Intravenous Immunoglobulin as a Therapeutic Option for Mycoplasma pneumoniae Encephalitis.

OBJECTIVE: To analyze the outcomes of a cohort of children diagnosed with Mycoplasma pneumoniae encephalitis whose treatment regimens included intravenous immunoglobulin (IVIG). METHODS: A retrospective study was performed at a single center between 2011 and 2016 of children diagnosed with Mycoplasma pneumoniae encephalitis whose acute treatment regimen included IVIG. Details of therapeutic interventions and the clinical course were retrieved from medical records via an institutionally approved protocol. The modified Rankin score was used to quantify outcomes. RESULTS: Four children met inclusion criteria, 3 of whom had prodromal symptoms of infection lasting 5 to 7 days before onset of their neurologic symptoms. One patient presented with neurologic symptoms with no clinical prodrome. The initial treatment regimen included systemic corticosteroids, antibiotics, or both. IVIG was administered for a total dose of 2 g/kg divided over 2 to 4 days to all 4 children. All children showed clinical improvement after IVIG. The 3 children with prodromal symptoms showed immediate and dramatic clinical improvement after IVIG therapy. DISCUSSION: The immediate response to immunomodulatory therapy in the patients with prodrome suggests that the neurologic syndrome may be caused at least in part by an autoimmune process. The child who did not respond to IVIG had no prodrome, and also had normal electroencephalographic (EEG) and brain magnetic resonance imaging (MRI) findings. These cases suggest that early administration of IVIG should be considered in patients suspected of having Mycoplasma encephalitis, particularly in those who have had prodromal symptoms.

An Objective Study of Anatomic Shifts in Intracranial Hypotension Using Four Anatomic Planes.

PURPOSE: Intracranial hypotension (IH) often remains undetected using current MR diagnostic criteria. This project aims to demonstrate that central incisural herniation is highly effective in helping to make this diagnosis. MATERIALS AND METHODS: Magnetic resonance imaging (MRI) was analyzed in 200 normal and 81 clinically known IH patients. MRI reference lines approximating the plane of the incisura, the plane of the diaphragma sella, the plane of the foramen magnum, and the plane of the visual pathway were utilized to measure the position of selected brain structures relative to these reference lines. RESULTS: All IH patients had highly statistically significant (p < 0.0001) measurable evidence of downward central incisural herniation when compared to normal controls. The first of the important observations was a downward shift of the mammillary bodies, which shortened the midsagittal width of the interpeduncular fossa cistern. A concurrent downward shift and deformity of the tuber cinereum accompanied the mammillary body shift. The second essential observation was an abnormal clockwise rotation of the long axis of the visual pathway. A severity grading system is proposed based on the extent of these shifts as well as secondary shifts of the brain stem, splenium, and cerebellar tonsils. CONCLUSION: This study objectively delineates the anatomic shifts of brain structures adjacent to the incisura and foramen magnum. This methodology is sufficient to recognize the features of IH and to stratify the spectrum of IH findings into a functional grading system for quantifying the results of interventional therapy.

Recurrence of Lobar Hemorrhage: A Red Flag for Cerebral Amyloid Angiopathy-related Inflammation?

