A case of childhood unilateral relapsing primary angiitis of the central nervous system.

The patient was a 17-year-old girl with transient right-sided weakness and dysesthesia associated with headache and nausea. Head magnetic resonance imaging (MRI) revealed white matter lesions confined to the left hemisphere. Initially, multiple sclerosis was suspected, and methylprednisolone (mPSL) pulse therapy was administered, followed by fingolimod hydrochloride. However, on day 267, the patient again experienced transient hypesthesia. Cranial MRI showed expansion of the highly infiltrated areas of the left hemisphere on fluid-attenuated inversion recovery (FLAIR) and T2 weighted image, accompanied by edema. Multiple contrasting areas were also observed. Susceptibility-weighted imaging demonstrated several streaks and some corkscrew-like appearances with low signals from the white matter to the cortex, suggestive of occluded or dilated collateral vessels. Multiple dotted spots indicating cerebral microbleeds (MBs) were also observed. A brain biopsy revealed lymphocytic, non-granulomatous inflammation in and around the vessels. Vascular occlusion and perivascular MBs were prevalent. The patient was diagnosed with relapsing primary angiitis of the central nervous system (PACNS), and immunosuppressive treatment was initiated, mPSL 1000 mg/day pulse therapy. The patient's clinical symptoms and neuroradiological abnormalities gradually improved. She is now receiving oral prednisolone (6 mg/day) and mycophenolate mofetil (1750 mg/day). This case corresponds to unilateral relapsing, which has recently been reported as a specific clinicopathological subtype of PACNS.

Persistent brain damage in reversible cerebral vasoconstriction syndrome on 99mTc-ethyl cysteinate dimer single-photon emission computed tomography: A long-term observational study.

BACKGROUND: Blood-brain barrier (BBB) breakdown is considered a key step in the pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS); however, its temporal course remains unclear. Based on the characteristics and dynamics of 99mTc-ethyl cysteinate dimer (99mTc-ECD) as a tracer, 99mTc-ECD single-photon emission computed tomography (SPECT) can detect not only hypoperfusion but also BBB breakdown and/or brain tissue damage. Therefore, this study aimed to investigate this course using 99mTc-ECD SPECT. METHODS: Between 2011 and 2019, we enrolled seven patients (one male and six female patients) with RCVS without ischemic or hemorrhagic stroke or posterior reversible encephalopathy syndrome. 99mTc-ECD SPECT was performed repeatedly in each patient. SPECT data were statistically analyzed using an easy Z-score imaging system. RESULTS: Thunderclap headache was the initial symptom in all the patients and was most commonly triggered by bathing (three patients). All the patients exhibited vasoconstriction and reduced cerebral uptake of 99mTc-ECD during the acute stage. Follow-up assessment from 3 to 16 months showed that reduced cerebral uptake persisted in all the patients, even after the vasoconstriction had resolved. CONCLUSION: Reduced cerebral uptake of 99mTc-ECD persisted in the late stage of RCVS, even after vasoconstriction and headache subsided. BBB breakdown and/or brain tissue damage may underlie this phenomenon. 99mTc-ECD SPECT is an effective neuroimaging method to detect brain functional abnormalities, reflecting BBB breakdown or tissue damages, throughout the treatment course of RCVS.

Ten-Year Evaluation of the TOYOTA Prehospital Stroke Scale for Tissue Plasminogen Activator Intravenous Therapy in the Real World.

BACKGROUND: Emergency medical services are an important part of acute stroke management. We devised a prehospital stroke scale, the TOYOTA prehospital stroke scale for tissue plasminogen activator (t-PA) intravenous therapy (TOPSPIN) for triaging patients with ischemic stroke and especial indications for intravenous t-PA therapy in December 2006; this scale comprises 5 items including consciousness, atrial fibrillation, language disorder, disturbance of the upper extremities, and disturbance of the lower extremities. The aim of this study was to analyze the results of 10 years of TOPSPIN implementation and to distinguish ischemic stroke from hemorrhagic stroke in the real world. METHODS: We prospectively enrolled consecutive patients who were transferred to our hospital and evaluated by Toyota city ambulance services using the TOPSPIN from December 2006 to January 2017. We examined definite diagnosis at the time of hospital discharge (ischemic stroke, hemorrhagic stroke, or stroke mimic), positive-predictive value of the stroke, the rate of the recanalization therapy, and clinical differentiation among patients with hemorrhagic stroke, ischemic stroke, and stroke mimics. RESULTS: A total of 1,482 consecutive patients were enrolled; 1,134 (76.5%) were patients with stroke (628 ischemic-type, 34 transient ischemic attack-type, and 472 hemorrhagic-type) and 348 (23.5%) without stroke (80 with seizure, 42 with syncope, 27 with hypoglycemia, and 199 other). Among 628 patients with ischemic stroke, 130 (20.7%) received intravenous recombinant t-PA treatment, endovascular therapy, or both. The presence of atrial fibrillation, older age, lower blood pressure, and lower total TOPSPIN score was more commonly associated with ischemic stroke than with hemorrhagic stroke. In multivariable logistic regression analysis, the presence of atrial fibrillation was independently associated with ischemic stroke (OR 2.33; 95% CI 1.61-3.40). CONCLUSIONS: The TOPSPIN is a simple prehospital stroke scale that includes an assessment of atrial fibrillation. Detection of atrial fibrillation in the prehospital stage may point to a higher probability of ischemic stroke.

