Successful treatment of ischemic stroke associated with brachiocephalic artery stenosis using alteplase.

Introduction: Brachiocephalic artery stenosis rarely causes right hemispheric infarction with associated left hemiparesis. To date, there have been no reported cases of stroke associated with brachiocephalic artery stenosis that were successfully treated with recombinant tissue-type plasminogen activator (rt-PA), alteplase. Case Report: An 80-year-old woman presented with left hemiparesis. Brain computed tomography showed no hemorrhage, and computed tomography angiography demonstrated brachiocephalic artery stenosis. Alteplase was administered based on a diagnosis of ischemic stroke. Brain magnetic resonance imaging showed multiple acute infarctions. Thereafter, the blood pressure of the right arm was found to be lower than that of the left arm. The patient's neurological deficits gradually improved; she was eventually able to walk again and was thus discharged home. Conclusion: While the combination of left hemiparesis and a decrease in blood pressure in the right arm are well known in patients with stroke associated with Stanford type A aortic dissections, it may also occur in patients with stroke due to brachiocephalic artery stenosis. Unlike stroke associated with Stanford type A aortic dissections, stroke due to brachiocephalic artery stenosis may be treated with alteplase.

Distribution of Deep Gray Matter Lesions on Magnetic Resonance Imaging in Lymphomatosis Cerebri.

We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus. Interestingly, we found seven previous reports of similar MRI findings, with relative sparing of the striatum, in patients with LC. This finding may be characteristic of LC and help facilitate its diagnosis. Further investigations of a larger number of LC patients are necessary to confirm these findings.

Practical guide to choosing dabigatran 150 mg twice daily or apixaban 5 mg twice daily for patients with atrial fibrillation.

Based on previous reports, we propose a practical guide to choose dabigatran 150 mg twice daily or apixaban 5 mg twice daily for patients with atrial fibrillation. We recommend the use of dabigatran 150 mg twice daily for patients with atrial fibrillation who have a high risk of embolism (e.g., ischemic stroke on other oral anticoagulants, presence of left atrial appendage thrombus) and a low risk of bleeding. However, the prevalence of such patients with atrial fibrillation is considered low because patients with atrial fibrillation with a high risk of embolism usually have a high risk of bleeding. In most other patients with atrial fibrillation, the use of apixaban 5 mg twice daily should be considered.

Reversible brainstem hypertensive encephalopathy (RBHE): Clinicoradiologic dissociation.

We report two cases of reversible brainstem hypertensive encephalopathy (RBHE) with unusual magnetic resonance (MR) findings. Patient 1, an 85-year-old man without a history of hypertension, developed acute severe hypertension and mild consciousness disturbance as the only symptoms. Patient 2, a 46-year-old man with an untreated hypertension, presented with extremely high blood pressure and general fatigue, vertigo, and mild dysarthria as the initial manifestations. In these patients, fluid-attenuated inversion recovery (FLAIR) and T2-weighted MR images revealed diffuse hyperintensities in the brainstem. Diffusion-weighted imaging (DWI) findings were normal, and apparent diffusion coefficient (ADC) values were increased in the brainstem. The supratentorial regions were largely spared, and mildly diffuse hyperintensities were noted in the white matter. There were no accompanying changes in the occipital lobe and cerebellum. The lesions completely resolved after stabilization of blood pressure. The normal DWI findings and high ADC values were consistent with vasogenic edema due to severe hypertension. The characteristics of RBHE are a very high blood pressure, mild clinical and neurologic symptoms, rapidly improved MR findings after initial treatment with the control of hypertension, and a marked clinicoradiologic dissociation.

[Dropped head syndrome as first manifestation of primary hyperparathyroid myopathy].

75 years old woman presented with 6-month history of progressive dropped head syndrome. Neurological examination revealed moderate weakness of flexor and extensor of neck and mild weakness of proximal appendicular muscles with normal deep tendon reflexes. The needle electromyography showed short duration and low amplitude motor unit potential. No fibrillation potentials or positive sharp waves were seen. Biopsy of deltoid muscle was normal. Laboratory studies showed elevated levels of serum calcium (11.8 mg/dl, upper limit of normal 10.1) and intact parathyroid hormone (104 pg/ml, upper limit of normal 65), and decreased level of serum phosphorus (2.3 mg/dl, lower limit of normal 2.7). Ultrasonography and enhanced computed tomography revealed a parathyroid tumor. The tumor was removed surgically. Pathological examination proved tumor to be parathyroid adenoma. Dropped head and weakness of muscles were dramatically improved within a week after the operation. Although hyperparathyroidism is a rare cause of dropped head syndrome, neurologists must recognize hyperparathyroidism as a treatable cause of dropped head syndrome.

