BMC Neurology

Deep brain stimulation improves cerebellar tremor symptoms in paraneoplastic cerebellar degeneration: a case report

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological syndrome caused by a remote effect or immune response involving the cerebellum due to tumor. Here, we report a rare case of PCD secondary to ovarian cancer, presenting clinically with cerebellar tremor. The patient presented with involuntary movements affecting the head, neck, and limbs, along with ataxia and horizontal nystagmus. After conventional medical treatments proved ineffective, the patient underwent a multidisciplinary assessment and received approval from the Institutional Review Board at Affiliated Nanjing Brain Hospital, Nanjing Medical University, to consent to deep brain stimulation (DBS) targeting the ventral intermediate nucleus (VIM) of the thalamus. During the 18-month follow-up, the frequency and amplitude of the tremor significantly improved, with the TRS (1-9) total score decreasing to 23, a 64.06% improvement compared to preoperative levels. The ADL scale score increased from 10 preoperatively to 35, indicating a significant improvement in quality of life. Additionally, the patient's cognitive and ataxic symptoms did not worsen. These results suggest significant improvement in symptoms compared to baseline, with enhanced daily life activities and improved quality of life.

Posterior reversible encephalopathy syndrome case report in an untreated, normotensive, ovarian cancer patient in the presence of paraneoplastic antibodies

Abstract Background Posterior reversible encephalopathy syndrome (PRES) is a rare neurological condition with many associated risk factors. The presentation varies and consists of seizures, impaired visual acuity or visual field deficits, disorders of consciousness, headaches, confusion and focal neurological deficits. The diagnosis relies on clinical presentation and MRI findings. Treatment and prognosis are related to the underlying etiology. Case presentation We present a 58-year-old woman with ovarian cancer who developed symptoms and radiologic signs of PRES with no apparent trigger other than a sudden increase in blood pressure for the first time in her life and before any treatment has begun. Antibodies to collapsin response-mediator protein-5 (CRMP-5), a malignancy related paraneoplastic protein, were identified in her CSF. Conclusions We present a novel and intriguing association between PRES and antibodies against CRMP-5 which may highlight a new etiology for this condition.

An atypical case of overlapped anti-NMDA-R and anti-GFAP encephalitis secondary to a teratoma of the fallopian tube: a case report

Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) and glial fibrillary acidic protein (anti-GFAP) encephalitis are autoimmune conditions that can occur concurrently, often presenting with severe neurological symptoms. Coexistence of both antibodies has been associated with greater resistance to immunotherapy and a higher risk of underlying neoplasms, particularly teratomas, which are commonly linked to anti-NMDA-R encephalitis. Detecting such tumors is crucial, though standard imaging may fail to identify them, especially in atypical locations. We report the case of a 32-year-old woman presenting with severe overlapping of anti-NMDA-R and anti-GFAP encephalitis. She required long-term admission to the intensive care unit and showed no improvement after receiving both first- and second-line immunotherapies. Despite unremarkable imaging studies, the lack of therapeutic response prompted further investigation for a potential hidden neoplasm. An exploratory laparoscopy was performed and revealed a fallopian tube teratoma. Following surgical removal of the tumor, the patient experienced marked clinical improvement and returned to normal daily activities within six months. This case underscores the importance of investigating occult neoplasms in patients with overlapping anti-NMDA-R and anti-GFAP encephalitis who do not respond to immunotherapy. The coexistence of these antibodies increases the likelihood of an underlying teratoma, which may not be detectable through conventional imaging, especially when located in atypical sites such as the fallopian tube. In such cases, additional imaging studies and early consideration of invasive diagnostic procedures, including laparoscopy, is warranted. Prompt tumor removal can significantly improve clinical outcomes, even when initial imaging is normal.

Systemic thromboembolism including multiple cerebral infarctions with middle cerebral artery occlusion caused by the progression of adenomyosis with benign gynecological tumor: a case report