[A pedigree of hereditary hyperekplexia].

A 31-year old women presented with excessive startle reflex and frequent falls. Her startle reflex is induced by slight stimuli which are not problematic in most people. Soon after her startle reflex is evoked, generalized muscle stiffness occurs. She becomes rigid and falls down without loss of consciousness. Because she cannot protect herself when she is startled and falls, she has repeatedly bruised her head and face. The pedigree includes her father and two sisters with similar symptoms. Gene analysis revealed GLRA1 mutation, and she was diagnosed with hereditary hyperekplexia (HPX). Symptoms improved with clonazepam 1 mg/day. HPX patients live with severe anxiety about frequent falls and sometimes suffer serious injury, such as cerebral concussion or bone fracture. Although HPX might sometimes be underestimated, accurate diagnosis is very important for effective treatment.

Familial Pernicious Chronic Intestinal Pseudo-obstruction with a Mitochondrial DNA A3243G Mutation.

We report the case of a mother and two children who shared a mitochondrial DNA A3243G mutation. The mother had diabetes mellitus, neurogenic bladder, bradykinesia, dystonia, and slowly progressive cerebellar ataxia. Her two daughters were diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes at adolescence. They all presented with gastrointestinal symptoms at an advanced clinical stage. They were diagnosed with chronic intestinal pseudo-obstruction, and they were resistant to therapy. The mother and her youngest daughter died from aspiration pneumonia because of vomiting. The determination of chronic intestinal pseudo-obstruction is an important prognostic factor in patients with the mitochondrial DNA A3243G variant.

A case of intracranial arterial dolichoectasia with 4 repeated cerebral infarctions in 6 months and enlargement of basilar artery.

A 78-year-old man was admitted to our hospital because of sudden right hemiparesis and dysarthria. His cranial MRI showed an area of hyperintensity in left pons on DWI and MRA revealed dilated, elongated and tortuous intracranial artery. We diagnosed as acute phase ischemic stroke and intracranial arterial dolichoectasia (IADE). Intravenous infusion of rt-PA was performed 157 minutes after the onset of symptoms, and his hemiparesis improved. However, he subsequently suffered from cerebral infarction 4 times in 6 months, and we treated him twice with thrombolytic therapy. Although thrombolytic therapy was effective in the short term and antithrombotic therapy was continued, he had bilateral hemiplegia and severe dysphagia because of repeated cerebral infarctions. Hence basilar artery was dilated with intramural hemorrhage over 6 months, and we discontinued antithrombolytic therapy. It is possible that antithrombolytic therapy affects enlargement of IADE. Antithrombolytic therapy for IADE should be done carefully.

[A case of amyloid-ß-related cerebral angiitis with ApoE ε4/ε2 genotype].

A 53-year-old male with a past medical history of hypertension and bipolar disorder gradually developed gait disturbance and cognitive dysfunction over half a year. His cranial MRI showed an area of hyperintensity in the right occipital lobe on T2 weighted images and the surface of the lesion was enhanced along the sulci. We diagnosed his condition as amyloid-ß-related angiitis (ABRA) based on brain biopsy. Repeated, frequent seizures resistant to several antiepileptic drugs (AEDs) occurred after the operation. Steroid therapy was effective and the symptoms, including the intractable seizures and MRI abnormalities dramatically improved. In contrast to the common wild type ε3/ε3 ApoE genotype, a majority of ABRA patients have ε4/ε4. However, in this case the rare ε4/ε2 type was detected. The ε4 allele is considered to promote Aß deposition on the vessel wall, and ε2 is speculated to trigger vessel ruptures or vascular inflammation. Although seizure is not a common complication of brain biopsy, it occurred repeatedly and responded poorly to AEDs in this case. Surgical stress in this patient with ε2 probably induced the uncontrolled seizures. ApoE genotype may be an effective and low-invasive marker in case of suspected ABRA and in predicting the risks of the complication from brain biopsy.

