Early Aggressive Immunotherapy Improves Functional Outcome in Chronic Immune Sensory PolyradiculopathyRead the full article
Case Reports in Neurological Medicine publishes case reports and case series focusing on diseases of the nervous system, as well as abnormal neurological function.
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Immediate Response to Chemotherapy in an Adult Neuroblastoma Patient Presenting with Cord Compression
We report a case of a 31-year-old female patient with high-risk neuroblastoma (NBL) who presented with a history of static back pain and bilateral lower limb weakness for almost a month. Her primary tumor was located in the right paraspinal region, causing spinal cord compression (SCC). Chemotherapy was administered with an immediate clinical improvement noted after 24 hours of starting treatment. We herein report the efficacy of chemotherapy in an adult neuroblastoma (aNBL) patient presenting with spinal cord compression.
A Patient with Combined CADASIL and MTHFR Homozygosity
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is an inherited disorder caused by a mutation in the NOTCH 3 gene, characterized by early onset of subcortical lacunar infarcts in the absence of vascular risk factors and cerebral microbleeds. Homozygosity for the factor Methylenetetrahydrofolate Reductase (MTHFR) is also associated with lacunar stroke risk and cerebral small-vessel disease regardless of the homocysteine level. The coexistence of MTHFR C677T homozygosity and NOTCH 3 mutation has never been reported in the literature previously, and that brings up the challenge of antithrombotic treatment in the presence of cerebral microbleeds.
Efficacy of Tocilizumab in Limbic Encephalitis with Anti-CASPR2 Antibodies
We report the case of a 64-year-old man who presented with subacute memory, balance impairment, behavioral and mood changes, and epileptic seizures. Magnetic resonance imaging (MRI) showed bilateral hippocampal abnormalities. Brain [18F]-FDG fluorodeoxyglucose positron emission tomography (PET) revealed hypometabolism in both the temporal lobe as well as in the left insular and parietal regions. The clinical and neuroradiological picture and the detection of anti-CASPR2 antibodies in serum oriented the diagnosis towards autoimmune limbic encephalitis. Intravenous high-dose steroid and immunoglobulin treatments were ineffective. We did not use rituximab for the presence of antibodies to HbcAg positivity. Tocilizumab given intravenously 8 mg/kg once a month for six months and then subcutaneously 162 mg every week for six months resulted in clinical and neuroradiological improvement. These data support the efficacy of tocilizumab in autoimmune limbic encephalitis associated with anti-CASPR2 antibodies, which has been sporadically reported in the literature.
Treatment of a Woman with Inoperable Meningioma Using Mifepristone for 26 Years
Meningioma treatment includes observation of its growth or surgery with or without associated radiotherapy. However, drug treatment can be used for tumors deemed inoperable because of their size and location. Due to the presence of progesterone receptors, the use of antiprogestin mifepristone is recommended. This study describes a case of inoperable meningioma treated with mifepristone for 26 years without interruption. The patient is a 45-year-old woman diagnosed with plaque meningioma, extending from the bottom of her right orbit, through the length of the small wing of the sphenoid, part of the large wing of the sphenoid, especially near the superior orbital fissure, and at the ceiling of the orbit. As this meningioma was considered inoperable, treatment with 200 mg oral mifepristone was administered uninterruptedly for 26 years. This treatment initially halted the growth of the meningioma and subsequently resulted in a small reduction of its volume; however, the meningioma has persisted until the last evaluation. After five years of mifepristone use, hydroxyurea was added for nine months but was discontinued due to anemia and leucopenia. In conclusion, mifepristone was useful for the survival of the patient for those 26 years. The drug interfered with the natural history of the meningioma, which generally evolves to death in such long follow-up durations without associated surgery or radiation therapy.
Endoscopic Endonasal Resection of Meckel’s Cave Epidermoid Cysts: Case Discussion and Literature Review
Meckel’s cave (MC) epidermoid cysts are relatively uncommon lesions. In cases where surgical excision is indicated, resection is often carried out via a frontosphenotemporal craniotomy from an anterolateral approach or a temporal craniotomy with or without a petrosectomy for a lateral corridor; both of these routes are associated with brain retraction and potential neurovascular injury. The anterior location of MC in the middle cranial fossa makes safe access via posterior fossa-based approaches—such as the retrosigmoid approach—challenging as well. Here, we present the cases of two patients diagnosed with epidermoid cysts in MC who underwent surgical resection via an endoscopic endonasal transpterygoid approach. Near-total resection was achieved in both cases, with only mild transient neurologic disturbances postoperatively. Radiographically, no evidence of residual disease was noted in either patient. We further review the nuances of an extended endoscopic endonasal approach to these lesions.
Subcutaneous EEG Monitoring Reveals AED Response and Breakthrough Seizures
Unrecognized seizures are a common problem in temporal lobe epilepsy potentially leading to undertreatment. Objective seizure counting using EEG home monitoring for prolonged periods with a minimally invasive device has not been feasible until now. We present a case in which a novel, subcutaneous EEG device was utilized to provide an objective seizure count. This information revealed unrecognized breakthrough seizures and informed treatment response, prompting treatment adjustment. The case illustrates how objective seizure counting in epilepsy using new devices can completely change diagnosis and management.