Acute pancreatitis is a very rare comorbidity of acute ischemic stroke.

Background: Although acute pancreatitis is listed among the exclusion criteria for the administration of recombinant tissue plasminogen activator according to the Japanese Guideline for the Management of Stroke, the co-occurrence of acute pancreatitis and acute ischemic stroke has not been investigated. The present study aimed to assess the incidence rate of acute pancreatitis in patients with acute ischemic stroke. Methods: This study consecutively enrolled all patients with ischemic stroke admitted to the Department of Neurology, JA Toride Medical Center between April 2014 and March 2016. Diagnosis of acute pancreatitis was made according to the revised Atlanta Classification of Acute Pancreatitis. We retrospectively analyzed serum amylase activity and the frequency of acute pancreatitis as a comorbidity of ischemic stroke. Results: A total of 411 ischemic stroke patients were included. Serum amylase activity was measured for 364 patients, 27 of whom presented with amylase activity exceeding the upper limit of normal. In two patients with serum amylase activity greater than three times-fold the upper limit of normal, computed tomography or transabdominal ultrasonography showed no characteristic findings of acute pancreatitis. No patient in the cohort met the diagnostic criteria for acute pancreatitis. Conclusions: Acute pancreatitis is a very rare comorbidity of acute ischemic stroke.

Retrobulbar optic neuropathy associated with sphenoid sinus mucormycosis.

Because fungi usually spread from the paranasal sinuses to the orbital apex in invasive fungal sinusitis (IFS), IFS often presents as an orbital apex syndrome (OAS) characterized by dysfunction of cranial nerves II, III, IV, V1, and VI. We report a case of sphenoid sinus mucormycosis that presented as isolated retrobulbar optic neuropathy. A 94-year-old woman presented with acute blindness in the right eye. Examination revealed the absence of light perception and pupillary reflex in the right eye. Head MRI showed a mass in the right sphenoid sinus, which was contiguous with the right optic nerve. She underwent endoscopic surgery, and a histopathological diagnosis of mucormycosis was established. Treatment with intravenous liposomal amphotericin B reduced the size of the mass. She has survived for more than 1 year without recurrence. Clinicians should consider that IFS can present as isolated retrobulbar optic neuropathy.

Spinocerebellar Ataxia Type 31 with Blepharospasm.

A 58-year-old man consulted our hospital due to a 2-year history of dysarthria and a 1-month history of blepharospasm. In addition to the ataxic dysarthria and blepharospasm, a neurological examination demonstrated slight ataxia of the trunk and lower limbs. Brain MRI demonstrated atrophy of the upper portion of the cerebellar vermis. Gene analysis established a diagnosis of spinocerebellar ataxia type 31 (SCA31). Single photon emission computed tomography (SPECT) with the three-dimensional stereotaxic ROI template (3DSRT) software program demonstrated hyperperfusion in the lenticular nucleus and thalamus. Although the association between SCA31 and blepharospasm in our patient remains unclear, we considered that this combination might be more than coincidental.

Bilateral Frontal Lobe Vasogenic Edema Resulting from Hypertrophic Pachymeningitis due to Granulomatosis with Polyangiitis.

A 61-year-old woman presented with a 1-month history of decreased activities of daily living. Magnetic resonance imaging revealed abnormal intensities of the bilateral frontal lobes and enhancement of the thickened dura matter. A biopsy of the dura mater revealed multinucleated giant cells. She had sinusitis and hematuria; she was diagnosed with granulomatosis with polyangiitis. Hypertrophic pachymeningitis (HPM) was considered to have interrupted the venous flow and caused vasogenic edema. Bilateral frontal lobe edema resulting from HPM due to granulomatosis with polyangiitis has not been reported. A biopsy and examination for other organ complications were useful for the diagnosis and treatment of our patient.

Pneumococcal Pyomyositis of the Neck Muscles.

