Neurological symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review.

BACKGROUND: Isolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH). CASE PRESENTATION: A 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly. CONCLUSION: Isolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.

Abscess formation within a cerebellar metastasis: Case report and literature review.

INTRODUCTION: The managements of brain abscesses and brain tumors including brain metastases differ greatly, especially in terms of surgical procedures. Therefore, differentiating between the two is essential for assuring optimal treatment. However, on rare occasion, these two lesions coexist. Only five cases with both a brain tumor and an abscess have thus, far been reported. We experienced a patient with a brain abscess within a cerebellar metastasis. CASE PRESENTATION: A middle-aged man receiving treatment for bile duct cancer presented with headache and nausea. Computed tomography (CT) and magnetic resonance (MR) imaging revealed two lesions, one in each cerebellar hemisphere. Although these lesions appeared to be brain metastases, the right lesion showed high intensity on diffusion-weighted images (DWI), and magnetic resonance spectroscopy (MRS) findings suggested an abscess. Surgical puncture of the lesion identified it as a brain abscess histologically, and we thus, administered antibiotics. However, since the lesion grew, we performed a second surgery for removal, which histopathologically the lesion to be a well-differentiated adenocarcinoma. The final diagnosis was an abscess within a cerebellar metastasis. DISCUSSION/CONCLUSION: Modern diagnostic tools such as DWI and MRS are reportedly reliable for differentiating brain tumors from brain abscesses, though they are not specific in cases with both lesions. The present case highlights the importance of considering coexisting diseases prior to surgery when we encounter a lesion which has the imaging characteristics of both a tumor and an abscess. The patient may have a better outcome if, preoperatively, surgeons take into consideration the possibility of the coexistence of a brain tumor and a brain abscess.

Ruptured intracranial aneurysm during pregnancy with false-negative computed tomography angiography findings: a case report.

A 34-year-old female was admitted at 34 weeks of gestation with sudden onset of a severe headache accompanied by vomiting. Neurological examination revealed neck rigidity, and computed tomography (CT) of the brain revealed a subarachnoid hemorrhage (SAH). Although the hemorrhage was located primarily in the left Sylvian fissure, computed tomography angiography (CTA) performed immediately after CT did not reveal any obvious vascular abnormalities such as an intracranial aneurysm. An emergency cesarean section was performed, and a healthy infant was delivered. Cerebral digital subtraction angiography (DSA) was performed the day following surgery, which revealed a saccular aneurysm measuring 4.3 mm × 2.4 mm in the left middle cerebral artery. Left craniotomy and clipping of the aneurysm were performed. The clot around the aneurysm was relatively solid. This case report is of significance given that initial CTA was negative for SAH during pregnancy, suggesting the requirement for immediate DSA or another CTA in such cases. There are many previous reports on false-negative CTA findings or disappearance and reappearance of aneurysms in SAH patients, and various biophysical and dynamic parameters are suggested to cause such phenomena. However, there are no reports on similar occurrences during pregnancy. Although the precise cause remains unclear, multiple factors associated with homeostasis during pregnancy were possibly associated with the transient disappearance in this patient.

Brain core temperature of patients with mild traumatic brain injury as assessed by DWI-thermometry.

INTRODUCTION: The aim of this study was to assess the brain core temperature of patients with mild traumatic brain injury (mTBI) using a noninvasive temperature measurement technique based on the diffusion coefficient of the cerebrospinal fluid. METHODS: This retrospective study used the data collected from April 2008 to June 2011. The patient group comprised 20 patients with a Glasgow Coma Scale score of 14 or 15 who underwent magnetic resonance imaging within 30 days after head trauma. The normal control group comprised 14 subjects who volunteered for a brain checkup (known in Japan as "brain dock"). We compared lateral ventricular (LV) temperature between patient and control groups. Follow-up studies were performed for four patients. RESULTS: LV temperature measurements were successfully performed for both patients and controls. Mean (±standard deviation) measured LV temperature was 36.9 ± 1.5 °C in patients, 38.7 ± 1.8 °C in follow-ups, and 37.9 ± 1.2 °C in controls, showing a significant difference between patients and controls (P = 0.017). However, no significant difference was evident between patients and follow-ups (P = 0.595) or between follow-ups and controls (P = 0.465). CONCLUSIONS: A reduction in brain core temperature was observed in patients with mTBI, possibly due to a global decrease in metabolism.

