Adult ; Female ; Humans ; Limbic Encephalitis ; diagnosis ; etiology ; Ovarian Neoplasms ; complications ; diagnosis ; Paraneoplastic Cerebellar Degeneration ; diagnosis ; etiology

Carcinoma ; Humans ; Limbic Encephalitis ; diagnosis ; surgery ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; diagnosis ; surgery

Limbic encephalitis (LE) is characterized by short-term memory loss, disorientation, agitation, seizures, and histopathological evidence of medial temporal lobe inflammation. Leucine-rich, glioma inactivated 1 (LGI-1) is an auto-antigen associated with LE. We report a 37-year-old male patient with LGI-1-related LE who presented with recurrent episodes of selective amnesia, seizure-like activity, confusion, and personality change. His symptoms were significantly improved with steroid therapy. Thorough differential diagnosis with consideration for autoimmune encephalitis should be in patients with presentation of symptoms, such as memory impairment, personality change and seizure-like activity, especially when other neurological diagnoses are excluded.
Adult ; Amnesia ; Diagnosis ; Diagnosis, Differential ; Dihydroergotamine ; Encephalitis ; Glioma ; Humans ; Inflammation ; Limbic Encephalitis ; Male ; Memory ; Memory, Short-Term ; Seizures ; Temporal Lobe

Anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) is a rare neurological disorder that has a subacute course of progressive encephalopathy and fasciobrachial dystonic seizures. We report a patient with anti-LGI1 LE that presented with atypical manifestations that complicated the diagnosis. A 62-year-old woman presented with a chronic course of memory disturbance and a subsequent relapse with an altered mental status after 10 months. The patient reported frequent chest pain of squeezing and dull nature, typically lasting 10-30 seconds. The chest pain was related to partial seizures, which were confirmed by video-EEG monitoring. Anti-LGI1 antibody was identified in serum and CSF. The patient's symptoms improved by immune modulation treatment. Patients with anti-LGI1 LE can experience atypical partial seizures, and a chronic relapsing course. Clinical suspicions and video-EEG monitoring are helpful for the early diagnosis and effective immune modulation.
Chest Pain ; Diagnosis ; Early Diagnosis ; Female ; Glioma ; Humans ; Limbic Encephalitis* ; Memory ; Middle Aged ; Nervous System Diseases ; Recurrence ; Seizures

Paraneoplastic limbic encephalitis (PLE) is a rare neurologic disorder that can be combined with several systemic tumor. PLE is commonly associated with small cell lung carcinoma, but others are uncommon. The cause is unknown, but it is thought to be a autoimmune disorder developing secondary to carcinomatous process. Progressive neurologic symptoms usually predate the diagnosis of cancer, most symptoms usually improve after resection of tumor. PLE can combined with several tumors, but it is very rare with immature ovarian teratoma. We report a case of 31-year-old woman with PLE combined with an immature ovarian teratoma whose neurologic symptoms improved after resection of primary tumor.
Adult ; Diagnosis ; Female ; Humans ; Limbic Encephalitis* ; Nervous System Diseases ; Neurologic Manifestations ; Prednisolone ; Small Cell Lung Carcinoma ; Teratoma*

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.
Brain Edema ; Central Nervous System* ; Dementia ; Diagnosis ; Ear ; Humans ; Inflammation ; Joints ; Limbic Encephalitis ; Meningitis, Aseptic ; Meningoencephalitis ; Nose ; Polychondritis, Relapsing* ; Trees

BACKGROUND: The aim of this study was to investigate the clinical features of paraneoplastic limbic encephalitis (PLE) with small cell lung cancer (SCLC) and improve clinical diagnosis and treatment. METHODS: The clinical data of 15 patients with SCLC combined with PLE from January 1980 to May 2017 were collected from Beijing Union Hospital. Their symptoms and laboratory data were analyzed and the prognosis of the patients was followed. RESULTS: PLE is a rare disease, the incidence rate in SCLC is about 0.842%. The data may be underestimated because of misdiagnose or missed diagnosis; High incidence crowd of the disease is the middle-aged male smoker, the tumor-node-metastasis (TNM) stages of them are later than others; Typical neurological symptoms include varying degrees of short-term memory loss, seizures and varying degrees of mental disorders; neurological symptoms usually occur before the onset of cancer or respiratory symptoms appear, an average of about 2 months be taken from onset to diagnosis; Serum antibody (anti-Hu, GABA-R-Ab), cerebrospinal fluid, head magnetic resonance imaging (MRI) and electroencephalogram (EEG) of the patients has abnormalities; Videography, especially computed tomography (CT) is a good means of screening the primary tumor, pathology diagnosis mainly rely on bronchoscopy; The treatment of primary tumors can be more effective in alleviating the nervous system symptoms than immunotherapy. CONCLUSIONS Paraneoplastic limbic encephalitis is a rare paraneoplastic syndrome in nervous system caused by malignant neoplasms often characterized by facial neurological symptoms. The disease are usually associated with lung cancer (especially SCLC). Its nervous system symptoms occur earlier than the tumor diagnosis. Early diagnosis and treatment for primary tumors will increase the benefit.
Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Limbic Encephalitis ; complications ; Lung Neoplasms ; complications ; diagnosis ; therapy ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Small Cell Lung Carcinoma ; complications ; diagnosis ; therapy

