumor marker and cytologic examination of sputum revealed class V. Fluid-attenuated inversion recovery and contrast-enhanced MR images demonstrated more prominently miliary metastases, in particular lesions in the cerebral cortex, than T1- and T2-weighted images. There was neither edema in the surrounding region of metastatic nodules nor mass effect on all MR images. Spinal MRI showed no metastatic lesions. The patient died of respiratory failure at the age of 58, about eight months after the disease onset. The brain weighed 1,575 g. Neuropathological findings revealed diffuse miliary brain metastases located in all parts of the brain, except for the medulla oblongata. Histological examination disclosed multiple metastases from a well-differentiated adenocarcinoma with a predominant tubular pattern. There was neither edema nor glial reaction in the surrounding area of metastatic lesions. Many pseudorosettes were recognized and carcinoma cells, extending through perivascular spaces into the subarachnoid space, were noticed.     

Two cases of brain infarction associated with hypertriglyceridemia

We reported two cases of brain infarction. They were cousins. Case 1 was a 12-year-old girl, who complained of aphasia, dyscalculia, right-left disorientation and right homonymous hemianopsia. CT showed low density areas in left superior and middle temporal gyri. Case 2 was a 15-year-old boy, who had left hemiplegia and hypesthesia to pain, temperature and touch on the left side of the body. CT showed low density areas from the genu of the internal capsule to the corona radiata, and from the posterior portion of putamen to the posterior limb of the internal capsule on the right side. Both cases had hypertriglyceridemia which might be associated with the etiology of infarction.     

Diagnostic criteria of neurosarcoidosis]

We have collaborated with Japanese Sarcoidosis Society and Japanese Society of Respiratory diseases to make a new diagnostic criteria of neurosarcoidosis. (definite) Having positive clinical findings which suggest a neurosarcoidosis. Pathology proven case. (probable) Having positive clinical findings which suggest a neurosarcoidosis. Pathology proven in other organ. Elevated serum ACE or BHL demonstrated by chest XP or CT scan. (possible) Having positive clinical findings which suggest a neurosarcoidosis. Elevated serum ACE or BHL demonstrated by chest XP or CT scan. CNS sarcoidosis, sarcoid neuropathy and sarcoid myopathy are separately diagnosed. To evaluate our criteria, about 100 papers on neurosarcoidosis published worldwide in these 6 years were retrospectively examined. Our criteria showed a better results than 1988 criteria (former Japanese criteria) and Zajicek's criteria which was published in 1999 (QJ Med 92: 103). Point is there is no more need to depend upon Kveim reaction which is time consuming, not so specific and difficult to obtain its antigen. Our new criteria is calculated to be sensitivity of 78-98% (BHL or ACE), and specificity of 83-99% (ACE). Because of their low specificity, Gallium scintigraphy and broncho alveolar lavage fluid examination are excluded from our diagnostic criteria, however, their high sensitivity are still useful to begin with.     

Diagnostic criteria of neurosarcoidosis]

We have collaborated with Japanese Sarcoidosis Society and Japanese Society of Respiratory Disease to provide a new diagnostic criteria of neurosarcoidosis. At least two of six examinations are important. These include bilateral hilar lymphadenopathy, abnormal uptake of 67Ga scintigraphy, broncho alveolar lavage fluid examination, elevated serum ACE, negative tuberculin reaction, and elevated serum or urinary calcium level. (definite) Having positive clinical findings which suggest a neurosarcoidosis. Pathology proven case. (probable) Having positive clinical findings which suggest a neurosarcoidosis. Pathology proven in other organ. At least two of six examinations are positive. (possible) Having positive clinical findings which suggest a neurosarcoidosis. At least two of six examinations are positive. CNS sarcoidosis, sarcoid neuroopathy and sarcoid myopathy are separately diagnosed. Kveim reaction which is time consuming, not so specific, and difficult to obtain its antigen is no more necessary.     

Venous infarction associated with carotid-cavernous fistula]

We report an 88-year-old woman who developed a hemorrhagic venous infarction in the left cerebral hemisphere and brainstem, in association with left carotid-cavernous fistula (CCF). Without aura the patient noticed diplopia due to left abducens palsy, and exophthal mos and congestion of the left eye. Brain CT revealed extrusion of the left eye, and dilatation of left superior orbital vein and cerebral cortical veins. She received diagnosis of CCF. Brain CT also revealed a small mass in the left ethmoidal sinus, which was not attached to the CCF. Biopsy of the mass was done under local anesthesia. On the following she had high fever. Her consciousness level deteriorated and she developed right hemiparesis FLAIR images of MRI showed, extensive high signal lesions in the left frontal and temporal cortices, basal ganglia, thalamus, midbrain and pons. These findings were consisted with venous infarction, possibly associated with peri-operative infection and hypovolemia. Intracranial hemorrhage occur in 3% of cases with CCF, but venous infarction was much rarer. The patients with CCF, who show dilatation of cortical veins in CT or MRI, have a higher risk of cerebral hemorrhage or infarction, and should be carefully observed.     

