An Autopsy Case of Lambl's Excrescences with Trousseau Syndrome that Caused Cardioembolic Stroke.

The frequency and risk of embolism by Lambl's excrescences (LEs) remain unclear. We herein report an autopsy case of LEs that caused cardioembolic stroke. A 74-year-old man with colon cancer was hospitalized for ischemic stroke. His D-dimer levels were elevated. Thus, a diagnosis of ischemic stroke with Trousseau syndrome was made. At the autopsy, we found LEs in the aortic valves and thromboembolism of the brain blood vessels. This finding demonstrated that fibrin clots had adhered to the LEs because of coagulation abnormalities associated with Trousseau syndrome and became embolized. This case highlights the risk of LEs in patients with coagulation abnormalities.

Effectiveness of 7-day versus weekday-only rehabilitation for stroke patients in an acute-care hospital: a retrospective cohort study.

PURPOSE: Early rehabilitation is widely recommended for acute-stroke patients. We tested the hypothesis that the functional prognosis of stroke patients receiving daily early rehabilitation in a clinical practice setting is generally better than that of patients receiving rehabilitation only on weekdays. MATERIALS AND METHODS: We analyzed hospitalized patients who experienced either cerebral infarctions or cerebral hemorrhages and subsequently underwent rehabilitation at our hospital between October 2010 and September 2014. We examined the association between training frequency and activities of daily living improvements, as indicated by the Barthel Index (BI) effectiveness. RESULTS: In total, 661 patients with cerebral infarctions and 245 with intracerebral hemorrhages (ICHs) were analyzed. The BI effectiveness was highest for patients receiving high-frequency therapy following cerebral infarction. In addition, multiple linear regression analysis indicated that BI effectiveness was significantly and positively correlated with high-frequency therapy (coefficient, 0.072; 95% confidence interval, 0.019-0.126; p < 0.01) in patients with cerebral infarctions. There was no significant difference in BI effectiveness between therapeutic protocols for patients with ICHs. CONCLUSION: This retrospective cohort study demonstrated that extensive therapy can result in functional recovery in patients with cerebral infarctions. Implications for Rehabilitation Early intervention with intensive rehabilitation therapy is important for improving the functional recovery of patients during acute-care hospitalization. Few acute hospitals provide more than 2 h of daily rehabilitation for patients with acute stroke. In a daily clinical practice setting, this clinical study demonstrates a direct relationship between early intervention with intensive rehabilitation therapy and good functional recovery of stroke patients in an acute ward.

Intervention by Speech Therapists to Promote Oral Intake of Patients with Acute Stroke: A Retrospective Cohort Study.

OBJECTIVE: Early rehabilitation for acute stroke patients is widely recommended. We tested the hypothesis that daily intervention by speech therapists promotes safe oral intake of patients with acute stroke. METHODS: We analyzed hospitalized patients who experienced cerebral infarction and cerebral hemorrhage and who underwent rehabilitation between October 2010 and September 2014 at our hospital. In total, 936 patients were analyzed, and 452 patients underwent daily speech therapy. We examined the association of training frequency and eating status. RESULTS: Multiple linear regression analysis indicated that daily speech therapy was correlated significantly and positively with a reduction in the number of days of hospitalization until oral intake commenced (coefficient, -.998; 95% confidence interval, -1.793 to -.202; P < .05), and was not correlated with the cessation of oral intake due to aspiration pneumonia after resuming oral intake. CONCLUSION: Our retrospective cohort study demonstrated that daily intervention by speech therapists in patients with acute stroke shortens the number of days until oral intake without increasing the incidence of aspiration pneumonia.

A case of superficial hemisensory dysfunction due to operculo-insular infarction: radiological depiction of thalamocortical projections to the secondary somatosensory cortex.

A 64-year-old obese man developed hypesthesia in the left arm and leg. Neurological examination revealed decreased senses of pain, touch, and temperature in the left face, arm, trunk, and leg. Remaining functions were normal. Electrocardiogram showed atrial fibrillation. Somatosensory-evoked potentials using the stimulation in the median nerve were normal on both sides. Brain magnetic resonance imaging revealed acute infarction in the right parietal operculum and insula. There were no pathognomonic lesions in the postcentral gyrus, the thalamus, or the brain stem. Cardioembolic operculo-insular infarction was diagnosed. Diffusion tensor tractography map displayed the thalamocortical projections to the primary and the secondary somatosensory cortex (S2). These radiological findings supported that the operculo-insular lesion could disrupt the thalamo-S2 pathway. Thus, the thalamocortical disconnection between the thalamus to the S2 could cause superficial hemisensory dysfunction in the present patient.

Combined treatment of methylprednisolone pulse and memantine hydrochloride prompts recovery from neurological dysfunction and cerebral hypoperfusion in carbon monoxide poisoning: a case report.

