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BACKGROUND: Researchers find it difficult to distinguish between depression with ASD (Depress-wASD) and without ASD (Depression) in adult patients. We aimed to clarify the differences in brain connectivity between patients with depression with ASD and without ASD. METHODS: From April 2017 to February 2019, 22 patients with suspected depression were admitted to the hospital for diagnosis or follow-up and met the inclusion criteria. The diagnosis was determined according to the Diagnostic and Statistical Manual of Mental Disorders-5 by skilled psychiatrists. The Hamilton Depression Rating Scale (HAM-D), Young Mania Raging Scale (YMRS), Mini-International Neuropsychiatric Interview, Parent-interview ASD Rating Scale-Text Revision (PARS-TR), and Autism-Spectrum Quotient-Japanese version (AQ-J) were used to assess the patients' background and help with diagnosis. Resting-state functional magnetic resonance imaging (rs-fMRI) was performed using the 3-T-MRI system. rs-fMRI was processed using the CONN functional connectivity toolbox. Voxel-based morphometry was performed using structural images. RESULTS: No significant difference was observed between the Depress-wASD and Depression groups using the HAM-D, YMRS, AQ-J, Intelligence Quotient (IQ), and verbal IQ results. rs-fMRI for the Depress-wASD group indicated a positive connection between the salience network (SN) and right supramarginal gyrus (SMG) and a negative connection between the SN and hippocampus and para-hippocampus than that for the Depression group. No significant structural differences were observed between the groups. CONCLUSIONS: We identified differences in the SN involving the SMG and hippocampal regions between the Depress-wASD and Depression groups.

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BACKGROUND: Patients with superficial siderosis (SS) rarely show brachial multisegmental amyotrophy with ventral intraspinal fluid collection accompanied with dural tear. CASE PRESENTATION: We describe spinal cord pathology of a 58-year-old man who developed brachial multisegmental amyotrophy with ventral intraspinal fluid collection from the cervical to lumbar spinal levels accompanied with SS, dural tear, and snake-eyes appearance on magnetic resonance imaging (MRI). Radiological and pathological analyses detected diffuse and prominent superficial deposition of hemosiderin in the central nervous system. Snake-eyes appearance on MRI expanded from the C3 to C7 spinal levels without apparent cervical canal stenosis. Pathologically, severe neuronal loss at both anterior horns and intermediate zone was expanded from the upper cervical (C3) to middle thoracic (Th5) spinal gray matter, and these findings were similar to compressive myelopathy. CONCLUSION: Extensive damage of the anterior horns in our patient may be due to dynamic compression induced by ventral intraspinal fluid collection.

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An encephalocele is a pathological brain herniation caused by osseous dural defects. Encephaloceles are known to be regions of epileptogenic foci. We describe the case of a 44-year-old woman with refractory epilepsy associated with a frontal skull base encephalocele. Epilepsy surgery for encephalocele resection was performed; however, the epilepsy was refractory. A second epilepsy surgery for frontal lobectomy using intraoperative electroencephalography was required to achieve adequate seizure control. Previous reports have shown that only encephalocele resection can result in good seizure control, and refractory epilepsy due to frontal lobe encephalocele has rarely been reported. To the best of our knowledge, this is the first report of frontal encephalocele plus epilepsy in which good seizure control using only encephalocele resection was difficult to achieve. Herein, we describe the possible mechanisms of encephalocele plus epilepsy and the surgical strategy for refractory epilepsy with encephalocele, including a literature review.

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Pathophysiological difference of depression in patients with and without autistic spectrum disorder (ASD) has not been investigated previously. Therefore, we sought to determine whether there were differences between non-ASD and ASD groups on resting-state functional magnetic resonance imaging (rs-fMRI) in patients with depression. We performed 3T MRI under resting state in 8 patients with depression and ASD and 12 patients with depression but without ASD. The ASD group showed increased functional connectivity in the cerebellar network of the left posterior inferior temporal gyrus and anterior cerebellar lobes compared to the non-ASD group in an analysis of covariance. Adding antipsychotics, antidepressants, benzodiazepines, nonbenzodiazepines, anxiolytics, hypnotics, or age as covariates showed a similar increase in functional connectivity. Thus, this study found that depressive patients with ASD had increased functional connectivity in the cerebellar network. Our findings suggest that fMRI may be able to evaluate differences in depressed patients with and without ASD.

