We discuss a patient who presented to our hospital with signs, symptoms, and clinical course consistent with transient global amnesia (TGA). Her hospital work-up was overall unremarkable except for the presence of a diffusion weighted imaging (DWI) abnormality on MRI suggestive of acute infarction in the left cingulate gyrus. Symptoms quickly resolved, and she was discharged home in stable condition. While the etiology of TGA remains controversial, in discussing this case and the current literature we hope to provide further data supporting the possibility of an underlying ischemic process, as well as help better illustrate the neurologic structures involved.

Lawrence Chau

Antonio Liu

Transient Global Amnesia as the Sole Presentation of an Acute Stroke in the Left Cingulate Gyrus

Takayasu arteritis is a rare chronic progressive granulomatous necrotizing large vessel panvasculitis mainly involving aorta and its main branches. It commonly affects the female in second to third decades. Common clinical features of Takayasu arteritis are hypertension, claudication, dizziness, headache, or fever. Takayasu arteritis is diagnosed with clinical history of claudication, absent pulse, discordant blood pressure, bruit over aorta, and typical angiographic findings. Stroke as a primary manifestation of Takayasu arteritis is rarely reported in the medical literatures. We are reporting a 16-year-old female who had malignant ischemic stroke as a first manifestation of Takayasu arteritis.

Bhupendra Shah

Roshan Chhetri

Malignant Ischemic Stroke in a Young Female: A Rare Primary Manifestation of Takayasu Arteritis

We describe our encounter with a 39-year-old man who exhibited acute painless visual loss and progressive gait disturbance. He had tendinous xanthoma and several neuroophthalmological findings indicative of optic neuropathy in the right eye, including afferent pupillary defect, cecocentral scotoma, and optic disc swelling. Neurological examination showed cerebellar ataxia and pyramidal weakness. Brain magnetic resonance imaging revealed bilateral swelling in the optic nerves with gadolinium-enhancement suggesting optic neuritis, an enlarged fourth ventricle, atrophy of the cerebellum, and hyperintensities in the bilateral dentate nuclei. The patient was diagnosed with cerebrotendinous xanthomatosis (CTX) based on an elevated serum cholestanol level and a homozygous missense mutation in CYP27A1. CTX is a genetic lipid storage disease caused by dysfunction of the mitochondrial enzyme sterol 27-hydroxylase. With respect to ophthalmological manifestations, juvenile cataracts and optic neuropathy are common findings in patients with CTX, but there have been no reports of optic neuropathy with features suggestive of optic neuritis. Thus, this case illustrates that clinicians should consider a diagnosis of CTX in patients with cardinal features of CTX even if the patients show signs indicative of optic neuritis.

Miyuki Miyamoto

Nobuyuki Ishii

Hitoshi Mochizuki

Kazutaka Shiomi

Tomoko Kaida

Hideki Chuman

Masamitsu Nakazato

Optic Neuropathy with Features Suggestive of Optic Neuritis in Cerebrotendinous Xanthomatosis

Recurrent painful ophthalmoplegic neuropathy, previously known as ophthalmoplegic migraine, is a rare condition that affects children and young adults. Its cause and classification are still controversial and, consequently, there are no published treatment guidelines or consensus. Glucocorticoids seem to be beneficial for some patients, but there is no established treatment when failure of this therapy occurs. The aim of this study was to report a case where pregabalin was successfully used after failure of glucocorticoid therapy in a patient with recurrent painful ophthalmoplegic neuropathy.

Laura N. Zamproni

Reinaldo T. Ribeiro

Marina Cardeal

Treatment of Recurrent Painful Ophthalmoplegic Neuropathy: A Case Where Pregabalin Was Successfully Employed

Marchiafava-Bignami Disease (MBD) is a toxic demyelinating disease often diagnosed in chronic alcoholics. The disease process typically involves the corpus callosum and clinically presents with various manifestations resulting in MBD type A and type B on the basis of clinical condition, extent of callosal involvement and extracallosal involvement at brain magnetic resonance imaging (MRI), and prognosis. The death rate is high. We report a patient affected by MBD type B, who presented an isolated reversible splenial lesion at brain MRI and achieved a favorable recovery.