A 49-year-old healthy man developed sudden unconsciousness under inadequate ventilation. Blood gas analysis showed carboxyhemoglobin of 7.3%. After normobaric oxygen therapy, he recovered completely 7 days later. At 3 weeks after carbon monoxide (CO) exposures, memory and gait disturbances appeared. Neurological examination revealed Mini-Mental State Examination (MMSE) score of 5 of 30 points, leg hyper-reflexia with Babinski signs, and Parkinsonism. Brain fluid-attenuated inversion recovery imaging disclosed symmetric hypointense lesions in the thalamus and the globus pallidus, and hyperintense lesions in the cerebral white matter. Brain single-photon emission tomography (SPECT) scanning with (99m)Technesium-ethyl cysteinate dimer displayed marked hypoperfusion in the cerebellum, the thalamus, the basal ganglia, and the entire cerebral cortex. He was diagnosed as CO poisoning and treated with hyperbaric oxygen therapy. The neurological deficits were not ameliorated. At 9 weeks after neurological onset, methylprednisolone (1000 mg/day, intravenous, 3 days) and memantine hydrochloride (20 mg/day, per os) were administered. Three days later, MMSE score was increased from 3 to 20 points. Neurological examination was normal 3 weeks later. Brain SPECT exhibited 20% increase of regional cerebral blood flows in the cerebellum, the thalamus, the basal ganglia, and the entire cerebral cortex. These clinicoradiological changes supported that the treatment with steroid pulse and memantine hydrochloride could prompt recovery from neurological dysfunction and cerebral hypoperfusion. Further clinical trials are warranted whether such combined therapy can attenuate neurological deficits and cerebral hypoperfusion in patients with CO poisoning.

Clinically meaningful treatment responses after switching to galantamine and with addition of memantine in patients with Alzheimer's disease receiving donepezil.

Clinical trials have shown the benefits of acetylcholinesterase inhibitors, such as donepezil and galantamine, and an N-methyl-D-aspartate receptor antagonist, memantine, in patients with Alzheimer's disease (AD). However, little is known regarding the effects of switching from donepezil 5 mg/day to galantamine 16 or 24 mg/day, or regarding the effects of adding memantine to established therapy compared with increasing the dose of donepezil. This report discusses two studies conducted to evaluate treatment with galantamine and memantine with respect to cognitive benefits and caregiver evaluations in patients with AD receiving donepezil 5 mg/day for more than 6 months. Patients with mild or moderate AD (scores 10-22 on the Mini-Mental State Examination) were enrolled in the Galantamine Switch study and switched to galantamine (maximum doses 16 mg versus 24 mg). Patients with moderate to severe AD (Mini-Mental State Examination scores 3-14) were enrolled in the Donepezil Increase versus Additional Memantine study and either had their donepezil dose increased to 10 mg/day or memantine 20 mg/day added to their existing donepezil dose. Patients received the study treatment for 28 weeks and their Disability Assessment for Dementia, Mental Function Impairment Scale, Cohen-Mansfield Agitation Inventory, and Neuropsychiatric Inventory scores were assessed with assistance from their caregivers. For the Galantamine Switch study after 8 weeks, agitation evaluated by the Cohen-Mansfield Agitation Inventory improved in both the 16 mg and 24 mg groups compared with baseline. However, there were no significant differences between the two galantamine groups. Agitation was also less in patients in the additional memantine group than in the donepezil increase group. In summary, switching to galantamine from donepezil and addition of memantine in patients with AD receiving donepezil were both safe and meaningful treatment options, and particularly efficacious for suppression of agitation.

Limb-onset amyotrophic lateral sclerosis patients visiting orthopedist show a longer time-to-diagnosis since symptom onset.

BACKGROUND: There have been several reports concerning the survival time after symptom onset in patients with amyotrophic lateral sclerosis (ALS). However, little is known about how the choice of physician (i.e., general practitioner, neurologist, etc.) may affect the time it takes for a diagnosis of ALS to be made. METHODS: We conducted a retrospective study, covering a 20-year period, comparing the type of physician first consulted by an ALS patient at the time of initial symptoms and the amount of time that elapsed to the final diagnosis of ALS. A total of 202 patients were diagnosed and stratified according to the onset of ALS symptoms (bulbar onset [BO] and limb onset [LO]). We noted the type of physician first seen by the patient. The diagnostic interval was calculated as the time between onset of symptoms and the final diagnosis of ALS. RESULTS: A total of 202 ALS patients were examined. Clinical BO and LO was observed in 78 (36.6%) and in 124 (61.4%) of these patients, respectively. The type of physician examining these patients at the first symptoms of disease was as follows (BO and LO): neurologist (38.5% and 25.8%), general practitioner (14.1% and 35.5%), orthopedist (12.8% and 35.5%), otolaryngologist (15.4% and 0%), and neurosurgeon (14.1% and 3.2%). Mean diagnostic interval (standard deviation) for patients with either set of symptoms was 13.1 (6.5) months; the diagnostic interval of patients with BO and LO was 9.2 (4.5) and 15.2 (7.7) months, respectively. ALS diagnosis in LO patients was delayed by more than 10 months when the patient first consulted an orthopedist rather than a neurologist. CONCLUSION: More than 50% of the ALS patients included in this study did not visit a neurologist at the first symptoms of disease onset. The diagnosis of ALS was prolonged in LO patients visiting an orthopedist. We speculate that this increase in the diagnostic interval in LO patients visiting an orthopedist was due to a lack of bulbar symptoms in the early stages of this disease.