[A Japanese SUNA patient with migraine without aura and medication overuse headache responsive to topiramate].

A 34-year-old woman presented with new intermittent short lasing headache around the left eye accompanied with lacrimation. She suffered from anemia and visual disturbance due to thalassaemia beta heterotype and retinitis pigmentosa. She also had continual cephalalgia from about 9 years old, and was taking nonsteroidal anti-inflammatory drug almost every day. After the medical treatment, we diagnosed her headache as migraine without aura, medication overuse headache (MOH) and short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). Triptan was effective for a migraine headache, but it was ineffective for attacks of SUNA, while topiramate dramatically reduced the SUNA attacks. A headache diary was effective to evaluate the clinical course and the effect of treatment for two different types of headaches by devising the approach to description. A migraine and MOH may coexist with SUNA, and our attention should be paid to the diagnosis and medical treatment in such cases.

[Reversible cerebral vasoconstriction syndrome with cortical subarachnoid hemorrhage presenting as thunderclap headache-a long followed up case].

A 59-year-old woman presented with thunderclap headache. Cranial CT showed cortical subarachnoid hemorrhage (cSAH) at the right parietal lobe and cerebral angiography on day 5 revealed multiple cerebral arterial constriction, diagnosed as reversible cerebral vasoconstriction syndrome (RCVS). We could not detect vasoconstriction in MRA at the first examination on day 4, and vasoconstrictive finding appeared around Willis circle 8 days later. There was a temporal difference in a cephalalgic symptom and vasoconstrictive appearance. Clinical symptoms completely recovered and head CT, MRI/MRA findings were reversible after two months, reflecting a rather good RCVS outcome. However, we also followed up this case precisely using single photon emission computed tomography (SPECT) with easy Z-score imaging system (e-ZIS), and hypoperfusion at the locus of cSAH persisted for more than one year. This finding strongly suggests that tissue damage in the cSAH locus induced by RCVS may be subclinicaly irreversible, even though clinical symptoms and abnormalities in cranial MRI and MRA completely recover.SPECT may be a high sensitive technique to detect the irreversible lesion in RCVS.

Dual-energy subtraction radiography improves laryngeal delineation in patients with moderate to severe cervical spondylosis.

PURPOSE: To investigate the feasibility of dual-energy subtraction (DES) in patients with moderate-severe cervical spondylosis for improving delineation of the larynx on flat panel detector (FPD) radiography. MATERIALS AND METHODS: For 118 patients, we graded conventional/DES anterior-posterior views for delineation of the vocal cords, subglottis, and pyriform sinus using a 5-point scale and lateral views from conventional laryngeal FPD radiography to determine cervical spondylosis severity on a scale from 0 (none) to 3 (severe). We compared the delineation of each anatomical structure in both groups of grades 0-1 and grades 2-3 of spondylosis severity between conventional and DES methods and the improved delineation rate for each anatomical structure by DES compared to the conventional method between both groups. RESULTS: With DES, the delineation of each anatomical structure was significantly better than with conventional radiography for both groups (P < 0.0001). The improved delineation rate of the vocal cord and subglottis using DES was significantly higher in grades 2-3 than in grades 0-1 (P < 0.05), although there was no significant difference in the delineation rate of the pyriform sinus between the groups (P = 0.847). CONCLUSION: DES provides better delineation of the laryngeal anatomy than conventional FPD radiography predominantly in patients with moderate-severe cervical spondylosis.

[Japanese SUNCT patient responsive to gabapentin].

We report a Japanese patient with short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUCNT) responsive to gabapentin. A 29-year-old man presented with sudden-onset intermittent left-sided orbital headache, which was not accompanied by lacrimation and conjunctival injection. We diagnosed trigeminal neuralgia at first and administered carbamazepine and loxoprofen. However, these medications were entirely ineffective at all and 6 days later, autonomic symptoms including conjunctival injection and tearing appeared. Diagnosis of SUNCT was made and gabapentin was started at up to 800 mg per day. Soon after, the headache and autonomic symptoms disappeared. Gabapantin at 800 mg per day was continued for 3 months and then reduced to 400 mg per day. Soon he had only a slight headache without tearing and conjunctival injection. He has continued to take gabapenin at 400 mg per day until now. This case indicated that headache and autonomic symptoms in SUNCT did not always emerge simultaneously, but they sometimes appear with time lag. Furthermore, the long-term clinical course and therapeutic outcome in SUNCT remain unknown. A therapeutic strategy and optimal dosage of medications including gabapentin should be established for the treatment of SUNCT.

