The lenticulostriate arteries (LSA) supply the lateral half of the head of the caudate nucleus, entire putamen, anterior limb, genu and the superior part of the internal capsule (IC) and a part of the corona radiata. The LSA consists with medial, intermediate and lateral branches. The medial branches perfuse the lateral segment of the globus pallidus, the head of the caudate nucleus and the anterior limb of the IC. The intermediate branches supply the anterior half of the LSA territory, while the lateral branches supply the posterior half. The anterior cerebral artery (ACA) perforators, predominantly Heubner’s artery, perfuse the inferomedial part of the caudate head, the anteromedial part of putamen, the anterior part of the lateral segment of the globus pallidus and anterior limb of the internal capsule. Such territories can be represented by the anterior and ventral basal ganglions. The anterior choroidal artery (AChA) gives off three main groups of branches including the lateral branches that supply the medial temporal lobe, the medial branches that supply the cerebral peduncle and the superior branches that supply the internal capsule and the basal ganglia. The superior branches are further discriminated into proximal branches that supply the anterior one third of the posterior limb of internal capsule (PLIC) and the medial segment of the globus pallidus and distal branches that supply the posterior two-third of PLIC, retro-lenticular part of the internal capsule and the lateral thalamic nuclei. The superficial penetrating arteries, i.e. medullary arteries, arise from the cortical branches of the middle cerebral artery (MCA) and supply the deep white matter. Infarcts caused by the medullary artery occlusion are located in the centrum-semiovale and half of them were caused by embolic mechanism. The centrum-semiovale corresponds to cortical border-zone (BZ) while the corona radiate corresponds to internal BZ.
Alzheimer’s disease (AD) and dementia with Lewy bodies (DLB) are two major types of dementia. Due to shared signs and symptoms, accurate diagnosis of these dementia subtypes is a clinical challenge. We assessed the sensitivity and specificity of the combined use of neuropsychological testing and brain imaging data for the differential diagnosis of these conditions. The study population included 77 patients with either AD or DLB. Ala score was calculated from Mini-Mental State Exam subscores, and the cingulate island sign score (CIScore) was obtained from image analysis of brain perfusion single-photon emission computed tomography. Correlation between Ala score and CIScore values was observed in the subgroup of patients aged ≤79 years (r = 0.485, P = 0.002), and the scatter plot revealed that 70% of DLB patients were within the range of cut-off values for DLB. In the group aged ≥80 years, there was poor correlation between the Ala and CIScores (r = 0.285, P = 0.083), the average CIScore exceeded the cut-off value, and the scatter plot showed lower sensitivity, illustrating the challenge of discriminating AD from DLB in an older patient population. The concurrent use of Ala score and CIScore enhanced the specificity and the area under the curve in both subgroups, indicating the improved ability of these tests to aid in the differential diagnosis of AD from DLB. Our findings suggest that the use of these methodologies in routine medical practice may increase the sensitivity and specificity of the diagnosis of dementia subtypes.
A small centrum ovale infarct in the territory of the white matter medullary artery can be caused not only by embolism but also small-vessel disease. In our study, thorough screening for emboligenic diseases was performed, including the modality of transesophageal echocardiography (TEE), in patients with an acute, isolated, small (less than 1.5 cm) infarct in the centrum ovale. Of 79 patients enrolled in this study, 45 had emboligenic diseases, in whom a patent foramen ovale was detected in 29 patients, complicated aortic arch lesion in 15, atrial fibrillation in 6, occlusive carotid disease in 2, and others in 2. The majority (80%) of the emboligenic diseases were diagnosed by TEE. Therefore, TEE may be mandatory for the etiologic diagnosis of centrum ovale infarcts.
A 64-year-old woman visited our hospital with early-onset dementia and progressive gait disturbance. She had demonstrated a mild communication disorder at the age of ~40 years; however, her psychiatric symptoms at that time were mild and were not accompanied by social problems. At the age of 59, she presented with memory loss, visual hallucinations, and delusions. Over the following five years she developed gait difficulties that gradually deteriorated and suffered frequent falls. On admission, neurological examinations revealed severe pyramidal and extrapyramidal signs of akinetic mutism. MRI of the brain showed cerebral atrophy, enlarged lateral ventricles, thinning of the corpus callosum, and leukoencephalopathy in the frontal-parietal lobes. Additionally, CT revealed a small spotty calcification in the frontal subcortical white matter. Genetic analysis revealed a single-base substitution (c.2330G>A/p.R777Q) in exon 18 of the colony stimulating factor 1 receptor (CSF1R) gene, encoding the CSF1R protein. She was diagnosed with hereditary diffuse leukoencephalopathy with spheroids (HDLS). HDLS is included in the differential diagnosis of early-onset dementia and should be considered in patients with mild personality change and abnormal behavior in the early course of the illness.
