OBJECTIVE: Paraneoplastic neurological disorders associated with autoantibodies are rare diseases, causing abnormal manifestations in the central or peripheral nervous system separately or simultaneously. Early recognizing the occurrence of paraneoplastic syndrome can lead to prompt and effective management.
METHODS: We presented a patient of subacute cerebellar degeneration with cachectic and bed-ridden status, who was proven to have positive SOX1 antibody. A coexisting Lambert-Eaton myasthenic syndrome was also documented by electrophysiological study.
CONCLUSIONS: Intensive and regular follow up for an occult malignancy is crucial in patients with SOX1 antibody. Coadministration of therapies for underlying malignancy and LEMS improve the functional disability.

BACKGROUND: Several brain structures, including the orbital prefrontal cortex, ventrolateral prefrontal cortex, dorsolateral prefrontal cortex, amygdala, and anterior cingulate cortex, are considered key structures in the neural circuitry underlying emotion regulation. We report on a patient showing behavior changes and degeneration of core neural tracts for emotional regulation following traumatic brain injury (TBI).
METHODS: A 51-year-old male patient suffered an in-car accident. The patient lost consciousness for approximately 30 days, and his Glasgow Coma Scale score was 3. He underwent stereotactic drainage for traumatic intraventricular and intracerebral hemorrhages. At approximately 6.5-year after onset, he began to show disinhibition behaviors such as shouting with anger, which worsened over time. At approximately 8-year after onset, he showed severe depression signs and disinhibition, including violence.
METHODS: The patient who showed delayed-onset behavioral changes (disinhibition and depression).
METHODS: Diffusion tensor imaging data were acquired at 3 months and 8 years after TBI onset.
RESULTS: The patient showed degeneration of core neural tracts for emotional regulation that was associated with delayed behavioral changes following TBI. On both 3-month and 8-year diffusion tensor tractographies (DTTs), the right dorsolateral prefronto-thalamic tract, ventrolateral prefronto-thalamic tract, orbital prefronto-thalamic tract, uncinate fasciculus, and both cinguli were reconstructed whereas other neural tracts were not reconstructed. Compared with the 3-month DTT, all reconstructed neural tracts on the 8-year DTT were narrow, except for the left cingulum, which showed new transcallosal fibers between both anterior cingula. The fractional anisotropy and tract volume of all reconstructed neural tracts were lower on the 8-year DTT than the 3-month DTT, except for the tract volume of left cingulum.
CONCLUSIONS: The evaluation of dorsolateral, ventrolateral, and orbital prefronto-thalamic tract, uncinate fasciculus, and cingulum using follow-up DTTs is useful when a patient with TBI shows delayed-onset behavioral problems.

Hypertrophic olivary degeneration (HOD) is a rare phenomenon that occurs after various insults to the Guillain-mollaret triangle (GMT). HOD is unique because the degeneration of inferior olivary nucleus becomes hypertrophic rather than atrophic. In this study, a 31-year-old woman developed HOD after pontine cavernoma surgery had been performed. The clinical manifestation was involuntary intorsion of right lower extremity during walking, which has not been reported in the literature. The woman also presented with palatal tremor, the most classic symptom of HOD. HOD\'s imaging trait include olive hypertrophy with increased T2 signal intensity on MRI, which are corresponding to the pathological findings. HOD is a self-limiting disease and excessive treatments are unnecessary.

暂无摘要。

Differential diagnosis of isolated single neurocysticercosis can be difficult, and management is controversial. We report here an original surgical strategy, and review previous studies reporting misdiagnosis, using the PRISMA guidelines. A 24-year-old man was admitted to our hospital for recent memory impairment, hypoesthesia of the right hand, and recurrent focal seizures without loss of consciousness. Brain MRI revealed a single ring-enhancing parenchymal lesion in the left superior postcentral gyrus, with large perilesional edema. Since exhaustive systemic exploration was negative, surgical resection of the lesion was decided on in a multidisciplinary team meeting. To preserve eloquent brain areas, surgery was performed in awake condition. It allowed complete resolution of clinical manifestations. The diagnosis of neurocysticercosis was confirmed on pathology. This case illustrates the utility of awake surgery in degenerating neurocysticercosis in functional areas, and emphasizes the importance of including it in differential diagnosis of cystic ring-enhancing brain lesions.

