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Recognition of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) in Two Oligosymptomatic Sisters with Low CADASIL Scale Scores and a Venous Dysplasia: Report of a Novel Greek Family
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) due to mutations of the NOTCH3 gene is the most common cause of inherited cerebral small-vessel disease and one of the genetic causes of migraine with aura. The so-called CADASIL scale has been proposed as a clinical screening tool, and a score of 15 or higher seems useful in identifying patients with high probability of carrying NOTCH3 mutations. We studied a novel Greek family with clinical features compatible with CADASIL.
Source: Journal of Stroke and Cerebrovascular Diseases - April 26, 2018 Category: Neurology Authors: George P. Paraskevas, Vasilios C. Constantinides, Christos Yapijakis, Evangelia Kararizou, Elisabeth N. Kapaki, Anastasia Bougea Tags: Case Studies Source Type: research

Acute Intracranial and Spinal Subdural Hematoma Associated with Vardenafil
A 28-year-old healthy man was admitted to our hospital because of right-sided headache, vomiting, and lower back pain after the administration of vardenafil. Computed tomography and magnetic resonance imaging of the brain showed a small, right-sided, subdural hematoma. A lumbar magnetic resonance imaging showed a longitudinally extended subdural hematoma. He had no history of trauma. We speculated that vardenafil might have had an association with the bleeding. Several reports have suggested a relationship between phosphodiesterase-5 inhibitors and intracerebral or subarachnoid hemorrhage.
Source: Journal of Stroke and Cerebrovascular Diseases - May 2, 2018 Category: Neurology Authors: Takaaki Nakamura, Genya Watanabe, Ryuhei Harada, Emiko Kawasaki, Kenichi Tsukita, Yasushi Suzuki Tags: Case Studies Source Type: research

Temporal Profile of CT and T2*-Weighted Gradient-Echo MRI in a Patient with Unilateral Thalamostriate Vein Thrombosis
Deep cerebral venous system thrombosis (DCVST) is an uncommon variety of thrombosis that accounts for 11% of cases of cerebral venous thrombosis. Thalamostriate vein (TSV) thrombosis is further rare among patients with DCVST. Although patients with cerebral venous thrombosis commonly have characteristic neurological deficits including headache, deterioration of consciousness, and seizures, patients with DCVST do not necessarily show such symptoms. Therefore, diagnose of DCVST is sometimes difficult.
Source: Journal of Stroke and Cerebrovascular Diseases - November 24, 2018 Category: Neurology Authors: Hikaru Kamo, Yuji Ueno, Mizuho Sugiyama, Nobukazu Miyamoto, Kazuo Yamashiro, Ryota Tanaka, Nobutaka Hattori Tags: Letter to the Editor Source Type: research

Acute-Onset Ataxia and Transient Cerebellar Diffusion Restriction Associated with a PRRT2 Mutation
PRRT2 gene mutations cause paroxysmal kinesigenic dyskinesia (PKD), infantile convulsions, hemiplegic migraine, and episodic ataxia. A 21-year-old woman reported an episode of dizziness and ataxic gait occurring after swimming. Brain MRI showed a hyperintense cerebellar lesion on diffusion-weighted imaging (DWI) with decreased apparent diffusion coefficient. The clinical course was favorable. Both clinical and MRI abnormalities regressed. Her brother had presented PKD since adulthood. A C.649dupC PRRT2 truncating mutation was identified in both patients.
Source: Journal of Stroke and Cerebrovascular Diseases - November 28, 2018 Category: Neurology Authors: Nicolas Legris, Olivier Chassin, Ghaidaa Nasser, Florence Riant, Elisabeth Tournier-Lasserve, Christian Denier Tags: Case Report Source Type: research

Subarachnoid Hemorrhage with Progressive Cerebral Steno-Occlusive Disease: Report of 2 Cases
Nontraumatic nonaneurysmal subarachnoid hemorrhage (SAH) is a rare condition. Among them, SAH with cerebral steno-occlusive disease is quite rare. Moreover there has been no report of SAH patient who had been diagnosed with steno-occlusive disease since before. We here report 2 cases of nontraumatic nonaneurysmal convexity SAH who originally had progressive cerebral steno-occlusive disease. Case 1, a woman in her 40s who had diagnosed left internal carotid artery (ICA) stenosis 6 years before complained of headache.
Source: Journal of Stroke and Cerebrovascular Diseases - January 21, 2019 Category: Neurology Authors: Soichiro Takamiya, Tetsuyuki Yoshimoto, Katsuhiko Maruichi Tags: Case Report Source Type: research