We report the case of a 76-year-old woman who presented with recurrent episodes of complex visual hallucinations in her right visual field without an anopsia. The electroencephalogram showed sharp transients in the left parietotemporal region with phase reversals at T5 and P3. FLAIR MRI revealed hyperintense lesions in the left temporo-occipital lobe, located mainly in the left inferior temporal lobe. Cerebral angiography revealed an arteriovenous shunt from the left occipital artery to the left transverse sinus with cortical venous reflux. The complex visual hallucinations were resolved after transarterial embolization. We therefore hypothesize that this patient’s complex visual hallucinations were caused by epileptic seizures or changes in cortical blood flow caused by the cortical venous reflux from the arteriovenous fistula. In general, epileptic hallucinations expand into the bilateral visual field. We reveal that in rare cases, complex visual hallucinations can also be limited to the unilateral visual field without an anopsia. Additionally, we reveal that a dural arteriovenous fistula can cause visual hallucinations without hemianopia.
We report a case of rheumatoid meningitis complicated with cryptococcal meningitis in a 59-year-old female with rheumatoid arthritis. Migraine symptoms were followed by abnormal behavior, and the patient was admitted with fever and headache. On admission, her cerebrospinal fluid (CSF) contained 115 cells/μl, a protein content of 95 mg/dl, and a sugar level of 47 mg/dl; Her serum anti-cyclic citrullinated peptide (CCP) antibody value was high (174 U/ml), and a brain MRI showed enhanced gadolinium lesions in the cerebral/cerebellar pia mater and subarachnoid space, etc. Probable rheumatoid meningitis was clinically diagnosed, and a prednisolone (PSL) pulse was started. Several days later, a CSF culture test was positive for Cryptococcus neoformans, and the antigen titer was 128-fold. Liposomal-amphotericin B (L-AMB) was started for cryptococcal meningitis, combined with three PSL pulses for rheumatoid meningitis. After about 4 weeks, the number of CSF cells and anti-CCP antibodies decreased rapidly. At 2 months after the onset, the meningitis recurred. The MRI contrast lesions reappeared, and the CSF cells increased to 24/μl. Serum and CSF anti-CCP antibodies increased at the time of recurrence, but the cryptococcal antigen titer decreased. Thus, we concluded that the rheumatoid meningitis mainly involved the pathogenesis of both types of meningitis. The number of PSL pulses was limited to four. Post-perioral therapy was avoided. Methotrexate was continued for the rheumatoid meningitis, fluconazole was continued for the cryptococcal meningitis, and neither type of meningitis has recurred.
We herein reported a patient with acute ischemic stroke in the bilateral medial medullary and the left tegmentum of the pons who presented with various neurological symptoms. Fusing digital subtraction angiography (DSA) and MRI (DSA-MR fusion imaging) could reveal the infarct-relevant arteries. A 41-year-old male presented with headache, bilateral arm’s dysesthesia, quadriplegia, left Horner’s syndrome, upbeat nystagmus, internuclear ophthalmoplegia and left peripheral facial paralysis. Diffusion weighted MRI (DWI) revealed the high intensity lesion in the bilateral medial medullary and the left tegmentum of the pons. MRA showed right vertebral artery (VA) occlusion. A high intensity on T1 weighted imaging was shown on the right VA vessel wall. DSA-MR fusion imaging revealed the anterior spinal artery (ASA) occlusion proximal to the infarction. The stenosis was located at the origin of the right VA perforating branch distributing into the infarct lesion. The steno-occlusive lesion of ASA and VA perforating branch due to VA dissection resulted in infarction in the pontomedullary junction and caused various neurological symptoms. DSA-MR fusion imaging would prove the radiological anatomy of infarct-relevant arteries and clarify the etiology of ischemic stroke.
An 81-year-old woman was admitted to our hospital due to paresthesia of the extremities and difficulty in walking for three months. She underwent a total hysterectomy and bilateral salpingo-oophorectomy for large cell neuroendocrine carcinoma (LCNEC) of the endometrium seven months before the admission. The serum levels of neuron specific enolase (NSE) reduced after the surgery. She showed numbness of her limbs, disturbance of vibration, areflexia and autonomic dysfunction. Nerve conduction studies showed sensory dominant sensory neuronopathy. CT scan of her abdomen and pelvis revealed the recurrence of LCNEC of the endometrium. The serum levels of NSE was elevated and anti-Hu antibody was also positive. Other laboratory test, including autoantibodies were unremarkable. We diagnosed her as paraneoplastic sensory neuronopathy associated with postoperative recurrence of LCNEC of the endometrium. Here we show a clinical picture of anti-Hu positive paraneoplastic neurological syndrome with LCNEC of the endometrium.
A 60-year-old, right-handed woman was admitted to our hospital for amnesia as the only neurological abnormal findings following the autonomic symptoms and transient episodes of loss of awareness. EEG during the amnesia showed rhythmic alpha activity arising from the left mid-temporal region. Although this ictal activity showed evolution in the frequency and amplitude, the location was limited in the bilateral temporal areas. After the EEG evaluation, her amnesia was resolved immediately, suggesting that her presentation was transient epileptic amnesia (TEA). Meanwhile, given the clinical course and MRI findings (high intensity in the bilateral mesial temporal areas, more on the left), she was diagnosed with non-herpetic limbic encephalitis and treated with steroid and anti-epileptic drugs, leading to the positive outcome. The ictal EEG findings during TEA as the one of the presentation in acute phase of non-herpetic limbic encephalitis may contribute to further investigation of underlying mechanism of TEA.