Peripheral nerves are routinely examined microscopically during the nonclinical safety assessment of therapeutics. In addition to test article-related on- or off-target changes, microscopic changes in peripheral nerves may also be caused by study procedures, such as parenteral test article administration and blood or tissue sampling. We present 2 nonclinical case studies in which nonstandard peripheral nerves had study procedure-related histologic changes. The first case study describes mouse trigeminal nerve changes as a result of blood sampling via retro-orbital sinus puncture. These changes included minimal-to-mild nerve fiber (axonal) degeneration associated with macrophage infiltration. The second case study presents rat brachial plexus changes associated with animal handling and blood sampling. Brachial plexus changes included minimal-to-moderate inflammation, focal hemorrhage, and nerve fiber degeneration. In both cases, the histological changes were morphologically indistinguishable from those that might be due to test article. Therefore, careful consideration of the incidence and severity across groups and a review of study procedures to rule out handling-related nerve damage are essential before identifying a test article-related effect on peripheral nerves. Study design considerations to avoid such procedure-related changes will be discussed, as well as sampling strategies to help distinguish these from test article-related effects.

暂无摘要。

This is a case report of autopsy findings for a male Japanese patient who presented with progressive gait disturbance and dysarthria. Neurological examination at the age of 61 years revealed pseudobulbar palsy and upper motor neuron disorder. The patient appeared unaware of his illness. Electrophysiological examination showed lower neuron damage. The patient was diagnosed with amyotrophic lateral sclerosis characterized by lower motor neuron damage. He died of pneumonia 2 years and 3 months after diagnosis. Neuropathological examination demonstrated severe degeneration of the upper neurons and mild degeneration of the lower neurons. Immunohistological examination indicated transactivation-responsive DNA-binding protein-43-positive pathology in the frontal and temporal lobes, amygdala, corpus striatum, and spinal cord. We believe the neuropathological findings correlate well with the clinical features. Furthermore, we also discuss the lesions involved in the patient\'s dementia. (Received March 28, 2017; Accepted September 19, 2018; Published November 1, 2018).

BACKGROUND: Recent studies have used diffusion tensor tractography (DTT) to demonstrate that central poststroke pain (CPSP) was related to spinothalamic tract (STT) injury in patients with stroke. However, few studies have been reported about delayed-onset CPSP due to degeneration of the STT following a stroke.
METHODS: A 57-year-old female patient presented with right hemiparesis after stroke. Two weeks after onset, she did not report any pain. At approximately 6 months after onset, she reported pain in the right arm and leg, and the pain slowly intensified with the passage of time. At 14 months after onset, the characteristics and severity of her pain were assessed to be continuous pain without allodynia or hyperalgesia; tingling and cold-sensational pain in her right whole arm and leg (visual analog scale score: 5).
METHODS: The patient was diagnosed as the right hemiparesis due to spontaneous thalamic hemorrhage.
METHODS: Clinical assessment and diffusion tensor imaging (DTI) were performed 2 weeks and 14 months after onset.
RESULTS: She suffered continuous pain in her right whole arm and leg (visual analog scale score: 5). On DTT of the 2-week postonset DTI scans, the configuration of the STT was well-preserved in both hemispheres. However, in contrast to those 2-week postonset results, the 14-month postonset DTT results showed partial tearing and thinning in the left STT. Regardless, both the 2-week and 14-month postonset DTT showed that the left STT passed through the vicinity of the thalamic lesion.
CONCLUSIONS: Diagnostic importance of performing a DTT-based evaluation of the STT in patients exhibiting delayed-onset CPSP following intracerebral hemorrhage.

Studies using optical coherence tomography (OCT) to compare retinal nerve fiber layer thickness in subjects with schizophrenia and healthy controls have yielded inconsistent results. We aimed to compare changes in retinal nerve fiber layer thickness in schizophrenia and healthy controls via a meta-analysis. Relevant studies were selected via an electronic search of the Cochrane Controlled Trials Register, Pubmed, and Embase. All included studies measured average and 4-quadrant (temporal, superior, nasal, and inferior) retinal nerve fiber layer thickness via OCT. Statistical analysis was performed using RevMan 5.0 software. Seven case-control studies involving collective totals of 245 eyes in patients with schizophrenia and 220 eyes in healthy controls were ultimately included in this meta-analysis. Statistical analysis revealed that average retinal nerve fiber layer thickness in patients with schizophrenia was significantly reduced compared to that of healthy controls. Additionally, retinal nerve fiber layer thickness in the inferior quadrant, nasal quadrant, and temporal quadrant differed significantly between the two groups, while differences in the superior quadrant did not. In view of these results, we suggest that peripapillary retinal nerve fiber layer thickness as measured by OCT may be a useful tool for the diagnosis of schizophrenia.