Spontaneous Dissection of Both Vertebral Arteries Diagnosed with Three-Dimensional T1-Weighted Image
A 48-year-old woman spontaneously developed occipital pain, without any other neurological deficit. A brain magnetic resonance angiography showed narrowing, irregular dilatation, and aneurysmal formation in both the vertebral arteries (VA). According to these findings and the clinical course, we diagnosed the patient with spontaneous VA dissection. There was no finding suggesting cerebral infarction or subarachnoid hemorrhage. Three-dimensional T1-weighted magnetic resonance imaging performed 27days after first onset of headache revealed a crescent-shaped high-intensity lesion in both the VA walls.
Source: Journal of Stroke and Cerebrovascular Diseases - March 17, 2019 Category: Neurology Authors: Takanori Iwamoto, Naoki Oyama, Takaya Kitano, Toshiaki Goda, Junichi Uemura, Yoshiki Yagita Tags: Case Reports Source Type: research

T2*-Weighted MRI Detected Dilated Cerebral Veins in a Patient With Acute-Phase Cerebral Venous Sinus Thrombosis —A Case Report
We describe a 45-year-old man who presented with nausea, vomiting, and strong occipital headache on the right side. Although no abnormalities on neurological examination or computed tomography imaging were found on admission, peripheral blood cell counts showed polycythemia (hemoglobin 20.6 g/dL) and electrocardiography demonstrated atrial fibrillation. Therefore, anticoagulant treatment with heparin was started immediately. On the following day, the occipital headache continued. Brain T2*-weighted (T2*WI) magnetic resonance imaging (MRI) and, to a lesser extent, susceptibility-weighted imaging showed dilation of numerous ...
Source: Journal of Stroke and Cerebrovascular Diseases - June 2, 2019 Category: Neurology Authors: Takuya Kiyohara, Chie Abe, Mio Yokoi, Yusuke Sakaki, Yasuhiro Kumai Tags: Case Report Source Type: research

Carotid-Cavernous Sinus Fistula caused by Persistent Primitive Trigeminal Artery Aneurysm rupture: A case report
Carotid-Cavernous sinus fistula (CCF) caused by a ruptured aneurysm of persistent primitive trigeminal artery (PPTA) is exceptionally rare. Herein, we reported the case of a 64-year-old female who presented with headache, pulsatile tinnitus, and diplopia. Angiography showed a PPTA and CCF. Three-dimensional digital subtraction angiograms revealed a direct shunt between the PPTA aneurysm and the left cavernous sinus. The trigeminal carotid fistula was successfully treated with detachable coils and Onyx, and the PPTA was preserved.
Source: Journal of Stroke and Cerebrovascular Diseases - August 18, 2019 Category: Neurology Authors: Yu Fan, Yuechun LI, Tianyou Zhang, Changchun Jiang, Peng Zhang Tags: Case Report Source Type: research

A Case of Cerebral Venous Thrombosis and Deep Venous Thrombosis Due to Hyperthyroidism with Increased Factor VIII Activity
A 48-year-old woman was admitted to our hospital because of headache and fever. She was diagnosed with aseptic meningitis. Five days later, she had a seizure and developed left hemiparesis. Magnetic resonance imaging showed hyperintensity in the right parietal area on fluid attenuated inversion recovery imaging. She was diagnosed as having cerebral venous thrombosis (CVT) because the suprasagittal sinus was invisible on the venographic studies. Moreover, deep venous thrombosis (DVT) was detected in her left lower extremity.
Source: Journal of Stroke and Cerebrovascular Diseases - September 10, 2019 Category: Neurology Authors: Mutsumi Yokoyama, Ryotaro Yamashita, Masayuki Furuya, Maiko Yamazaki, Kazuo Koyama, Fumiaki Tanaka Tags: Case Report Source Type: research