Dual-energy subtraction imaging for diagnosing vocal cord paralysis with flat panel detector radiography.

OBJECTIVE: To investigate the clinical feasibility of dual energy subtraction (DES) imaging to improve the delineation of the vocal cord and diagnostic accuracy of vocal cord paralysis as compared with the anterior-posterior view of flat panel detector (FPD) neck radiography. MATERIALS AND METHODS: For 122 consecutive patients who underwent both a flexible laryngoscopy and conventional/DES FPD radiography, three blinded readers retrospectively graded the radiographs during phonation and inspiration on a scale of 1 (poor) to 5 (excellent) for the delineation of the vocal cord, and in consensus, reviewed the diagnostic accuracy of vocal cord paralysis employing the laryngoscopy as the reference. We compared vocal cord delineation scores and accuracy of vocal cord paralysis diagnosis by both conventional and DES techniques using kappa statistics and assessing the area under the receiver operating characteristic curve (AUC). RESULTS: Vocal cord delineation scores by DES (mean, 4.2 +/- 0.4) were significantly higher than those by conventional imaging (mean, 3.3 +/- 0.5) (p < 0.0001). Sensitivity for diagnosing vocal cord paralysis by the conventional technique was 25%, whereas the specificity was 94%. Sensitivity by DES was 75%, whereas the specificity was 96%. The diagnostic accuracy by DES was significantly superior (kappa = 0.60, AUC = 0.909) to that by conventional technique (kappa = 0.18, AUC = 0.852) (p = 0.038). CONCLUSION: Dual energy subtraction is a superior method compared to the conventional FPD radiography for delineating the vocal cord and accurately diagnosing vocal cord paralysis.

Obstructive sleep apnea syndrome (OSAS) presenting as cerebral venous thrombosis.

There are many reports that obstructive sleep apnea syndrome (OSAS) is a risk factor for ischemic stroke of arterial origin, however, the relationship between OSAS and cerebral venous thrombosis (CVT) remains unknown. We report the case of a 44-year-old man who had episodic headaches, unconsciousness attacks and seizures. Cerebral angiography showed CVT. He was obese and had severe OSAS. Noninvasive continuous positive airway pressure (CPAP) therapy prevented the recurrence of CVT as well as of headache. OSAS may be one of the multiple risk factors of CVT as well as arterial ischemic stroke.

A head-tilt caloric test for evaluating the vertical semicircular canal function.

CONCLUSIONS: The caloric test with head-tilt can be used as a tool for assessing vertical canal function as an office procedure. OBJECTIVE: Evaluation of vertical canal function. PATIENTS AND METHODS: We provoked caloric response by cold water in the vertiginous patients in supine position. During the culmination of the response we rotated the head 45 degrees from the sagittal plane to place the posterior canal to earth-vertical. Thereafter we rotated the head 45 degrees to the opposite direction to place the anterior canal to earth-vertical. The eye movements were recorded by two-dimensional electronystagmography. The data collected from the examination of 100 ears with normal caloric response in horizontal component were analyzed. RESULTS: The down-beating vertical component intensified when the posterior canal was placed to earth-vertical. The up-beating vertical component intensified when the anterior canal was placed to earth-vertical. These findings suggested that the vertical canals were functioning.

An autopsy case with non-herpetic acute limbic encephalitis (NHALE).

Non-herpetic acute limbic encephalitis (NHALE) is a cause-unknown, inflammatory disease entity that affects the limbic system restrictedly. Neuropathological changes of NHALE have been described in only one report. A 53-year-old Japanese woman developed high fever, disturbance of consciousness and intractable generalized convulsions. Her symptoms were resistant to aggressive therapy including corticosteroid, acyclovir, gamma-globulin and anticonvulsants, and progressed acutely, leading to status epilepticus at 6 days after the onset. Brain MRI revealed inflammatory changes restricted to the limbic system. Herpes encephalitis was excluded by laboratory tests. Although paraneoplastic limbic encephalitis could not be excluded completely, no tumor lesions were detected on chest CT and abdominal ultrasonography. The patient died of multiple organ failure at 34 days after the onset of the disease. Histologically, there were neuronal loss and severe gliosis with an increase in hypertrophic astrocytes and with perivascular cuffings around several small blood vessels restricted to the hippocampus and the amygdala. Immunohistochemically, macrophages and activated astrocytes were distributed more widely over the limbic system, namely internal capsule, thalamus, caudate nucleus and substantia nigra. Herpes simplex virus I(1) and II(2) were negative immunohistochemically. The neuropathological findings of this case were similar to that of the previous report. NHALE is supposed to be a distinct disease entity neuropathologically and the fulminant form of NHALE exists.