Takotsubo cardiomyopathy in Guillain-Barré syndrome.

BACKGROUND AND PURPOSE: Takotsubo cardiomyopathy (TCM) is a serious autonomic complication of Guillain-Barré syndrome (GBS). However, the association between TCM and GBS has not been investigated in detail. We investigated the characteristics of GBS patients with TCM (GBS-TCM). METHODS: Clinical features and anti-ganglioside antibody between the GBS-TCM patients and 62 classical GBS patients without TCM as control patients were compared. RESULTS: Eight GBS-TCM patients were identified, in whom TCM was diagnosed at a mean of 6.5 [range 3-42] days after the onset of GBS. The age at onset of GBS was elder in the GBS-TCM patients than in the control GBS patients (76.5 [56-87] vs. 52 [20-88] years, p < 0.01). Notably, cranial nerve deficits, particularly in the lower cranial nerves, were observed in all GBS-TCM patients (100% vs. 41.9%, p < 0.01). Additionally, the GBS-TCM patients showed a higher GBS disability score at nadir (5 [4-5] vs. 4 [1-5], p < 0.01), and lower Medical Research Council sum scores at admission and nadir (37 [30-44] vs. 48 [12-60] at admission, p < 0.05, and 20 [12-44] vs. 40 [0-60] at nadir, p < 0.05, respectively). Mechanical ventilation was more frequently required in the GBS-TCM patients (62.5% vs. 11.3%, p < 0.01). Three GBS-TCM patients were positive for anti-ganglioside antibodies. CONCLUSIONS: TCM occurred at a relatively early phase of GBS. The characteristics of GBS-TCM were the elder, lower cranial nerve involvements, severe limb weakness, and respiratory failure.

Thrombolysis and Mechanical Thrombectomy for Acute Ischemic Stroke in Pregnancy: A Case Report.

Objective: Intravenous (IV) recombinant tissue plasminogen activator (rt-PA) and mechanical thrombectomy (MT) are effective treatments for acute ischemic stroke (AIS). However, the treatment for AIS in pregnancy is not established because no clinical trials have included pregnant patients. We present a case of middle cerebral artery (MCA) M2 segment occlusion in pregnancy treated with IV thrombolysis and endovascular therapy. Case Presentation: A 36-year-old woman being 6 weeks pregnant presented with right-sided hemiparesis and aphasia. MRI showed a high-intensity area on diffusion-weighted imaging of the left parietal lobe, and MRA showed left MCA M2 segment occlusion. She underwent IV rt-PA and MT and achieved thrombolysis in cerebral infarction 2b revascularization without complications. The protein S concentration was lower than that in the physiological changes during pregnancy. She was diagnosed with embolic stroke related to coagulopathy in pregnancy, and she underwent anticoagulation. At the 3-month follow-up, the modified Rankin Scale was 0. She miscarried at 4 months, and the fetal death was presumed to be obstetric cause. Conclusion: IV rt-PA and MT may be effective and safe treatments for pregnant patients. Estimated fetal radiation exposure during MT is low and is presumed not to affect fetal development. We should mitigate the radiation dose and reduce the dose of iodinated contrast agents, particularly in pregnant patients.

Mechanical Thrombectomy for Acute Middle Cerebral Artery Occlusion Caused by Tumor Embolism: A Case Report.

Objective: We report a case of acute middle cerebral artery (MCA) occlusion caused by tumor embolism. Case Presentation: A 64-year-old man with lung cancer presented with sudden onset left-sided hemiparesis and sensory disturbance. Diffusion-weighted imaging (DWI) revealed hyper-intense foci in the right MCA territory and magnetic resonance angiography (MRA) demonstrated right MCA M2 segment occlusion. Mechanical thrombectomy (MT) was performed with Thrombolysis in Cerebral Infarction 2B recanalization. On histopathology, thrombus composed of fibrin and squamous cell carcinoma was observed. We diagnosed him with tumor embolism from lung cancer that invaded the pulmonary vein and the left atrium. Conclusion: Tumor cells may be confirmed by pathological examination regardless of the morphology of the embolus. Pathological examination of the cerebral embolus is useful for the accurate diagnosis of ischemic stroke subtypes.

[An autopsy case of elderly-onset herpes simplex encephalitis with acute respiratory failure caused by brainstem lesions].