Strategies to prevent positioning-related complications associated with the lateral suboccipital approach.

The lateral positioning used for the lateral suboccipital surgical approach is associated with various pathophysiologic complications. Strategies to avoid complications including an excessive load on the cervical vertebra and countermeasures against pressure ulcer development are needed. We retrospectively investigated positioning-related complications in 71 patients with cerebellopontine angle lesions undergoing surgery in our department between January 2003 and December 2010 using the lateral suboccipital approach. One patient postoperatively developed rhabdomyolysis, and another presented with transient peroneal nerve palsy on the unaffected side. Stage I and II pressure ulcers were noted in 22 and 12 patients, respectively, although neither stage III nor more severe pressure ulcers occurred. No patients experienced cervical vertebra and spinal cord impairments, brachial plexus palsy, or ulnar nerve palsy associated with rotation and flexion of the neck. Strategies to prevent positioning-related complications, associated with lateral positioning for the lateral suboccipital surgical approach, include the following: atraumatic fixation of the neck focusing on jugular venous perfusion and airway pressure, trunk rotation, and sufficient relief of weightbearing and protection of nerves including the peripheral nerves of all four extremities.

Gamma Knife surgery for patients with brainstem metastases.

OBJECT: Because brainstem metastases are not deemed resectable, stereotactic radiosurgery (SRS) is the only treatment modality expected to achieve a radical cure. The authors describe their treatment results, focusing particularly on how long patients can survive without neurological deterioration following SRS for brainstem metastases. METHODS: This was an institutional review board-approved, retrospective cohort study in which the authors pulled from their database information on 2553 consecutive patients with brain metastases who underwent Gamma Knife surgery (GKS) at the Mito GammaHouse between July 1998 and July 2011. Among the 2553 patients, excluding cases in which there was meningeal dissemination, 200 cases of brainstem metastases (78 women and 122 men with a mean age of 64 years [range 36-86 years]) were identified and analyzed. The most common primary site was the lung (137 patients) followed by the gastrointestinal tract (24 patients), breast (17 patients), kidney (12 patients), and others (10 patients). Among the 200 patients, 15 patients (7.5%) harbored at least 2 tumors in the brainstem: 11 patients had 2 tumors, 2 patients had 3 tumors, and 1 patient each had 4 or 5 tumors. Therefore, a total of 222 tumors were irradiated. These 222 tumors were located in the pons (121 lesions), the midbrain (65 lesions), and the medulla oblongata (36 lesions). The mean and median tumor volumes were 1.3 and 0.2 cm(3) (range 0.005-10.7 cm(3)), and the median peripheral radiation dose was 18.0 Gy (range 12.0-25.0 Gy). RESULTS: The overall median survival time (MST) was 6.0 months. Distribution of MSTs across Recursive Partitioning Analysis (RPA) classes showed that the MSTs were 9.4 months in Class I (20 patients), 6.0 months in Class II (171 patients), and 1.9 months in Class III (9 patients). Better Karnofsky Performance Scale score, single metastasis, and well-controlled primary tumor were significant predictive factors for longer survival. The neurological and qualitative survival rates were 90.8% and 89.2%, respectively, at 24 months post-GKS. Better KPS score and smaller tumor volume tended to be associated with prolonged qualitative survival. Follow-up imaging studies were available for 129 patients (64.5%). The tumor control rate was 81.8% at 24 months post-GKS. Smaller tumor volume tended to contribute to tumor control. CONCLUSIONS: The present results indicate that GKS is effective in the treatment of brainstem metastases, particularly from the viewpoint of maintaining a good neurological condition in the patient.