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A 65-year-old woman presented with slowly progressive aphasia with gait disturbance associated with parkinsonism. She experienced a fall that resulted in a brain trauma. Brain imaging revealed a small amount of subarachnoid hemorrhage (SAH) with intraventricular bleeding. Despite conservative therapy, gait disturbance and hyporesponsiveness gradually deteriorated following that brain trauma. One month later, she was transferred to our hospital, and magnetic resonance imaging revealed prominent communicating hydrocephalus. A ventriculoperitoneal shunt and brain biopsy were performed. Neurosurgical intervention did not improve the patient's neurological condition. Clinical-pathological analysis confirmed the diagnosis of corticobasal degeneration (CBD) as an underlying disease relating to parkinsonism and aphasia. In patients with parkinsonism with high risks of falling, attention should be paid to neurological deterioration due to traumatic SAH-related hydrocephalus. Particularly, in patients with aphasia such as in those with CBD, delayed detection of posttraumatic complications might cause poor responsiveness to surgical intervention.

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INTRODUCTION: Cognitive function is an important factor that affects functional recovery after hip fracture (HipFx) surgery. The literature on the pathophysiology of dementia in HipFx patients is scarce. We performed a differential diagnosis of dementia in HipFx patients using clinical and brain MRI findings. METHODS: This is a prospective study in which brain MRI was evaluated for patients with HipFx for research purposes. One-hundred-and-five HipFx patients (85 females and 20 males) who underwent surgery and were subsequently able to undergo brain MRI at our hospital were evaluated. The mean age was 84 years. The presence of dementia was determined based on clinical findings and whether the patient meets its diagnostic criteria according to the International Classification of Diseases 10th Edition (ICD-10). The differential diagnosis of dementia was made based on brain MRI findings and the dementia diagnostic flow chart published in the Clinical Practice Guideline for Dementia 2017 (Japanese Society of Neurology). The Voxel-based Specific Regional Analysis System for Alzheimer's Disease (VSRAD) advance 2 diagnostic software was used to evaluate atrophy of the para-hippocampal gyrus. RESULTS: Fifty-six (53%) patients were clinically diagnosed with dementia according to the ICD-10 criteria. The MRI findings were diverse: Alzheimer's disease (AD)-type, asymptomatic multiple ischemic cerebral lesions, past symptomatic cerebral infarction or cerebral hemorrhage, Binswanger's disease (BW)-type, chronic subdural hematoma, disproportionately enlarged subarachnoidal hydrocephalus (DESH), and their combinations thereof. A combination of MRI and clinical findings of dementia patients demonstrated the following distribution of dementia subtypes: AD (n = 20), vascular dementia (n = 33), AD and BW vascular dementia (n = 3). CONCLUSION: This study revealed that the brain MRI findings of HipFx patients were diverse. Although vascular dementia is found to be common in this particular population, this could be an incidental finding. Further study is warranted to clarify the specificity of our findings by increasing the number of patients, setting the control, and investigating whether dementia subtypes affect postoperative gait acquisition and fall risk.

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A 49-year-old Japanese man had shown developmental delay, learning difficulties, epilepsy, and slowly progressive gait disturbance in elementary school. At 46 years old, he experienced repeated drowsiness with or without generalized convulsions, and hyperammonemia was detected. Brain magnetic resonance imaging detected multiple cerebral white matter lesions. An electroencephalogram showed diffuse slow basic activities with 2- to 3-Hz δ waves. Genetic tests confirmed a diagnosis of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Leukoencephalopathy was resolved following the administration of L-arginine and lactulose with a decrease in plasma ammonia levels and glutamine-glutamate peak on magnetic resonance spectroscopy. Leukoencephalopathy in HHH syndrome may be reversible with the resolution of hyperammonemia-induced glutamine toxicity.

