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.

Sidonie Ibrikji

Tarek El Halabi

Bassem Yamout

A Patient with Combined CADASIL and MTHFR Homozygosity

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&#x2009;mg/kg once a month for six months and then subcutaneously 162&#x2009;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.

Maurizio Benucci

Luciana Tramacere

Maria Infantino

Mariangela Manfredi

Valentina Grossi

Arianna Damiani

Francesca Li Gobbi

Maristella Piccininni

Gaetano Zaccara

Massimo Cincotta

Efficacy of Tocilizumab in Limbic Encephalitis with Anti-CASPR2 Antibodies

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&#x2009;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.

Maria das Dores Medina-Lopes

Luiz Augusto Casulari

Treatment of a Woman with Inoperable Meningioma Using Mifepristone for 26 Years

Meckel&#x2019;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&#x2014;such as the retrosigmoid approach&#x2014;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.

Jehad Zakaria

Pravesh Saini

Mariya Yanovskaya

John T. Tsiang

Krishnan Ravindran

Stephen Johans

Chirag R. Patel

Anand V. Germanwala

Endoscopic Endonasal Resection of Meckel&#x2019;s Cave Epidermoid Cysts: Case Discussion and Literature Review

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.

Sigge Weisdorf

Ivan C. Zibrandtsen

Troels W. Kjaer

Subcutaneous EEG Monitoring Reveals AED Response and Breakthrough Seizures

Primary intradural malignant peripheral nerve sheath tumor (MPNST) is an extremely rare diagnosis and is associated with an extremely poor prognosis. A 77-year-old man diagnosed with an intradural MPNST, more than 40 years after radiation for a testicular seminoma, is reported. Intradural MPNSTs of the spine outside the setting of neurofibromatosis is extremely rare and can masquerade as common benign nerve sheath tumors, on imaging. An older age at presentation with short duration of symptoms and prior regional radiation treatment encompassing the spine in the treatment field regardless of remoteness should alert the oncologists and neurosurgeons to the possible existence of this rare and aggressive tumor, as the management, and overall prognosis of this tumor is distinctly different compared to the usual intradural spinal tumors.

Osmond C. Wu

Berje H. Shammassian

Arunit J. S. Chugh

Aparna Harbhajanka

Manish K. Kasliwal

Ominous Occurrence of Spinal Intradural Primary Malignant Peripheral Nerve Sheath Tumor Four Decades following Radiation Therapy for Testicular Seminoma

Introduction. Intravascular lymphoma (IVL) is an uncommon and often fatal disease characterized by intraluminal proliferation of lymphomatous cells within blood vessels. Because of a heterogeneous clinical presentation and lack of sensitive diagnostic protocols, diagnosis of IVL is most often made at autopsy. However, with early diagnosis and appropriate chemotherapy, the prognosis is greatly improved and complete remission is possible. In order to broaden the possible presentations of IVL, we present a patient with an atypical manifestation of biopsy-proven intravascular large B-cell lymphoma who suffered dissections of both intracranial and extracranial arteries in addition to progressive intracranial hemorrhages. Case Report. A 47-year-old woman presented with unilateral paresthesias. She developed progressive multifocal infarcts and hemorrhage with dissections of both intracranial and extracranial arteries, resulting in coma. Brain biopsy revealed IVL. She received aggressive chemotherapy and remains in complete remission with good neurologic recovery. Conclusion. IVL is known to exert its pathology on small arteries and capillaries, but is not known to cause dissections of large vessels. The diagnosis should be considered in cases with unexplained arterial dissections and progressive strokes. Early diagnosis with appropriate laboratory screening and tissue confirmation by biopsy can lead to greatly improved outcomes.

Ricky Chen

Gurjeet Singh

J. Scott McNally

Cheryl A. Palmer

Adam de Havenon

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections

Conversion disorder is characterized by one or more symptoms of altered voluntary motor or sensory functions that cannot be explained by a neurological disease (Keynejad, 2019; Samuels et al., 2019). We present a patient with conversion disorder and discuss her process in overcoming this disorder. Additionally, we review the literature about this specific disorder. A 15-year-old white female was diagnosed with conversion disorder and has shown significant recovery with physical therapy and group therapy since. It is essential to recognize this disorder early to lessen the financial burden on families and to speed up the recovery process for these patients.

Lauren Miller

Robert L. Archer

Nidhi Kapoor

Conversion Disorder: Early Diagnosis and Personalized Therapy Plan Is the Key

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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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Case Reports in Neurological Medicine - Table of contents 2020

Case Reports in Neurological Medicine | Hindawi

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