Background. Recurrent lobar intracerebral hemorrhage is more commonly associated with cerebral amyloid angiopathy and less likely associated with hypertension. Cerebral amyloid angiopathy-related inflammation is a subgroup of cerebral amyloid angiopathy that can present with lobar intracerebral hemorrhage, encephalopathy, and seizures; wherein corticosteroids may facilitate favorable outcome. Whether recurrence of lobar intracerebral hemorrhage in cerebral amyloid angiopathy is related to cerebral amyloid angiopathy-related inflammation is unknown. Case presentation. A 68-year-old woman presented with an acute onset of confusion. She was known to have a history of recurrent lobar intracerebral hemorrhage related to cerebral amyloid angiopathy. Brain imaging revealed previous sequelae of cerebral amyloid angiopathy and a new lobar intracerebral hemorrhage. An empirical diagnosis of cerebral amyloid angiopathy-related inflammation was made given the patent's clinical course of recurrence. Utilizing current evidence of criteria used to diagnose cerebral amyloid angiopathy-related inflammation, corticosteroid therapy was initiated with significant improvement in clinical and imaging characteristics. Discussion. Inflammatory pathways incited as a result of cerebrovascular amyloid deposition play a vital role in pathogenesis of cerebral amyloid angiopathy-related inflammation. We highlight the need to consider corticosteroid therapy in patients presenting with recurrent lobar intracerebral hemorrhage in the setting of cerebral amyloid angiopathy since inflammation may play a role in its pathophysiology. Evidence in the literature is sparse to suggest that cerebral amyloid angiopathy-related inflammation might be the root cause for the lobar intracerebral hemorrhage recurrence in cerebral amyloid angiopathy. Further studies are needed to identify mechanisms of recurrent hemorrhage, its correlations with cerebral amyloid angiopathy-related inflammation, and the potential role of corticosteroid therapy.

Determining Organ Doses from CT with Direct Measurements in Postmortem Subjects: Part 1--Methodology and Validation.

PURPOSE: To develop a methodology that allows direct measurement of organ doses from computed tomographic (CT) examinations of postmortem subjects. MATERIALS AND METHODS: In this institutional review board approved study, the x-ray linear attenuation coefficients of various tissues were calculated from the mean CT numbers of images that were obtained in eight embalmed adult female cadavers and compared with the corresponding linear attenuation coefficients calculated from CT images obtained in eight living patients that were body mass index (BMI)-matched. Dosimetry was performed in three of the cadavers by accessing organs of interest and affixing partially sealed vinyl tubes inside them. Optically stimulated luminescent dosimeters (OSLDs) were inserted into the tubes and positioned within the organs of interest and on the skin. OSLDs were read with an InLight MicroStar (Landauer, Glenwood, Ill) reader, and readings were corrected for energy and scatter response. Fifteen tubes containing dosimeters were used, and imaging was repeated twice in each cadaver, for a total of five standard clinical protocols. Average dosimetry values were used for analysis. RESULTS: Differences in linear attenuation coefficients between living and embalmed cadaveric tissues were within 3% for the tissues investigated. Measured organ doses for a chest-abdomen-pelvis CT protocol were less than 32 mGy for all organs measured. Organs that were completely irradiated during a given examination received similar doses, whereas organs that were partially irradiated displayed a large variation in measured organ dose. CONCLUSION: The anatomic and radiation attenuation characteristics of cadavers are comparable to those of living human tissue. This methodology allows direct measurement of organ doses from clinical CT examinations.

Determining Organ Doses from CT with Direct Measurements in Postmortem Subjects: Part 2--Correlations with Patient-specific Parameters.

PURPOSE: To generate empirical sets of equations that can be used to calculate patient-specific organ doses resulting from a group of computed tomographic (CT) studies by using data from direct dose measurements performed within a human body. MATERIALS AND METHODS: Organ dose measurements were obtained in eight postmortem female subjects. A chest-abdomen-pelvis protocol was used for this study. The relationships among measured organ doses, body mass index, effective diameter (D(eff)), and volume CT dose index (CTDI(vol)) were investigated. Organ dose equations were developed by means of linear regression from organ dose data, with CTDI(vol) and D(eff) as variables, by using Pearson correlation coefficients and P values to determine correlation strength of fit. Measured organ doses were compared with corresponding size-specific dose estimates (SSDEs). RESULTS: The central-section D(eff) presented similar correlations with organ doses to those from D(eff) measured at specific organ locations. The strongest correlations were observed between the central-section D(eff) and CTDI(vol)-normalized organ doses (R(2): 0.478-0.941). The average of measured organ doses for each subject resulted in an average difference of only 5% from SSDE-calculated doses; however, individual organ doses differed from +31% to -61% from the calculated SSDE. CONCLUSION: The organ dose equations developed represent a method for organ dose estimation from direct organ dose measurements that can estimate organ doses more accurately than the calculated SSDE, which provides a less-specific patient dose estimate.