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BACKGROUND: Postoperative intracranial complications are rare in spine surgery not including cranial procedures. We describe an uncommon case of pseudohypoxic brain swelling (PHBS) and secondary hydrocephalus after transforaminal lumbar interbody fusion (TLIF) presenting as impaired consciousness and repeated seizures. CASE PRESENTATION: A 65-year-old man underwent L4-5 TLIF for lumbar spondylolisthesis and began experiencing generalized seizures immediately postoperatively. Computed tomography (CT) revealed diffuse cerebral edema-like hypoxic ischemic encephalopathy. He was transported to our hospital, at which time epidural drainage was halted and anti-edema therapy was commenced. His impaired consciousness improved. However, he suffered secondary hydrocephalus due to continuous bleeding from a dural defect and spinal epidural fluid collection 3 months later. Following the completion of dural repair and insertion of a ventriculoperitoneal shunt, his neurologic symptoms and neuroimaging findings improved significantly. CONCLUSIONS: PHBS can be considered in patients with unexpected neurological deterioration following lumbar spine surgery even with the absence of documented durotomy. This might be due to postoperative intracranial hypotension-associated venous congestion, and to be distinguished from the more common postoperative cerebral ischemic events-caused by arterial or venous occlusions-or anesthetics complications.

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OBJECTIVES: Salivary dysfunction, such as reduced salivary flow and an altered salivary composition, is caused by several diseases, medical conditions, and medications. The purpose of the present study was to clarify the relationship between hypertension and morphological changes in the submandibular glands. MATERIALS AND METHODS: An epidemiological study was conducted to elucidate the relationship between hypertension and dry mouth. The effects of hypertension on morphological changes and the intima thickness of arteries in the submandibular glands were histopathologically investigated. RESULTS: Among 1933 subjects in the epidemiological study, 155 (8.0%) had dry mouth. A multivariate analysis revealed that dry mouth correlated with age (p < 0.001), sex (p < 0.001), and hypertension (p < 0.05). No significant differences were observed in the size of the submandibular glands between patients with or without hypertension. The average area of acinar cells was smaller in patients with than in those without hypertension (0.366 ± 0.153 vs. 0.465 ± 0.178, p < 0.05). The arteriosclerotic index was significantly higher in patients with than in those without hypertension (0.304 ± 0.034 vs 0.475 ± 0.053, p < 0.05). CONCLUSIONS: Hypertension may contribute to the degeneration of the submandibular glands by decreasing the number of acinar cells and promoting fatty infiltration and stenosis of the arteries. CLINICAL RELEVANCE: There may be a correlation between hypertension and the degeneration of the submandibular glands by decreasing the number of acinar cells and promoting fatty infiltration and stenosis of the arteries.

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Primary central nervous system lymphoma (PCNSL) sometimes occurs in immune-compromised hosts or patients with autoimmune diseases. Some cohort studies have previously reported an increased risk of non-Hodgkin's lymphoma in systemic lupus erythematosus (SLE), while some cases of PCNSL in patients with SLE were reported. We present the case of PCNSL which developed in a patient with the active phase of neuropsychiatric SLE (NPSLE). Furthermore, we reviewed published English articles to confirm the characteristics of PCNSL related to SLE. To our knowledge, this is the first report of PCNSL occurring in NPSLE. Histology demonstrated B-cell lymphoma with a positive Epstein-Barr virus-encoded RNA. This patient recovered following surgical resection of the lymphoma, whole brain radiation therapy, intravenous infusion of rituximab (RTX), and administration of belimumab after RTX. Given the series of reviews, our report suggests that the persistence of damage in the central nervous system (CNS) and long-term exposure to immunosuppressants may impact oncogenic immune responses within the CNS, leading to PCNSL development.

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BACKGROUND: Generally, asymptomatic epidermoid cysts (ECs) or ECs with minor symptoms controlled by medication are not treated further. Although epidermoid carcinomas are only sporadically reported, the possibility of malignant transformation of ECs is of concern. CASE DESCRIPTION: A 77-year-old man under observation for a left cerebellopontine angle tumor presented with no symptoms, including headache, dizziness, or other cranial nerve abnormalities typical of cerebellopontine angle tumors. The tumor manifested as an extra-axial mass in the left cerebellopontine angle and measured 24 × 16 mm (length × width) on magnetic resonance imaging (MRI). As he was asymptomatic, his primary physician did not pursue invasive treatment but ordered follow-up MRI every 2 years. The tumor decreased in size 2 years after the initial MRI, and no tumor fragmentation was observed on the patient's spinal MRI. Ultimately, the tumor shrank to 19 × 11 mm over 22 years. CONCLUSIONS: The reduction of an EC is extremely rare. We report a case of spontaneous regression of an EC and consider similar previous cases.