A surgical case of cerebral hemorrhage in a patient with factor XI deficiency.

A 63-year-old man suddenly presented with right hemiplegia and was taken to our hospital. Computed tomography (CT) scan revealed subcortical hemorrhage of the left parietal lobe. He had no medical history except hypertension; thus, it initially appeared to be a typical hypertensive hemorrhage. However, blood analysis showed an abnormally elevated activated partial thromboplastin time. One hour after admission, his Glasgow Coma Scale fell from 14 to 11. We performed an echo-guided stereotaxic removal of the hematoma. He improved immediately and was diagnosed with congenital factor XI (FXI) deficiency a few days after surgery. FXI deficiency, described as hemophilic syndrome C, rarely manifests as spontaneous bleeding, but surgical intervention has been known to manifest as bleeding. This case highlights the importance of evaluation of coagulopathies in patients with intracerebral hemorrhage before surgery, and, in cases wherein blood analysis results suggest coexisting coagulation disorders, less invasive surgical methods would likely lead to good outcomes.

[Munchausen syndrome, a factitious injury, presenting brain abscess and intraventricular hemorrhage: a case report].

Munchausen syndrome is a factitious disorder. Patients sometimes inflict injury on themselves in order to assume a sick role. The authors report a patient with Munchausen syndrome suffered from brain abscess, reopened wound and intraventricular hemorrhage. A 64-year-old male was admitted to our hospital after head injury. CT and MR imaging revealed a mass with surrounding edema in the right frontal lobe. The mass was surgically removed, and diagnosed as brain abscess. During the surgery, the authors noticed a small bone defect in the frontal bone above the brain abscess; therefore, we considered that head injury just concerned this lesion. There were no particular clues leading to other possible pathologies. After the first surgery, the patient presented atypical seizures several times. Once we discharged him from our hospital, we hospitalized him again because the wound had reopened. A subsequent operation was needed, and we removed the bone flap which we considered the origin of the infection. After the second surgery, he stabbed a nail into his head where the bone had been removed due to the previous surgery, and presented intraventricular hemorrhage. The hemorrhage decreased in size through non-surgical treatment and he was referred to the psychiatry department under a diagnosis of Munchausen syndrome. Diagnosis of this entity is difficult and often made at the later stage of hospitalization, because patients present a variety of complaints and clinical symptoms, which are hardly proved factitious. Early consideration of this syndrome will offer an early and accurate diagnosis, and is mandatory for a good prognosis.

Moyamoya patients exhibit higher brain temperatures than normal controls.

The balance between heat production (metabolism) and heat removal (blood flow) helps in keeping the temperature of the brain constant. In patients with moyamoya disease, this balance may be disturbed. The purpose of this study was to assess the thermal pathophysiology of the brain in patients with moyamoya disease. The study included 12 consecutive patients with moyamoya disease and 10 controls. Temperature was measured by image postprocessing of diffusion-weighted images. Our noninvasive thermometry showed that the ventricular temperature of moyamoya disease patients was higher than that of normal controls. The mean temperature difference of 1.1 degrees C between the two groups was significant. Patients with moyamoya disease tend to have elevated ventricular temperatures, which may represent a mismatch between cerebral metabolism and perfusion.

Unruptured middle cerebral artery aneurysm associated with a duplicated middle cerebral artery and a dolichoectasic anterior cerebral artery.