Normal relationship of the cervicomedullary junction with the obex and olivary bodies: a comparison of cadaveric dissection and in vivo diffusion tensor imaging.

PURPOSE: The purpose of our study is to compare cadaver dissections with in vivo diffusion tensor imaging (DTI) to determine the position of the cervicomedullary junction (CMJ) relative to the readily identified anatomic landmarks, namely the obex and olivary bodies (olives), in normal subjects. The information gained from this study would allow further investigation into abnormalities of the CMJ, such as Chiari malformation, without the need for time-intensive tractography studies. METHODS: Six formalin-fixed human cadaver brains were compared with DTI studies in 15 normal controls. Measurements were made from the upper border of the crossing fibers of the pyramidal decussation to both the obex and the inferior margin of the olive. RESULTS: For the cadaver specimens, the average distance from the inferior border of the olive to the upper border of the decussation measured 3.7 mm (±1.2 mm). The average distance from the obex to the upper decussation was 6.7 mm (±2.1 mm). In the DTI subjects, the inferior olive to the upper decussation averaged 3.4 mm (±0.9 mm). The distance from the obex to the decussation averaged 6.4 mm (±1.3 mm). CONCLUSION: The CMJ reliably lies 3.4 mm (±0.9 mm) caudal to the inferior border of the olive and 6.4 mm (±1.3 mm) caudal to the obex. Awareness of this anatomic relationship readily allows recognition of abnormalities of the position of the CMJ with routine imaging.

Vagus nerve stimulation in ischemic stroke: old wine in a new bottle.

Vagus nerve stimulation (VNS) is currently Food and Drug Administration-approved for treatment of both medically refractory partial-onset seizures and severe, recurrent refractory depression, which has failed to respond to medical interventions. Because of its ability to regulate mechanisms well-studied in neuroscience, such as norepinephrine and serotonin release, the vagus nerve may play an important role in regulating cerebral blood flow, edema, inflammation, glutamate excitotoxicity, and neurotrophic processes. There is strong evidence that these same processes are important in stroke pathophysiology. We reviewed the literature for the role of VNS in improving ischemic stroke outcomes by performing a systematic search for publications in Medline (1966-2014) with keywords "VNS AND stroke" in subject headings and key words with no language restrictions. Of the 73 publications retrieved, we identified 7 studies from 3 different research groups that met our final inclusion criteria of research studies addressing the role of VNS in ischemic stroke. Results from these studies suggest that VNS has promising efficacy in reducing stroke volume and attenuating neurological deficits in ischemic stroke models. Given the lack of success in Phase III trials for stroke neuroprotection, it is important to develop new therapies targeting different neuroprotective pathways. Further studies of the possible role of VNS, through normally physiologically active mechanisms, in ischemic stroke therapeutics should be conducted in both animal models and clinical studies. In addition, recent advent of a non-invasive, transcutaneous VNS could provide the potential for easier clinical translation.

Role of inflammatory markers in Takayasu arteritis disease monitoring.