Abstract Background Adenomyosis, a benign gynecological disease, causes cerebral infarction. Similar to Trousseau’s syndrome, it elevates cancer antigen 125 (CA125) and D-dimer levels; causes hypercoagulability; and results in cerebral infarction. However, no case of adenomyosis causing major cerebral artery occlusion and requiring endovascular thrombectomy has yet been reported. We report on a woman with middle cerebral artery occlusion caused by adenomyosis progression with a benign gynecological tumor and recurrent cerebral infarction. She was successfully treated by endovascular thrombectomy and hysterectomy. Case presentation A 48-year-old woman with heavy uterine bleeding was transported by ambulance to our hospital. Upon arrival, she presented with impaired consciousness. Laboratory test results revealed decreased hemoglobin (8.2 g/dL) and elevated D-dimer (79.3 µg/mL) levels. Radiological imaging revealed adenomyosis, a left ovarian tumor, multiple uterine myomas, and old and new bilateral renal infarctions. She experienced repeated episodes of excessive menstruation caused by adenomyosis and was scheduled for hysterectomy in 2 months at another hospital. After hospital admission, uterine bleeding stopped. However, 5 days after initial bleeding, she had another episode of heavy uterine bleeding and developed left hemiparesis and dysarthria 20 min later. Brain magnetic resonance imaging revealed bilateral multiple cerebral infarctions indicating right middle cerebral artery occlusion. Thus, endovascular thrombectomy was performed, and anticoagulant therapy was administered. Laboratory test results after thrombectomy revealed elevated CA125 (3536 U/mL) and CA19-9 (892 U/mL) levels. She was at a risk of recurrent heavy uterine bleeding leading to repeated cerebral infarction because of anticoagulant treatment. Therefore, we performed hysterectomy and ovariectomy 11 days after initial bleeding. Histopathological assessment revealed no malignancy. Although she developed asymptomatic pulmonary thromboembolism 14 days after initial bleeding, D-dimer and tumor marker levels returned to normal soon after gynecological surgery. At 15 months post-surgery, she had not experienced further ischemic events. Conclusions Adenomyosis with benign gynecological tumors may be associated with elevated D-dimer and tumor marker levels; excessive menstruation; and anemia. It may cause systemic thromboembolism, including cerebral infarction. To our knowledge, no other study has reported that adenomyosis causes major cerebral artery occlusion requiring endovascular thrombectomy. Hysterectomy may be an effective radical treatment of this condition.

Anti-NMDAR encephalitis with delayed ovarian teratoma in a young woman: a case report with 5 years of follow-up

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder with a variety of clinical manifestations. It has been established that anti-NMDAR encephalitis may be related to ovarian teratoma in female patients. However, a considerable number of patients have no obvious evidence of ovarian teratoma during the onset of the disease. A 25-year-old previously-healthy female experienced a series of acute symptoms within two days, including confusion, disorientation, short-term memory loss, auditory hallucinations, abnormal behavior, refractory status epilepticus, etc. Her brain MRI and abdominal imaging showed no definite abnormality while her electroencephalogram exhibited the presence of low to moderate amplitude sharp, spike, and multi-spike waves. Serum and cerebrospinal fluid tests yielded positive results for anti-NMDAR antibodies. However, an ultrasound scan failed to identify an ovarian teratoma. Consequently, the diagnosis of anti-NMDAR encephalitis without teratoma was made after 4 days onset. After the plasma exchange and immunoglobulin therapy, her neurological symptoms improved and obtained a clinical cure. In the next eight months of follow-up, the patient accidentally touched a lump in the lower abdomen without any symptoms, and abdominal ultrasound and CT scan revealed a left ovarian tumor. Then she underwent left ovarian teratoma resection surgery and histopathology showed a mature cystic teratoma with neural components. The patient continued to receive five years of follow-up, and her condition remained stable without any recurrence, except that there had been a low titer of anti-NMDAR antibody in her serum. Our case demonstrated the importance of long-term follow-up for female patients with anti-NMDAR encephalitis, since anti-NMDAR encephalitis-associated ovarian teratomas may develop in a delayed manner, even without any symptoms.

Case report: radiation-induced lumbosacral plexopathy – a very late complication of radiotherapy for cervical cancer

Abstract Background Lumbosacral plexopathy caused by radiotherapy is a rare but severe consequence of cancer treatment. This condition often leads to varying degrees of sensory and motor impairment. Neurological complications, which are typically permanent, manifest a long period after irradiation. Case presentation We describe a case of progressive lower extremity weakness and sensory impairment in a woman who had been effectively treated with radiotherapy for cervical cancer with development 36 years after irradiation. The electrophysiological assessment revealed a subacute bilateral axonal lesion of the lumbosacral plexus. None of the clinical manifestations, serology, cerebrospinal fluid or imaging data discovered an explanation other than radiation-induced lumbosacral plexopathy (RILP). Conclusions This case demonstrates that RILP may emerge more than 30 years after the radiotherapy.