Hemicrania Continua Subsequent to Vertebral Artery Dissection: A Case Report
We herein report the case of a 45-year-old woman who developed a continuous hemicranial headache subsequent to vertebral artery dissection (VAD). After remission of VAD, the patient repeatedly experienced right forehead and temporal region throbbing headache, accompanied by nausea, ocular hyperemia and lacrimation of the right eye, nasal congestion, and rhinorrhea. Magnetic resonance angiography did not reveal the recurrence of dissection. Daily use of indomethacin (190.8 mg/day) showed an excellent effect on the headache, suggesting that the patient had developed hemicrania continua subsequent to VAD.
Source: Journal of Stroke and Cerebrovascular Diseases - October 10, 2019 Category: Neurology Authors: Hiromitsu Tabata, Hiroshi Kitaguchi Source Type: research

Reversible Cerebral Angiopathy after Viral Infection in a Pediatric Patient with Genetic Variant of RNF213
We report a case of reversible cerebral angiopathy after viral infection in a pediatric patient with genetic variant of RNF213 mutation. The patient had developed a severe headache after hand, foot, and mouth disease.
Source: Journal of Stroke and Cerebrovascular Diseases - December 6, 2019 Category: Neurology Authors: Ikuma Echizenya, Kikutaro Tokairin, Masahito Kawabori, Ken Kazumata, Kiyohiro Houkin Source Type: research

Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report
A 51-year-old Japanese woman was admitted to our hospital because of speech difficulty following severe headache. Neurological examination showed dysarthria and tongue weakness on the right side, indicating right hypoglossal nerve palsy. Needle electromyography of the right side of the tongue showed fibrillation potentials. Magnetic resonance angiography and computed tomography angiography revealed a right, persistent, primitive hypoglossal artery (PPHA) that met Lie's diagnostic criteria. Digital subtraction angiography showed an extended PPHA with irregular caliber in the portion running through the right hypoglossal canal.
Source: Journal of Stroke and Cerebrovascular Diseases - December 11, 2019 Category: Neurology Authors: Hiroki Hikichi, Tatsuya Ueno, Masatoshi Iwamura, Haruo Nishijima, Akira Arai, Chieko Suzuki, Hiroshi Midorikawa, Jin-ichi Nunomura, Masahiko Tomiyama Tags: Case Report Source Type: research

Cardiac-Neurovascular Adverse Effects Responsible to Contrast Transcranial Doppler: A Case Report
Introduction and Case Presentation: A 44-year-old female patient suffered migraines and underwent contrast-enhanced transcranial Doppler (c-TCD). During the rapid injection of contrast agent, she suffered chest tightness, palpitation, decreased consciousness, perimouth numbness, and headache, respectively. Meanwhile, “curtain” pattern of air embolic signals lasted up to 115 seconds in her decreased right middle cerebral artery accompanied with arrhythmia. The microair embolic signals lasted as long as 340 seconds.
Source: Journal of Stroke and Cerebrovascular Diseases - January 13, 2020 Category: Neurology Authors: Xiuyun Mo, Zhengzheng Xuan, Weijun Zhang, Wei Wang, Jingxin Zhong Tags: Case Report Source Type: research

A Novel Heterozygous Variant in Exon 19 of NOTCH3 in a Saudi Family with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL; OMIM #125310) is the most common cause of monogenic familial cerebral small vessel disease. It typically manifests at middle adulthood with highly variable clinical features including migraine with aura, recurrent transient ischemic attacks or ischemic strokes, mood disorders, and progressive cognitive decline. It is caused by mutations in the NOTCH3 gene, which maps to the short arm of chromosome 19 and encode for epidermal growth factor-like repeats.
Source: Journal of Stroke and Cerebrovascular Diseases - May 13, 2020 Category: Neurology Authors: Hussein Algahtani, Bader Shirah, Suzan Y. Alharbi, Mohammad H. Al-Qahtani, Angham Abdulrahman Abdulkareem, Muhammad Imran Naseer Tags: Case Report Source Type: research

Response to letter to the editor
We would like to thank Dr. Sombat Muengtaweepongsa et al. for the interest in our article. We agree with the opinion that the contrasted Transcranial Doppler(TCD) is a safe procedure and the incidence of adverse effects is low. But we notice that in the letter, it was mentioned that up to 20% patients suffered from adverse transient neurological symptoms, such as headache, dizziness and visual aura after contrasted TCD and the number was beyond our expectation. So we are curious about the reference of this statement.
Source: Journal of Stroke and Cerebrovascular Diseases - June 29, 2020 Category: Neurology Authors: Zheng-zheng Xuan, Xiu-yun Mo, Jing-xin Zhong Tags: Letter to the Editor Source Type: research

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