Duplicated middle cerebral artery (DMCA) has been reported in 0.7%-2.9% of autopsy cases, and sme reports have described aneurysms in the MCA. Both ruptured and unruptured aneurysms in the MCA have been reported, often requiring surgical intervention. During surgery for aneurysm in DMCA, care must be taken to avoid damaging the MCA, which supplies the territory of the temporopolar artery and the anterior and/or middle temporal artery. Regarding dolichoectasia of intracranial arteries, a relationship between dolichoectasia and ischemic brain lesions, especially lacunar infarctions, has been noted. We report a case with unruptured MCA aneurysm associated with DMCA and a dolichoectasic anterior cerebral artery. In this case, surgical intervention was not prioritized because of the narrowed and calcified parent artery of the aneurysm. In selecting treatment for a patient with multiple vascular anomalies, the pathophysiology of each anomaly should be estimated carefully.

Detection of necrotic neural response in super-acute cerebral ischemia using activity-induced manganese-enhanced (AIM) MRI.

Immediate and certain determination of the treatable area is important for choosing risky treatments such as thrombolysis for brain ischemia, especially in the super-acute phase. Although it has been suggested that the mismatch between regions displaying 'large abnormal perfusion' and 'small abnormal diffusion' indicates a treatable area on an MRI, it has also been reported that the mismatch region is an imperfect approximation of the treatable region named the 'penumbra'. Manganese accumulation reflecting calcium influx into cells was reported previously in a middle cerebral artery occlusion (MCAO) model using activity-induced manganese-enhanced (AIM) MRI. However, in the super-acute phase, there have been no reports about mismatches between areas showing changes to the apparent diffusion coefficient (ADC) and regions that are enhanced in AIM MRI. It is expected that the AIM signal can be enhanced immediately after cerebral ischemia in the necrotic core region due to calcium influx. In this study, a remote embolic rat model, created using titanium-oxide macrospheres, was used to observe necrotic neural responses in the super-acute phase after ischemia. In addition, images were evaluated by comparison between ADC, AIM MRI, and histology. The signal enhancement in AIM MRI was detected at 2 min after the cerebral infarction using a remote embolic method. The enhanced area on the AIM MRI was significantly smaller than that on the ADC map. The tissue degeneration highlighted by histological analysis corresponded more closely to the enhanced area on the AIM MRI than that on the ADC map. Thus, the manganese-enhanced region in brain ischemia might indicate 'necrotic' irreversible tissue that underwent calcium influx.

Usefulness of phase contrast cine mode magnetic resonance imaging for surgical decision making in patients with hydrocephalus combined with achondroplasia. Case report.

A 1-year 9-month-old girl presented with achondroplasia. Serial magnetic resonance (MR) imaging demonstrated mild compression of the medulla oblongata by the occipital bone, macrocrania, and progressive hydrocephalus. Cerebrospinal fluid (CSF) flow study using MR imaging clearly demonstrated CSF flow disturbance at the cervicospinal junction. Foramen magnum decompression was performed for her hydrocephalus and compressed medulla. Postoperative CSF flow study demonstrated improvement of CSF flow at the craniocervical junction. The patient has remained in a stable condition for 7 months postoperatively. Achondroplasia represents hydrocephalus or medullary compression caused by narrowed foramen magnum, which can result in sudden death in some infants. Surgical indications and methods for hydrocephalus combined with achondroplasia remain controversial because the natural history of the hydrocephalus has remained unclear. CSF flow study using MR imaging can provide useful information regarding the surgical indication and methods for the treatment of hydrocephalus combined with achondroplasia.

Surgical strategy of brain tumors adjacent to the optic radiation using diffusion tensor imaging-based tractography.

The present study evaluated the efficacy of diffusion tensor imaging (DTI)-based tractography in the surgery of brain tumors adjacent to the optic radiation. Of the 14 surgical cases included, 11 had metastatic brain tumors and 3 cerebral gliomas. Additionally, 4 of the 14 patients had pre-operative visual field defects, while the remaining 10 patients experienced no visual impairment. The optic radiations on the lesion side were evident in all 14 patients. On the basis of these tractographic findings, we employed optimal surgical approaches in each patient to avoid injury to the eloquent neural structures, including optic radiation, during surgery. Successful surgical resection was performed in all 14 patients. Of the 14 patients, 2 with visual field defects during the pre-operative period showed improvement in their visual field, and the remaining 12 patients experienced no visual deterioration. DTI-based tractography thus is a feasible modality for the surgical planning of brain lesions adjacent to the optic radiation.