An 89-year-old man was admitted because of persistent fever and impaired consciousness. On admission, his consciousness level was E3V3M4 according to the Glasgow Coma Scale. MRI of the brain showed high intensity lesions in the bilateral cingulate gyri. In the cerebrospinal fluid, both cell counts and glucose level were in the normal ranges. He had received antibiotics and intravenous isotonic saline. On the fifth day of hospitalization, blood examination revealed elevation of anti-herpes simplex virus (HSV) immunoglobulin M antibody, and herpes simplex encephalitis (HSE) was diagnosed. Despite treatment with acyclovir, his respiratory function and consciousness level deteriorated rapidly. On the eighth day, he died of respiratory failure. At autopsy, the brain showed multiple softenings of the gray and white matter in the hippocampus, amygdala, and temporal, insular, and cingulate cortices. Some of these lesions were hemorrhagic. Microscopic examination revealed that the lesions were necrotic and associated with perivascular inflammatory cell infiltration in the limbic system, hypothalamus, brainstem tegmentum area, and medulla. Eosinophilic intranuclear inclusions were rarely found in the astrocytes in the medulla. Immunohistochemistry revealed anti-HSV-1 antibody positive neurons in the brainstem tegmentum including reticular formation and the raphe nuclei. HSV-DNA was also detected in the postmortem cerebrospinal fluid. This was a rare case of HSE in which inflammation in the brainstem proved to be the cause of lethal respiratory failure.

[A case of systemic AL amyloidosis diagnosed on muscle biopsy].

A 69-year-old man was admitted to our hospital with a 1-year history of progressive easy fatigability while walking. He presented with proximal muscle weakness dominant in the lower extremities, hoarseness, and mild dysphagia. Muscle pseudo-hypertrophy was observed in the gastrocnemius. A biopsy specimen from the left deltoid muscle revealed amyloid deposition in the blood vessels and ring-like fibers. These findings suggested amyloid myopathy. The serum and urine immunofixation electrophoresis detected κ type Bence-Jones proteins, and bone marrow examination showed an increase in atypical plasma cells; thus, we established a diagnosis of multiple myeloma. Thereafter, he experienced frequent diarrhea, and the gastrointestinal endoscopy revealed extensive amyloid deposition in the upper and lower digestive tract. We started treatment with lenalidomide and dexamethasone; however, his condition worsened, and he died of aspiration pneumonia. Amyloid myopathy indicated systemic AL amyloidosis; therefore, muscle biopsy was necessary in this case.

[A case of cholesterol embolization syndrome with cognitive impairment and pulmonary hemorrhage].

A seventy-year-old man developed color change in his left toes and was treated for frostbite. Eight months later, he developed cognitive impairment and was admitted to our hospital. A remarkable increase of eosinophils was observed in peripheral blood. Brain MRI revealed abnormal lesions in the fornix, corpus callosum, basal ganglia and frontal lobe. Steroid therapy ameliorated his symptom temporarily, but he suddenly developed cardiopulmonary arrest. His autopsy revealed severe pulmonary hemorrhage with alveolar vasculitis and cholesterol crystals in the brain, kidneys, liver, and the other organs. It was possible that cholesterol embolization to multiple organs including the brain induced systemic vasculitis that caused pulmonary hemorrhage and his critical prognosis. Cholesterol embolization should be considered when we see a patient with brain lesions accompanied with eosinophilia.

[Brain abscess mediated through a pulmonary arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia].

The patient is a 66-year-old man with hereditary telangiectasia. He was diagnosed with pulmonary arteriovenous malformation (PAVM), which was revealed by contrast-enhanced chest computed tomography at the age of 65. He developed headache, right homonymous hemianopsia, and right hemiparesis and was admitted to our hospital. Contrast-enhanced magnetic resonance imaging revealed multiple lesions in the left hemisphere, which indicates brain abscesses. Thus, the diagnosis of brain abscess mediated through PAVM was established. Following management with drainage and coil embolization, all neurological symptoms resolved. Therefore, coil embolization should be considered for PAVM at an early stage to prevent brain abscess, even if it is asymptomatic.

[A case of possible neuro-Sweet disease with prolonged disturbance of consciousness and no dermal lesion during the course of dementia].

The patient was a 58-year-old man with 1-year history of cognitive decline, which was diagnosed as Alzheimer's disease in another hospital. He was admitted to our hospital for extreme fatigue, weight loss, and dysphagia, subsequent to the left peripheral facial paresis. Brain magnetic resonance (MR) imaging showed bilateral diffuse white matter lesions and hippocampal atrophy. After admission, he presented with sudden high fever, recurrent exacerbations of consciousness, and increased C-reactive protein level with marked neutrophilia, with the result that he underwent mechanical ventilation. Routine cerebrospinal fluid findings at the exacerbation were normal i.e. 4.7 cells/mm(3), 40 mg/dl of protein, but IL-6 concentration was mildly elevated to 22.2 pg/ml. After confirming the positivity of HLA (human leukocyte antigen) B54 and Cw1, we administered steroid to him and his physical activity and state of consciousness significantly improved. During the course of treatment, dermal lesion characterisitic of Sweet disease was absent. We diagnosed this case was possible neuroSweet disease proposed by Hisanaga in 2005.

[Long-term prognosis of analgesics abuse headache].