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A 36-year-old woman visited a local hospital suffering from acute onset dizziness. Brain MRI revealed multiple white matter lesions without gadolinium enhancement in the both hemispheres. Although she began to receive a treatment under a clinical diagnosis of multiple sclerosis, she developed newly emerging brain lesions and was referred to our hospital. Neurological examination detected intention tremor, right-sided dysdiadochokinesis, and gait ataxia. Both blood and cerebrospinal fluid tests were unremarkable but follow-up brain MRIs showed rapidly relapsing and remitting lesions. The first brain biopsy ended up showing non-specific changes but the second biopsy with five months interval confirmed primary central nervous system lymphoma (PCNSL). The patient was treated by chemotherapy and showed partial response. It is important to consider sequential brain biopsies if needed because PCNSL may present diverse brain lesions on MRI including non-neoplastic early lesions.

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Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal tumor in children and a rare entity. Although adult AT/RT is extremely rare, some cases of adult-onset AT/RT in the sellar region have been described. Here, we report an adult patient with AT/RT of the sellar region in whom it was difficult to make a definitive diagnosis and the clinical course was aggressive. This is the first report of autopsy findings that could confirm the clinical characteristics of this rare unresolved pathology, and will contribute to the improvement of prognosis. In addition, a literature review was performed to clarify this exceptionally rare condition. Interestingly, all reported adult patients with sellar AT/RT, along with the present case, were female. This raises the possibility of its being a sex-related disease. However, further studies are required to come to a definitive conclusion.

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There have been few studies regarding physical training-induced peripheral nerve dysfunction in patients with hereditary neuropathy with liability to pressure palsies (HNPP), with the exception of soldiers that trained intensively. Here, we report a 15-year-old boy without family history of HNPP who developed bilateral painless brachial plexopathy following short-term barbell and plank training during a school baseball club activity. Muscle training-induced painless brachial plexopathy could be an initial symptom and may be underdiagnosed in adolescents with sporadic HNPP.

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Crossed cerebellar diaschisis (CCD) is an interesting phenomenon which classically refers to the depressed blood flow and metabolism affecting one cerebellar hemisphere after a contralateral hemispheric infarction. However, CCD can also be caused by a prolonged seizure. We herein report a case of CCD due to status epilepticus in a patient who showed unique magnetic resonance imaging findings.

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BACKGROUND: It is well known that congenital cytomegalovirus infection exhibits white matter and other types of lesions in magnetic resonance imaging (MRI), but little is known on the clinical significance of white matter lesions because they are also present in asymptomatic congenital cytomegalovirus infection. We investigated for relationships among white matter lesions, intelligence quotient, and other neurodevelopmental features. METHODS: Nine children (five boys and four girls; mean age: 87.4 months, range: 63-127 months) with sensorineural hearing loss (five bilateral and four unilateral) had been diagnosed as having congenital cytomegalovirus infection by positive polymerase chain reaction findings of dried umbilical cords. They were evaluated for the presence of autistic features, tested using Wechsler Intelligence Scale for Children-Fourth Edition for intelligence quotient, and underwent brain MRI to measure white matter lesion localization and volume. RESULTS: At the time of MRI examination (mean age: 69.4 months, range: 19-92 months), white matter lesions were detected in eight of nine patients. Five subjects were diagnosed as having autism spectrum disorders. We observed increased white matter lesion volume was associated with lower intelligence quotient scores (R(2) = 0.533, P = 0.026) but not with autism spectrum disorders. CONCLUSIONS: In individuals with congenital cytomegalovirus, an increased white matter lesion volume is associated with lower intelligence quotient scores but not with an increased likelihood of autistic behavior.

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AIM: This study investigated whether the characteristic changes in hippocampal atrophy seen in coronal scans are useful for differentiating Alzheimer's disease (AD), amnestic mild cognitive impairment (aMCI), and major depressive disorder (MDD). METHODS: Subjects included 58 patients with AD, 33 with aMCI, 20 with MDD, and 22 normal controls, all aged 60 years or older. For each subject, eight coronal short TI inversion recovery images perpendicular to the hippocampal longitudinal axis were obtained. Images were manually measured using the conventional region of interest method of quantitative analysis. RESULTS: The overall trend in the corrected volumes of the hippocampus was AD < aMCI < MDD < normal controls. We found atrophy in all slices in AD, atrophy centred on the hippocampal head in aMCI, and atrophy in the slice of the hippocampal body 12 mm from the amygdala in MDD. CONCLUSIONS: The present study suggested that our method of comparing hippocampal atrophy by region may be useful in distinguishing AD, aMCI, MDD, and normal controls.

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