[A case of pontine astrocytoma with unusual neuroimaging features].

We would like to report a rare case of pontine glioma with unusual neuroimaging features. The patient was a 3-year-old girl who suffered from chronic nausea and gait disturbance for several months. Computed tomography (CT) demonstrated ventricular dilatation, and ventricular peritoneal (VP) shunt was performed for idiopathic hydrocephalus at another hospital. Fever of unknown origin continued for a month after the VP shunt. At our hospital, cerebrospinal fluid examination showed bacterial meningitis, and it was assumed that shunt infection lead to shunt failure. Magnetic resonance imaging (MRI) revealed hydrocephalus and pontine swelling, and serial MRI suggested brainstem tumor extending to the bilateral thalamus. The patient underwent stereotactic biopsy of the left thalamic tumor, under general anesthesia, and the histological diagnosis was anaplastic astrocytoma. Diffuse pontine glioma rarely increases without cranial nerve deficits. In the present case, pontine glioma extended to the bilateral thalamus symmetrically. It was difficult to diagnose the presented lesion as pontine glioma in the early period because of its unusual neuroimaging.

Usefulness and limitations of fluorine-18-fluorodeoxyglucose positron emission tomography for the detection of malignancy of orbital tumors.

Fluorine-18-fluorodeoxyglucose positron emission tomography ([18F]FDG PET) was assessed as a method for providing information about the malignancy of orbital tumors. Twelve patients with 13 orbital tumors underwent [18F]FDG PET followed by biopsy or tumor removal via a transcranial approach. The accumulation ratio between the tumor and the contralateral normal tissue (T/N ratio) was calculated for 10 of the 13 lesions. The T/N ratio in benign lesions was compared with that in malignant tumors. Histological examination identified 7 lesions as malignant: anaplastic astrocytoma of the optic nerve in 1 patient, which recurred as glioblastoma of the optic nerve, malignant lymphoma of mucosa-associated lymphoid tissue type in 1 patient, malignant melanoma in 1 patient, adenoid cystic carcinoma in 2 patients, and adenocarcinoma (unknown origin) in 1 patient. The T/N ratio was 1.06 +/- 0.03 (mean +/- standard deviation) in benign tumors, and significantly higher at 1.81 +/- 0.27 in malignant tumors (p = 0.0027). Both patterns of high and iso uptake of [18F]FDG were found in orbital pseudotumor. [18F]FDG PET can determine the malignancy of orbital tumors, but cannot distinguish malignant tumor from inflammatory disease such as pseudotumor.

Transanal prolapse of a ventriculoperitoneal shunt catheter--case report.

A 4-year-old girl presented with asymptomatic bowel perforation and transanal protrusion of a ventriculoperitoneal (VP) shunt catheter. She had undergone repair of myelomeningocele at birth and subsequent VP shunting for congenital hydrocephalus 1 month later. Seven months after VP shunting, she underwent revision of the peritoneal catheter. She complained of abdominal pain and nausea at the age of 4 years. She was treated conservatively for 1 month for intestinal obstruction. One month later, her mother noticed the shunt catheter protruding from her anus. Computed tomography (CT) of the abdomen revealed that the peritoneal catheter had migrated into the colon, and CT of the head showed symmetrical dilation of the ventricles. The shunt system was removed immediately, repair of the fistula in the sigmoid colon was performed, and external ventricular drainage was continued for 6 weeks until shunt replacement. One month after the first operation, intestinal obstruction recurred. Duplication of the terminal ileum was removed to prevent further recurrence of the intestinal obstruction. She underwent ventriculoatrial shunting 2 weeks after the second operation and was discharged without neurological sequelae.