Remission of refractory neurosarcoidosis treated with brain radiotherapy: a case report and a literature review

Sarcoidosis is a chronic disease of unknown etiology characterized by the presence of T lymphocytes, mononuclear phagocytes, and noncaseating epithelioid granulomas in the tissues. Central nervous system involvement occurs in about 5% of the cases. The chronic form of neurosarcoidosis is particularly resistant to medical treatments. No universally accepted therapeutic protocols are currently available. Corticosteroids are the first line of therapy, but other immunosuppressive treatments are frequently added to the patient's regimen, although this strategy is not adequately supported by controlled clinical trials. For patients resistant to or not tolerating multiple alternate immunotherapeutic drugs, some authors suggest central nervous system radiotherapy. We present a case of a patient with neurosarcoidosis involving the hypothalamo-hypophyseal region and causing panhypopituitarism who had a poor response to and experienced severe side effects from conventional immunosuppressive treatments. The patient experienced a good clinical response to cranial irradiation. We review the literature on this subject.     

Juvenile cerebral infarction associated with heparin cofactor II abnormality. A case report]

A 15-year-old woman with a history of transient dysarthria two years before, suddenly developed weakness of right upper extremity, right facial palsy, and dysarthria. She was admitted to our hospital on the third day. She had no hypertension, heart murmur and oedema. On neurological examination, she had mild right hemiparesis including face muscles and mild dysarthria. The right knee jerk was brisk with no Babinski's sign. Ataxia and sensory disturbance were not present. T2-weighted MRI showed a hyperintensity at the posterior limb of the left internal capsule. Cerebral angiography was unremarkable. Ultracardiography and 24-hour electrocardiography were normal. Laboratory data revealed no inflammatory findings, liver dysfunction, hyperglycemia and hyperlipidemia. Antinuclear and anticardiolipin antibodies were negative. Prothrombin time was normal, but activated partial thromboplastin time was slightly prolonged (35.4 sec, normal 25.2-34.4). Protein C, protein S and antithrombin III were normal. Heparin cofactor II (HC II) activity was decreased (44%) with normal HC II antigen (79%) and so she was diagnosed as heparin cofactor II deficiency type II (heparin cofactor II abnormality). Her father manifesting thromboangitis obliterans also had low HC II activity with normal HC II antigen. However, on her genetic analysis, we didn't detect any mutations in the coding region of HC II gene. Until now she has no recurrence of cerebrovascular attacks. On the basis of these results, we suspect that HC II deficiency was a possible risk factor of cerebral infarction in this case because she was so young and had no general risk factors except for HC II. No stroke associated with HC II deficiency type II has been reported up to date. This case is worth considering etiologies of juvenile cerebral infarction.     

A case of cardioembolic brain infarction in arrhythmogenic right ventricular dysplasia]

We report a 45-year-old woman with arrhythmogenic right ventricular dysplasia (ARVD). Because of congestive heart failure and atrial fibrillation, she underwent tricuspid valvular replacement and warfarin was prescribed. She suddenly had dysarthria, left hemiparesis and left hemispatial neglect. After brain CT examination, and cerebral angiography, she was diagnosed as cardiogenic brain embolism and infusion of low molecular heparin was started. On day 25, she suddenly had ventricular tachycardia and died in spite of treatment for arrhythmia. This is the first report of the case of cardiogenic brain embolism following ARVD. In this type of case, we must take care of arrhythmia besides the management of atrial fibrillation and brain infarction.     

An operated case of medial temporal lobe epilepsy associated with schizencephaly]

A 24-year-old male of medial temporal lobe epilepsy associated with schizencephaly was presented. He developed complex partial seizure after head trauma at the age of a year and 7 months, which became intractable at the age of 13 year. MRI demonstrated a schizencephalic cleft in the right peri-Rolandic area, cortical dysplasia in the right medical parietal and occipital lobes, and right hippocampal atrophy. Scalp-recorded EEG failed to localize the ictal onset zone. Interictal FDG-PET and ECD-SPECT indicated hypometabolism and hypoperfusion of the right entire temporal lobe, and ictal ECD-SPECT increased perfusion of this area. Chronic subdural electrode recording clearly demonstrated that ictal onset zone was located not on the schizencephalic cleft or its surrounding cortex but on the right medial temporal lobe. Following right anterior temporal lobectomy with hipppocampectomy, seizure control became easy. For the identification of the epileptogenic zone in patients with schizencephaly, chronic subdural electrode recording is mandatory.