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Objective:To investigate the clinicopathological features and imaging differential diagnosis of intravascular large B-cell lymphoma (IVLBCL) in the central nervous system (CNS).Methods:A case of CNS IVLBCL with multiple intracerebral microbleeds (CMBs) diagnosed in the Department of Neurology, Qilu Hospital of Shandong University in 2017 was reported. The clinical and imaging data, histological and immunohistochemical markers were retrospectively analyzed, and the relevant literature was reviewed.Results:The patient was a 31-year-old woman presented with headache and seizures. Cranial magnetic resonance imaging (MRI) showed multifocal lesions involving mainly the cortical and subcortical white matter (bilateral cerebral hemisphere and right cerebellar hemisphere), hyperintense signal on T 2-weighted and fluid-attenuated inversion recovery images, with hypointense signal on T 1-weigthed and diffusion-weighted images and contrast enhancement in some lesions. The susceptibility weighted imaging revealed multifocal cortical or subcortical hypointense lesions, involving mainly the subcortical white matter. Brain magnetic resonance angiography was normal. Brain magnetic resonance venography showed left side transverse sinus was hypoplastic. Cranial magnetic resonance spectroscopy showed decreased N-acetylaspartylglutamate peak, elevated choline peak and inverted lipid double peak. Her symptoms and the lesions once improved after starting steroid treatment. However, CNS recurrence occurred after 1 week of steroid withdrawal. She underwent the biopsy of the right frontotemporal lobe. The pathological examination showed multiple microscopic hemorrhages and edema scattered in the brain tissue. A large number of heterologous mononuclear cells were aggregated in small blood vessels in the parenchyma and meninges. Immunohistochemical analysis revealed that the tumor was negative for Epstein-Barr virus encoded small RNAs, CD 3, CD 10, cytokeratin and CD 138, and positive for CD 20, CD 79α, B-cell lymphoma (BCL)-2, BCL-6, myelocytomatosis oncogene (C-myc) and multiple myeloma oncogene-1 (MUM-1). The Ki67 proliferation index was about 70%. The diagnosis of IVLBCL was confirmed. Conclusions:IVLBCL in CNS is a rare and swiftly progressive disease with poor prognosis. Its clinical symptoms and imaging are nonspecific. Early diagnosis and treatment is critical. Biopsy is the gold standard for diagnosis. Random skin biopsy may be helpful for the early diagnosis. Furthermore, regarding the cause of multiple CMBs, the possibility of IVLBCL should be considered in the differential diagnosis, in addition to the common causes, such as primary angiitis of the CNS and cerebral amyloid angiopathy.
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Objective:To investigate the clinical manifestations and imaging features of spastic paraplegia caused by spinal cord Wallerian degeneration after pontine infarction, and to analyze its occurrence process and mechanism.Methods:The clinical manifestations and imaging features of two patients with spastic paraplegia caused by spinal cord Wallerian degeneration after pontine infarction were reported for the first time in China. Combined with anatomy and review of the literature, the mechanism, imaging manifestations and clinical features of Wallerian degeneration of lateral funiculus of spinal cord after pontine infarction were analyzed systematically.Results:Case 1 was a 65 years old female, and case 2 was a 58 years old male, who were treated in Qilu Hospital of Shandong University on December 7, 2018 and June 23, 2019 respectively. All the two patients presented with strength weakness of both limbs, hypertonia, symmetric hyperreflexia, and bilateral extensor plantar responses, which suggested spastic paraplegia secondary to upper motor neuron involvement. Spastic paraplegia appeared eight months after pontine infarction in case 1 and appeared six months after pontine infarction in case 2. Magnetic resonance imaging revealed continuous iso-T 1 and high-T 2 signals of bilateral pyramidal tracts below the pontine foci. Case 1 showed lesions of lateral cord of medulla oblongata, cervical spinal cord and thoracic spinal cord, and case 2 showed lesions of lateral cord of medulla oblongata and cervical spinal cord. At the same time, motor neuron disease and metabolic disease were excluded by electromyography and laboratory examination, inflammatory demyelinating disease was excluded by cerebrospinal fluid examination in one case. The syndromes, in combination with a continuous strip of abnormal signal revealed by magnetic resonance imaging which was consistent with the pyramidal tract and connected with the primary lesion suggested wallerian degeneration of spinal cord secondary to pontine infarction. The clinical symptoms of two cases were gradually aggravated in follow-up. Conclusions:Spinal cord Wallerian degeneration is a sequel after pontine infarction, which is related to the prognosis of the disease. A full understanding of its clinical manifestations and imaging features can avoid clinical misdiagnosis as other diseases.
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Objective:To explore the etiology, clinical features and treatment of superficial siderosis of central nervous system (SSCNS) in China.Methods:The clinical data of four patients with SSCNS diagnosed by magnetic resonance imaging (MRI) and susceptibility weighted imaging (SWI) from Qilu Hospital of Shandong University during 2015—2019 were retrospectively reviewed. The etiology and clinical features of the four cases were summarized and analyzed.Results:All the four patients were male, with an average age of 60.5 years. Clinical symptoms included headache, hearing loss, and cerebellar ataxia. Brain MRI and SWI showed that hemosiderin mainly deposited (short linear T 2 signal, low SWI signal) on the surface of cerebellum, brainstem, temporal lobe, frontal lobe, and spinal cord. Potential bleeding sites were found in all four patients, including brain traumatic history, spinal intradural extramedullary cavernous hemangioma, brain metastasis and intracranial aneurysm. Patients were followed up for six months to four years. Headache symptoms improved in only one patient who received surgical treatment, while symptoms of the other three patients progressed. Conclusions:SSCNS is mainly characterized by hearing loss, progressive cerebellar ataxia and myelopathy. The diagnosis of this disease mainly depends on imaging examination. The linear low signal on the surface of T 2WI is the main basis for the diagnosis of SSCNS. Surgical treatment of bleeding sites and iron chelator are the main treatments of the disease.
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Objective The objective of this study was to study LGI1 limbic encephalitis (LE). Methods We performed a retrospective analysis on the clinical features,laboratory findings,imaging profiles and treatment outcomes of 17 patients with LGI1. Results The study included 14 male and 3 female cases. The median age was 61 years old. The clinical manifestations includes 14 cases with cognitive dysfunction, 11 cases with faciobrachial dystonic seizures (FBDS), 10 cases with focal seizures, 8 cases with generalizedtonic-clonic seizure and 9 cases with mental and behavioral disorder. Among 17 examined patients, there were 16 patients with positive and 1 with negative serum LGI1 antibody (but whose CSF LGI1 antibody was positive). Among 16 examined patients, there were 14 patients with positive cerebrospinal fluid LGI1 antibody. All patients had good responses to the first-line treatment and 2 patients experienced recurrence. During more than one-year follow-up, the recurrence rate was 33％ and no patient died. Conclusion LGI1 LE is an autoimmune encephalitis, which is mainly unidirectional progression and can relapse. FBDS and focal seizures usually are first symptoms, followed by cognitive dysfunction. Patients are responded to immunotherapy well and have good prognosis.