High fever, severe neck pain and neck stiffness can result from meningitis. We report a case of pneumococcal pyomyositis of the neck muscles. A 72-year-old man developed high fever and severe neck pain. His chief complaint mimicked bacterial meningitis. Although his condition was initially suspected to be bacterial meningitis, his cerebrospinal fluid did not show pleocytosis. MRI showed areas of high intensity in the posterior deep neck muscles on short tau inversion recovery (STIR) sequences and gadolinium-enhanced T1-weighted images. Pneumococcal myositis should therefore be included in the differential diagnosis of severe neck pain with fever.

Disappearance of the Hummingbird Sign after Shunt Surgery in a Case of Idiopathic Normal Pressure Hydrocephalus.

A 79-year-old man presented with a slowly progressive gait disturbance. Brain MRI demonstrated ventriculomegaly and the hummingbird sign. A lumbar puncture showed no abnormalities of the cerebrospinal fluid. The improvement of the gait disturbance after the ventriculoperitoneal shunt led to a diagnosis of idiopathic normal pressure hydrocephalus. Interestingly, postoperative brain MRI demonstrated the disappearance of not only ventriculomegaly, but also the hummingbird sign. The disappearance of the hummingbird sign suggests that an increase in the cerebrospinal fluid in the lateral and third ventricles could cause the compression of the superior surface of the midbrain tegmentum, which manifests as the hummingbird sign.

Perspectives concerning living wills in medical staff of a main regional hospital in Japan.

OBJECTIVE: Living wills, written types of advanced directives, are now widespread in western countries, but in Japan, their recognition still remains restricted to a small part of the population. As an initial step to introduction of such patient-oriented medicine, we surveyed present recognition and acceptance patterns concerning living wills in a main regional hospital located in a suburban area of Tokyo. METHODS: Without any preceding guidance on living wills, the questionnaire on living wills was distributed to all the staff working at JA Toride Medical Center in September 2013, and their responses were collected for analysis within one month. RESULTS: Questionnaires were distributed to all hospital staff, 843 in total, and 674 responses (80.0% of distributed) were obtained. The term of living will was known by 304 (45.1%) of the respondents, and introduction of living wills to patients was accepted in 373 (55.3%) of the respondents, meanwhile, 286 (42.4%) respondents did not indicate their attitude toward living wills. As to styles of document form, 332 respondents (49.3%) supported selection of wanted or unwanted medical treatments and care from a prepared list, and 102 respondents (15.1%) supported description of living wills in free form. As preferred treatment options that should be provided as a checklist, cardiac massage (chest compression) and a ventilator were selected by more than half of the respondents. Based on their responses, we developed an original type of living wills available to patients visiting the hospital. CONCLUSIONS: Although not all the respondents were aware of living wills even in this main regional hospital, introduction of living wills to patients was accepted by many of the hospital staff. Awareness programs or information campaigns are needed to introduce living wills to support patient-centered medicine.

Sphenoid Sinusitis Complicated by Pneumococcal Meningitis and an Infectious Aneurysm in the Intracavernous Carotid Artery.

A 61-year-old Japanese woman presented with a headache and appetite loss lasting for nine days and was admitted to our hospital, where she was diagnosed with pneumococcal meningitis associated with acute sphenoid sinusitis. While the administration of meropenem and dexamethasone ameliorated the meningitis, right third and sixth nerve palsy suddenly developed 10 days after admission. CT angiography subsequently demonstrated an aneurysm in the cavernous portion of the right internal carotid artery. This is the first reported case of sphenoid sinusitis simultaneously complicated by both pneumococcal meningitis and an infectious aneurysm in the intracavernous carotid artery.

Survival-determining factors in patients with neurologic impairments who received home health care in Japan.

BACKGROUND: The Japanese have become the longest-lived nation population in the world, and numbers of elderly who require medical and nursing care are increasing. The capacity of nursing homes and nursing institutions is sharply limited in Japan; further, as a group, elderly Japanese patients prefer home care to institutional care. For these reasons, the home health care system in Japan has been increasingly important. OBJECTIVE: We sought to identify factors determining long-term survival in Japanese patients receiving home health care for neurologic disorders. PATIENTS AND METHODS: We retrospectively evaluated 180 patients with neurologic disease, who received home health care conducted by our hospital between 1992 and 2001. Factors considered were age; gender; illnesses; prognosis; follow-up period; activities of daily living (ADL); behavioral, cognitive, and communicative functions; swallowing function; feeding method; serum nutritional values (total protein, albumin, and total cholesterol); hemoglobin concentration; and social care services provided at home. RESULTS: Variables affecting long-term survival in 180 patients with neurologic disease were age (P<0.0002) and severity of dysphagia (P<0.04) by Cox's proportional hazard test. CONCLUSION: Maintenance of swallowing function and adequate nutrition through a variety of feeding methods that can be provided by a home health care program are important for long-term survival of patients with stroke and also that of patients with other neurologic diseases.