Pulse wave velocity study in middle-aged migraineurs at low cardiovascular disease risk.

BACKGROUND: Migraine is associated with an increased risk for ischemic stroke and cardiovascular disease (CVD). Recent studies have suggested vascular dysfunction in the aorta, the brachial and femoral artery. Little is known about such arterial changes in Japanese midlife migraineurs. We aimed to evaluate arterial pulse wave velocity (PWV) and ankle-brachial index (ABI) in middle-aged migraineurs at low CVD risk. METHODS: Brachial-ankle PWV (baPWV) and ABI, using an oscillometric technique, were measured in 111 migraineurs (81 women and 30 men) and 110 controls. All participants had no CVD risk factors. Statistical comparison of baPWV and ABI between both groups and the relationship to clinical variables of migraineurs were analyzed. RESULTS: Twenty-two subjects had migraine with aura and 89 had migraine without aura. Mean age (SD) of migraineurs was 44.4 (8.4) years. Mean duration (SD) was 18.0 (10.8) years. Attack frequency was 60 subjects in ≥1 time/month and 51 subjects in <1 time/month. Mean score (SD) of Headache Impact Test-6 (HIT-6) was 61.4 (8.7). CVD risk profile did not differ statistically between migraineurs and controls. Mean baPWV (SD) of migraineurs was 1247 (189) cm/second in women and 1356 (126) in men. That of controls was 1138 (136) in women and 1250 (121) in men. baPWV was increased significantly in female and male migraineurs. Mean ABI (SD) was 1.05 (0.06; 1.04 [0.07] in men and 1.05 [0.06] in women) in migraineurs and 1.06 (0.07) in controls (1.05 [0.08] in men and 1.06 [0.08] in women). ABI did not differ statistically between migraineurs and controls. Migraine subtypes, duration, attack frequency, and HIT-6 score were not associated with baPWV and ABI. CONCLUSION: The present study indicated higher baPWV in midlife migraineurs without CVD risk factors. This pathogenesis could reflect distinct vascular reactivity rather than arterial stiffness due to atherosclerosis.

Serological profiles of urate, paraoxonase-1, ferritin and lipid in Parkinson's disease: changes linked to disease progression.

BACKGROUND: Oxidative stress plays a role in the pathogenesis of neuronal death. Serum levels of urate or lipid were associated with the incidence of Parkinson's disease (PD). OBJECTIVE: We compared urate, paraoxonase-1 (PON1), iron, ferritin and lipid in sera of 119 PD patients and 120 healthy controls matched by age, sex and body mass index. We aimed to elucidate whether those serological data are correlated with disease progression. RESULTS: Mean age (SD) of PD patients was 73.4 (8.7) years. Mean Yahr stage (SD) was 3.2 (0.9). Mean disease duration (SD) was 6.9 (5.1) years. Mean dose of L-DOPA (SD) was 355 (157) mg/day. As compared to controls, serum levels of total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), urate and PON1 activity were significantly reduced, and serum ferritin levels were significantly increased in male and female PD patients. Serum urate levels and PON1 activities were inversely related, and serum ferritin levels were correlated with Yahr stage and PD duration in men and women. Serum levels of TC and LDL-C were inversely related to Yahr stage or PD duration in female patients. CONCLUSIONS: Our studies indicated serological profiles of urate, PON1, ferritin, TC and LDL-C in PD patients. These serological changes were linked to PD progression. Metabolism of lipid, oxidant- and antioxidant-related substances may contribute to the pathogenesis and the progression of PD.

Cheiro-oral syndrome associated with lateral medullary infarction.

A 62-year-old man was admitted to our department because of the sudden onset of dysaesthesia in the right perioral region of the face and right hand. Neurological examination demonstrated a loss of pain and temperature sensation in both the right perioral region and in the fingers of the right hand. These findings suggested damage to parts of the ipsilateral spinothalamic and trigeminothalamic ascending tracts, producing restricted sensory impairment of the perioral region and ipsilateral hand. This is the first reported case of a medullary lesion causing cheiro-oral syndrome.