Carmine Franco Muccio

Luca De Lipsis

Rossella Belmonte

Alfonso Cerase

Reversible MR Findings in Marchiafava-Bignami Disease

The treatment protocol of status epilepticus has many associated toxicities so there is interest in alternate nonmedicinal therapies for managing New Onset Refractory Status Epilepticus (NORSE) patients. Vagus nerve stimulation (VNS) is an FDA-approved therapy for refractory epilepsy that has been shown to decrease the frequency and severity of seizures. We present the case of a patient with new-onset refractory status epilepticus (NORSE) whose seizures were successfully treated with vagus nerve stimulation. A 25-year-old male with no history of epilepsy or other neurological disorders presented with altered mental status and generalized tonic-clonic seizures following a two-week history of an upper respiratory tract infection. Lumbar puncture showed neutrophilic pleocytosis, and he was treated for bacterial and viral meningoencephalitis. In spite of treatment, his seizures began increasing in frequency. On day three, the patient entered status epilepticus (SE) refractory to intensive pharmacotherapy with maximal doses of valproate, levetiracetam, and propofol. On day four, SE remained refractory, so pentobarbital was introduced with targeted burst suppression pattern on electroencephalography (EEG). Patient continued to be refractory to these measures, so a vagus nerve stimulator (VNS) was implanted (day eight). Following VNS implantation, EEG demonstrated significant reduction of seizure activity and subsequent magnet swiping continued aborting electrographic seizures. No SE or electrographic seizures were reported for seventy-two hours, but few occasional discharges were reported. Seizures eventually recurred on day fourteen and the patient succumbed to his multiple comorbidities on day seventeen. Due to the efficacy of VNS in refractory epilepsy, there was interest in using it in refractory status epilepticus. Multiple case reports have described a benefit from implantation of VNS in the treatment of SE. The successful use of VNS to acutely terminate status epilepticus for seventy-two hours in this critically ill patient adds to current evidence that there is utility in using VNS for refractory status epilepticus.

Mohankumar Kurukumbi

James Leiphart

Anam Asif

Jing Wang

Vagus Nerve Stimulation (VNS) in Super Refractory New Onset Refractory Status Epilepticus (NORSE)

Background. Pituitary metastasis of cervical adenocarcinoma is an exceedingly rare phenomenon. Case Description. The authors present a case of a 66-year-old female with cervical adenocarcinoma who was discovered to have a rapidly growing intrasellar mass in the work-up of adrenal insufficiency and hypothyroidism. The patient underwent subsequent endoscopic endonasal subtotal resection of the mass. Histopathological analysis of the resected lesion demonstrated features consistent with metastatic mucinous adenocarcinoma of the cervix. While initially neurologically asymptomatic following surgery, the patient developed an oculomotor nerve palsy several weeks following surgical debulking, at which time neuroimaging revealed marked regrowth and suprasellar extension of the metastatic lesion. Conclusions. While metastatic cervical adenocarcinoma to the sella is rare, it should be considered in the differential based on the history of the patient.

Krishnan Ravindran

Brandon M. Zsigray

Michael P. Wemhoff

John D. Spencer

Ewa Borys

Chirag R. Patel

Anand V. Germanwala

Sellar Metastasis of Cervical Adenocarcinoma

Although primary hemifacial spasm (HFS) is mostly related to a vascular compression of the facial nerve at its root exit zone (REZ), its occurrence in association with distal, cisternal portion has been repeatedly reported during the last two decades. We report two patients with typical HFS caused by distal neurovascular compression, in which the spasm was successfully treated with microvascular decompression (MVD). Vascular compression of distal, cisternal portion of the facial nerve was identified preoperatively in the magnetic resonance imaging (MRI). It was confirmed again with intraoperative findings of compression of cisternal portion of the facial nerve by the meatal loop of the anterior inferior cerebellar artery (AICA) and absence of any offending vessel in the REZ of the facial nerve. Immediate disappearance of lateral spread response (LSR) after decompression and resolution of spasm after the operation again validated that HFS in the current patients originated from the vascular compression of distal, cisternal portion of the facial nerves. According to our literature review of 64 patients with HFS caused by distal neurovascular compression, distal compression can be classified by pure distal neurovascular compression (31 cases, 48.4&#x25;) and double compression (both distal segment and the REZ of the facial nerves, 33 cases [51.6&#x25;]) according to the presence or absence of simultaneous offender in the REZ. Eighty-four percent of 64 identified distal offenders were the AICA, especially its meatal and postmeatal segments. Before awareness of distal neurovascular compression causing HFS and sophisticated MRI imaging (before 2000), the rate of reoperation was high (58&#x25;). Preoperative MRI and intraoperative monitoring of LSR seems to be an essential element in determination of real offending vessel in MVD caused by distal offender.

Byung-chul Son

Hak-cheol Ko

Jin-gyu Choi

Hemifacial Spasm Caused by Vascular Compression in the Cisternal Portion of the Facial Nerve: Report of Two Cases with Review of the Literature

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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 2019

Case Reports in Neurological Medicine | Hindawi

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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