Limbic encephalitis is a rare neurological syndrome, which develops after stem cell transplantation, and is characterized by a series of neurological symptoms, including retrograde amnesia, behavioral disturbance, and progressive intellectual deterioration and high signal intensity in the hippocampus on magnetic resonance imaging (MRI). Herein is described the case of a patient with limbic encephalitis, which developed after allogeneic bone marrow transplantation, and was possibly due to HHV-6 infection. An 18-year-old man, with acute lymphoid leukemia, who underwent HLA-matched unrelated donor bone marrow transplantation, developed a fever and chill accompanied by neurological symptoms, including behavioral disturbance and retrograde amnesia, during the bone marrow recovery phase. A brain MRI revealed bright signal-intensity in both hippocampi. Examination of his cerebrospinal fluid suggested viral encephalitis. Based on these findings, a diagnosis of viral limbic encephalitis was highly suspected. Tests for casual causes of viral limbic encephalitis, including the CMV, HZV and HSV-1 and 2, in serum or CSF were all negative. The encephalitis responded well to ganciclovir therapy.
Adolescent ; Amnesia, Retrograde ; Bone Marrow ; Bone Marrow Transplantation ; Brain ; Cerebrospinal Fluid ; Diagnosis ; Encephalitis ; Encephalitis, Viral ; Fever ; Ganciclovir ; Herpesvirus 1, Human ; Herpesvirus 6, Human ; Hippocampus ; Humans ; Limbic Encephalitis* ; Magnetic Resonance Imaging ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Stem Cell Transplantation* ; Stem Cells* ; Unrelated Donors

The high signal intensities in bilateral mesiotemporal lobes on T2-weighted images are typical findings of herpes encephalitis or paraneoplastic limbic encephalitis. We report a case of neurosyphilis with mesiotemporal involvement on MRI. Positive antibodies in the cerebrospinal fluid confirmed the diagnosis. The results suggest that neurosyphilis should be considered when MRI results indicate mesiotemporal abnormalities.
Adult ; Anti-Bacterial Agents/administration & dosage ; Biopsy, Needle ; Chancre/diagnosis/pathology ; Diagnosis, Differential ; Encephalitis, Herpes Simplex/diagnosis ; Follow-Up Studies ; Humans ; Limbic Encephalitis/diagnosis ; Magnetic Resonance Imaging/*methods ; Male ; Neurosyphilis/*diagnosis/drug therapy/pathology ; Penicillins/administration & dosage ; Rare Diseases ; Temporal Lobe/*pathology ; Tongue/pathology

Paraneoplastic syndrome is a rare neurologic disorder caused not by the direct invasion or metastasis of cancer, but by the remote effects of cancer. The central- and peripheral-nervous system or neuromuscular junction area were involved in this syndrome. The pathogenesis was thought as the autoimmune disease, the result of an immunologic response to cancer and to cross-react with self-cells of the nervous system or of the neuromuscular junction, causing neuronal dam-age. Specific forms of this syndrome are often associated with specific paraneoplastic autoantibodies and cancer. The onset of neurological symptoms and detection of these antibodies often precede the diagnosis of the cancer; therefore, detection of these antibodies greatly assists the diagnosis of this syndrome and prompts investigations for the underly-ing cancer. Some paraneoplastic central neurological syndromes, such as cerebellar degeneration, limbic encephalitis, and necrotizing myelitis, were not improved by putative pathogenic autoantibodies, or by immunosuppresant or tumor removal, inspite of improvement in other peripheral neurologic syndrome, Lambert-Eaton myasthenic syndrome, neu-romyotonia, and Stiff-man syndrome. A more detailed understanding of the relationship between the cancer and the neural involvement from the molecular biological standpoint may lead to rational tumor therapy and elucidation of the mechanism of neural death. Here, major clinical forms with well-known antineuronal antibodies and specific cancers are reviewed.
Antibodies ; Autoantibodies ; Autoimmune Diseases ; Diagnosis ; Lambert-Eaton Myasthenic Syndrome ; Limbic Encephalitis ; Myelitis, Transverse ; Neoplasm Metastasis ; Nervous System ; Nervous System Diseases ; Neuromuscular Junction ; Neurons ; Paraneoplastic Syndromes* ; Small Cell Lung Carcinoma ; Stiff-Person Syndrome