A distinct phenotype of leg hyperreflexia in a Japanese family with Gerstmann-Sträussler-Scheinker syndrome (P102L).

Gerstmann-Sträussler-Scheinker Syndrome (GSS) is an inherited prion disease characterized by midlife onset and slowly progression of cerebellar ataxia and dementia. We report a distinct phenotype of leg hyperreflexia in a Japanese family with GSS. A 38-year-old woman noticed unsteady gait at 33 years of age. Afterwards, dysarthria and writing difficulty were appeared. Her family history revealed that her grandfather and her mother had a clinical history of unsteadiness and mental changes. At 1 year after clinical onset, neurological examination showed cerebellar ataxia and leg hyperreflexia. At 4 years after onset, she suddenly developed insomnia and nocturnal howling. Her mental status disclosed marked disorientation, anxiety and irritability. Muscle stretch reflexes were increased in four extremities with Babinski's signs. Remarkable dysarthria and cerebellar ataxia were presented. Brain diffusion weighted imaging showed extensive hyperintensity signal areas in the cerebral cortex. A point mutation of the prion protein gene (PRNP) at codon 102 resulting in the substitution of proline by leucine (P102L) was identified. PRNP polymorphism exhibited homozygous methionine at codon 129 and homozygous glutamate at codon 219. She had verbal perseveration, somnolence and myoclonus of lower limbs, leading to akinetic mutism at 4 months after neuropsychiatric events. Phenotypic hallmark of our patient indicates leg hyperreflexia from an early disease course. This neurological sign differs from the previously reported clinical expression of Japanese and foreign patients with GSS (P102L). Thus, physicians should pay more attention to phenotypic heterogeneity in this prion disease.

Atorvastatin treatment attenuates motor neuron degeneration in wobbler mice.

We aimed to study whether atorvastatin treatment can retard motor neuron degeneration in wobbler mice. Wobbler mice and their normal littermates were fed 0.01% atorvastatin-mixed food (10 mg/kg/day) or regular food from age 3-4 weeks of disease onset for four weeks (n=10/group) or more than eight weeks (n=10/group). Motor function was evaluated by pull-strength and deformity scale of the forelimbs. For those symptomatic assessment and body weight were measured weekly. Neuropathological and biochemical changes of the biceps muscle and the cervical cord were analyzed at four weeks after treatment. Compared to vehicle, atorvastatin-treated wobbler mice delayed progression of forelimb motor deficits by more than four weeks. Wobbler mice significantly increased body weight from three weeks post-treatment of atorvastatin, compared with vehicle (p<0.05). Atorvastatin administration suppressed denervation atrophy at 30% (p<0.01), maintained choline acetyl transferase activities of the biceps muscle (p<0.05), and attenuated approximately 30% loss of motor neurons in wobbler mice (p<0.01). Atorvastatin fed to normal littermates did not influence body weight gain, neuropathological and biochemical findings. These data suggest that atorvastatin has neuroprotective effects on denervating muscles and degenerating motor neurons in wobbler mice.

Scopolamine-induced migraine like headache.

OBJECTIVE: Scopolamine butylbromide (SB), a muscarinic receptor antagonist, is used commonly in gastric X-ray examination in the physical check-up in Japan. This study describes clinical features of SB-induced headache. PATIENTS AND METHODS: SB-induced headache was defined as headache that started within 20 minutes after intramuscular administration of SB (20 mg/body). The Primary and the secondary headaches were diagnosed according to the ICHD-II criteria. SB-induced headache was classified as headache induced by acute substance use or that due to exposure (ICHD-II code 8.1). Clinical features and background of subjects with SB-induced headache were analyzed. We also estimated the frequency of SB-related headache between migraineurs and non-migraineurs. RESULTS: A total of 54 subjects (39 women and 15 men) experienced SB-induced headache. All subjects had the present history of migraine. Nine subjects had > or =2 times of the headache. Mean age (SD) was 46.2 (9.7) years [46.2 (9.7) for women and 46.3 (10.0) for men]. Clinical hallmarks of headache showed that pulsating / throbbing pain occurred in diffuse or bilateral head sites. Headache worsened at 20-30 minutes from the onset and persisted for 6-18 hours, and ameliorated gradually 8 hours later. All subjects had repeated nausea and vomiting. Severity of headache revealed severe degree requiring complete bed rest in 50 subjects (92.6%). SB-induced headache had similar characteristics as migraine without aura (MO) attacks. Liver and renal functions were normal in all SB-related migraineurs. They had no allergic history of medication and food. In 1,865 non-migraine controls, one healthy subject had a mild degree of migraine like headache triggered by SB injection. CONCLUSION: SB triggers a severe degree MO like headache or worsens pre-existing migraine in some migraineurs. SB-induced headache could contribute to disequilibrium between acetylcholine and other neuropeptides. We should use SB more carefully as it can be an aggravating drug of migraine.