Effect of vascular stenosis on perfusion-weighted imaging; differences between calculation algorithms.

PURPOSE: To determine the most suitable postprocessing technique for magnetic resonance (MR) perfusion imaging in patients with vascular stenosis, by comparing the cerebral blood flow (CBF) maps of single photon emission tomography (SPECT) and perfusion MR imaging (MRI). MATERIALS AND METHODS: In 15 consecutive patients (14 men and one woman, mean age 73.9 +/- 6.0 years) with stenosis of common carotid artery (CCA) or internal carotid artery (ICA) of more than 75%, both brain perfusion MRI and brain perfusion SPECT were performed. From perfusion MR images, CBF maps were calculated with the first moment, singular value decomposition (SVD), and block circulant SVD (b-SVD) methods, and CBF maps from each algorithm were compared with those from SPECT. RESULTS: The b-SVD method had the best correlation with SPECT (R = 0.814), followed by the first moment method (R = 0.776) and the SVD method (R = 0.723). The b-SVD method has the least mean difference with SPECT (0.118), the first moment method also had less difference (0.121), and the SVD had greatest mean difference (0.164). CONCLUSION: Our results suggest that in patients with vascular impairment the b-SVD method will be the technique of choice rather than SVD or first moment method.

Cerebral oxygen metabolism in idiopathic-normal pressure hydrocephalus.

OBJECTIVE: To distinguish idiopathic-normal pressure hydrocephalus (i-NPH) from the elder with brain atrophy is difficult. This investigation was undertaken to determine the cerebral oxygen metabolism and the cerebral blood flow using positron emission tomography (PET) in patients with i-NPH. Comparison of the variables between i-NPH patients and the age-comparable control with asymptomatic ventricular dilatation were performed. METHODS: Nineteen patients were studied. Nine i-NPH patients with a mean age of 74.8 +/- 1.8 years (mean +/- SD) were examined using PET. The subjects who underwent a ventriculoperitoneal shunt (VPS) had the triad of NPH and ventricular dilatation on computed tomography (CT) and/or magnetic resonance imaging (MRI). The results of the PET study were compared with those for ten age-comparable controls (74.8 +/- 5.5 years) with asymptomatic ventricular dilatation and no severe cerebrovascular disease on MRI and magnetic resonance angiography (MRA). The PET study included analyses of regional cerebral blood flow (rCBF), regional cerebral blood volume (rCBV), regional oxygen extraction fraction (rOEF) and regional cerebral metabolic rate of oxygen (rCMRO(2)). RESULTS: In i-NPH, rCBF tended to decrease in the frontal lobe and the basal ganglia. rCMRO(2) in the frontal lobe of i-NPH was significantly higher than that in the controls (p<0.05 by Student's t-test), although rCMRO(2) in the basal ganglia of i-NPH was reduced. rCBV and rOEF showed no significant differences. CONCLUSION: Reduction of oxygen metabolism in the basal ganglia might be one of the factors causing symptoms in i-NPH. Particular pattern of cerebral oxygen metabolism in i-NPH was not obvious in the present study.

[Primary intracerebral hemorrhage due to probable cerebral amyloid angiopathy complicated by brain abscess: case report].

Cerebral amyloid angiopathy (CAA), in which deposition of amyloid within the arterial media and adventitia is the remarkable feature, causes spontaneous lober cerebral hemorrhage in elderly person, and some reports show the quite high occurrence rate of this entity among intracerebral hemorrhage in patents above 70 years old. Brain abscess resulting from intracerebral hemorrhage is rare. To our knowledge, no report of such hemorrhage which is caused by CAA has been published so far. We report a case of brain abscess, from which Stenotrophomonas maltophilia was isolated, following spontaneous non-hypertensive intracerebral hemorrhage caused by probable CAA, with a review of the relevant literature.