OBJECTIVE: To evaluate the long-term prognosis of analgesics abuse headache. METHODS: Ninety-five consecutive patients with analgesics abuse headache were treated in Toyonaka Municipal Hospital. Seventy-three patients (76.8%) had migraine, eighteen (19.0%) had tension-type headache and four (4.2%) new daily persistent headache. Seventy-seven (81.1%) were females and eighteen (18.9%) males. All patients were treated for six years from November 1997 to October 2003 and a total of sixty-nine patients were available for interview as of October 2004 at a mean time interval of 41.5 months after drug withdrawal therapy. Twenty-two patients were admitted to our inpatient withdrawal unit, twenty-five patients were treated by outpatient withdrawal therapy. Tapering analgesics gradually in conjunction with instituting preventive therapy treated twenty-two patients. RESULTS: Inpatient-Nine cases (41%) reported intake of analgesics on < or = 8 days/month, five cases (23%) on 9-15 days/month and eight cases (36%) > 15 days/month. Three cases (14%) developed recurrent analgesic abuse. Outpatient (abrupt discontinuation)--Twelve cases (48%) reported intake of analgesics on < or = 8 days/month, five cases (20%) on 9-15 days/month and eight cases (32%) >15 days/month. One case (4%) reported on recurrent analgesic abuse. Outpatient (tapering analgesics gradually)--One case (5%) reported intake of analgesics on < or = 8 days/month and twenty-one cases (95%) reported daily intake. Fifteen cases (68%) reported continuous analgesic abuse. Comparison between migraine and tension-type headache suggested that patients with migraine showed a tendency towards a better prognosis than patients with tension-type headache. CONCLUSIONS: These results demonstrate the efficacy of withdrawal treatment in difficult cases suffering from analgesics abuse headache. If patients cannot be safely or adequately treated as outpatients, inpatient treatment may be needed.

[Primary new daily persistent headache (NDPH): clinical characteristics of forty-three cases in Japan].

Although the primary chronic daily headache is subdivided into chronic migraine, chronic tension-type headache, hemicrania continua, and new daily persistent headache (NDPH), the characteristics of NDPH have not been well studied in Japan. The purpose of this study is to investigate the clinical characteristics of the primary NDPH. Twenty-one men and twenty-two women with primary NDPH, aged 14 to 73 years were diagnosed at Toyonaka Municipal Hospital from November 1997 to April 2003. Age of the onset ranged from 13 to 73 years (mean, 34.8). The onset of headache occurred in relation to a stressful life event in 16%, a systemic infection in 12% and an extracranial surgery in 7%. Any precipitating events could not be identified in approximately two-third of patients (65%). Quality of the headache was described as dull and/or pressure-like sensation in 91%, throbbing and/or pulsating sensation in 28%. NDPH needs further study, because it appears to be one of the most refractory headaches.

[A case of CIDP with diabetes mellitus who had good response to intravenous immune globulin].

A 64 year-old man began to feel numbness on his bilateral feet in 1990. He was diagnosed as diabetes mellitus with a high fasting glucose level of 580 mg/dl in 1993 and he received oral hypoglycemic agents. Since then, his blood glucose levels had been in good control under diet therapy and medication. However, his numbness worsened and progressive weakness of bilateral lower legs occurred in 1997. Bilateral anterior tibial muscles were atrophic and deep tendon reflexes were decreased on bilateral upper and lower limbs. Protein level of his cerebrospinal fluid was 63 mg/dl. Nerve conduction study fulfilled the electrophysiological diagnostic criteria of CIDP. Superficial peroneal nerve biopsy showed loss of myelinated fibers, small amount of onion bulbs and thickening of the basement membrane of arterioles. Demyelination was predominant in teased fiber study. These findings were compatible with CIDP combined with diabetes mellitus (DM-CIDP). His numbness and leg weakness improved after intravenous high dose immune globulin therapy. DM-CIDP must be distinguished from diabetic peripheral polyneuropathy because immunological therapy may be effective in DM-CIDP patients.

[A case of bilateral perisylvian polymicrogyria with epileptic attacks of focal inhibitory seizure followed by complex partial seizure].

A 20 year-old woman began to have epileptic attacks of focal inhibitory seizure with paralysis and hypesthesia of her left or right upper limb followed by complex partial seizure several times a week since age 19. She was born by breech presentation and umbilical cord was coiling around her neck at birth. EEG showed spike foci on P 3, O1 and T5. Cerebral MRI with 4 mm-section inversion recovery image revealed bilaterally symmetrical polymicrogyria involving the posterior portion of sylvian fissure extending posteriorly to the inner cortex of the postcentral gyrus and the supramarginal gyrus, and she was diagnosed as bilateral perisylvian polymicrogyria. 99mTc-ECD SPECT showed increased cerebral blood flow over the bilateral polymicrogyric lesion. On cerebral MRA, bilateral middle cerebral arteries were narrow all way through. Epileptic attacks were controlled with zonisamide and carbamazepin. This is a rare case of bilateral perisylvian polymicrogyria because epileptic attacks were the only manifestation and the patient showed neither mental retardation nor neurological abnormality.