Polymyalgia rheumatica (PMR): clinical, laboratory, and immunofluorescence studies in 13 patients.

Thirteen elderly patients with polymyalgia rheumatica (PMR) are presented. The clinical and laboratory findings suggest that many progressive symptoms are due to the non-specific inflammatory changes in various organs of the body, especially in muscles and joints. An immunofluorescence study of muscle biopsy specimens revealed IgG, IgA, and fibrinogen deposits in the perifascicular area of the perimysium. This finding suggests that immune complexes play a role in the pathogenesis of this condition and that the pathophysiology of PMR is an interstitial inflammatory process. We think that the inflammatory findings affecting the interstitial tissue of muscles in the immunofluorescence study are relatively specific to PMR, and will be affected by steroid treatment.

Right hand predominant constructional apraxia due to right hemisphere infarction without corpus callosum lesions.

A 74-year-old right-handed woman without cognitive impairment suddenly developed nonfluent aphasia. Brain MRI showed acute infarction in the right frontal lobe and insula without involvement of the corpus callosum. A neurological examination demonstrated not only transcortical motor aphasia, but also ideomotor apraxia and right hand predominant constructional apraxia (CA). To date, right hand predominant CA has only been reported in patients with corpus callosum lesions. The right hand predominant CA observed in our patient may be associated with the failure to transfer information on the spatial structure from the right hemisphere to the motor cortex of the left hemisphere.

Efficacy and ethics of artificial nutrition in patients with neurologic impairments in home care.

Outcomes, particularly survival, for home-care patients with neurologic impairments who receive artificial nutrition, such as home parenteral nutrition (HPN) or percutaneous endoscopic gastrostomy (PEG) feeding, remain unclear. The efficacy of tube feeding for life prolongation in elderly patients remains controversial. The aim of this study was to assess the survival of elderly patients with neurologic impairments after the start of HPN or PEG. We retrospectively evaluated 80 patients with neurologic impairments who had received home care before they died. They were divided into three groups according to feeding method: oral-intake group (n = 23), HPN group (n = 21) and PEG group (n = 36). The factors considered were: age; survival period after commencement of home care; swallowing function; serum albumin concentration; level of activities of daily living (ADL); and behavioral, cognitive and communication functions. Survival periods of the patients in the PEG (736 ± 765 days) and HPN (725 ± 616 days) groups were twice that of the self-feeding oral-intake group (399 ± 257 days) despite lower serum albumin concentration (for PEG patients), reduced swallowing function and cognitive function, and poorer levels of ADL at the start of home care. Almost all patients were incapable of deciding whether they should receive artificial nutrition due to dementia or poor comprehension. Physicians should provide clinical evidence to families before commencing PEG feeding or HPN and support their decisions to maintain the dignity of the patient.

Thalamic hypoperfusion in early stage of progressive supranuclear palsy (Richardson's syndrome): report of an autopsy-confirmed case.

Progressive supranuclear palsy-Richardson's syndrome (PSP-RS) is a neurodegenerative disease characterized by postural instability and vertical gaze palsy, but the clinical diagnosis of PSP-RS is often difficult in the early stage of the disease. A 64-year-old male experienced frequent falls, followed by dysarthria and dysphagia. Neurological examination at age 64 demonstrated vertical gaze palsy, dysarthria, dysphagia, and retropulsion. At that time, while brain MRI demonstrated no apparent abnormalities, SPECT showed the reduction of the cerebral blood flow in the thalamus as well as the medial frontal lobe cortices. The patient was diagnosed with probable PSP-RS, and died at age 70. On postmortem examination, there were abundant tuft-shaped astrocytes, neurofibrillary tangles, coiled bodies, and argyrophilic threads in the brain, establishing the diagnosis of PSP-RS. Our definite PSP-RS case suggests that thalamic hypoperfusion may provide helpful evidence to support a diagnosis of PSP-RS in the early stage of the disease.