BACKGROUND: Takayasu arteritis (TA) is an idiopathic large-vessel vasculitis that can result in significant morbidity and mortality secondary to progressive stenosis and occlusion. Monitoring disease progression is crucial to preventing relapse, but is often complicated by the lack of clinical symptoms in the setting of active disease. Although acute phase reactants such as ESR and CRP are generally used as an indicator of inflammation and disease activity, mounting evidence suggests that these markers cannot reliably distinguish active from inactive TA. CASE PRESENTATION: We report a 24-year-old Hispanic female with a 5-year history of TA who presented with stroke-like symptoms and evidence of left MCA occlusion on imaging, despite a history of decreasing inflammatory markers. CTA revealed complete occlusion of the left common carotid artery, left subclavian, and left MCA from their origins. It also revealed a striking compensatory circulation supplying the left anterior circulation as well as the left subclavian as a response to progressive stenosis. CONCLUSION: Monitoring ESR and CRP levels alone may not be a reliable method to evaluate disease progression in patients with TA, and should be taken in context with both patient's clinical picture and the imaging. We recommend that serial imaging be performed regularly in the setting of active disease to monitor progression and allow for immediate therapy in response to evidence of disease advancement, with a relaxation of the imaging interval once the disease is presumed inactive.

Isolated middle cerebral artery dissection: a systematic review.

Acute stroke can be missed in the emergency department, particularly in younger patients and in those with more vague symptoms such as headache or dizziness. Cervicocephalic dissections are one group of etiologies for acute stroke in the young. While cervicocephalic dissections are not uncommon in clinical practice, isolated middle cerebral artery dissection (MCAD) has been rarely reported as a cause for stroke. We sought to review the clinical implications and pathophysiology of an isolated MCAD. We searched the medical literature for isolated MCAD in clinical stroke patients using MEDLINE, HighWire, and Google Scholar databases from 1966 to 2013 using the keywords 'middle cerebral artery dissection,' 'intracerebral artery dissection,' and 'middle cerebral artery dissection stroke.' We reviewed cases to learn various characteristics of isolated MCAD. A total of 61 cases (62.3% male, mean age 44.16 ± 19.17 years) were reviewed from 54 publications. Most cases were reported from Asian countries (78.7%). Ischemic strokes were more common than hemorrhagic strokes (68.9%). Digital subtraction angiography was the most common imaging modality used to diagnose isolated MCAD (75.4%). Surgery was the preferred form of therapeutic intervention (39.3%). Males (n = 27/48, p = 0.0008) and those who presented with only ischemic syndromes (n = 22/48, p = 0.0009) had significantly higher rates of favorable outcome. Isolated MCAD is a rare disease that can contribute to the stroke burden of young patients. Further studies are needed to better characterize optimal treatment strategies and define outcomes for this rare condition.

Spontaneous intracranial hypotension: dilemmas in diagnosis.

Intracranial hypotension is not an uncommon diagnosis after lumbar puncture or neurosurgery. However, spontaneous intracranial hypotension (SIH) is a poorly understood entity that can present with a wide variety of symptoms/signs ranging from headache to coma. SIH may result from an occult spinal cerebrospinal fluid (CSF) leak. Alternatively, because a CSF leak is not always found, some posit that SIH is caused by venous hypotension that results in increased CSF absorption. The true incidence of SIH is unknown and the diagnosis is frequently missed given the wide range of presenting symptoms and imaging findings that are mistaken for other diagnoses (ie, subdural hematomas, Chiari malformation). Here, based on a comprehensive literature review, we describe the epidemiology, presentation, diagnostic workup and treatment of SIH.

Extensions of the sphenoid sinus: a new classification.

OBJECTIVE: The transsphenoidal approach has been extended in recent years from tumors of the sellar region to lesions involving other areas bordering the sphenoid sinus including the cavernous sinus, Meckel's cave, middle cranial fossa, planum sphenoidal, suprasellar region, and clivus. The goal of this study was to examine various pneumatized extensions of the sphenoid sinus that may facilitate extended approaches directed through the sinus. METHODS: The sphenoid sinus and its surrounding structures were examined in 18 cadaver heads, and the results were correlated with the findings from 100 computed tomography images of the sinus. The sellar type of the sphenoid sinus in which the pneumatization extended beyond the anterior sellar wall was further classified according to the various extensions of the sinus. RESULTS: The sellar type of the sphenoid sinus was classified into the following 6 basic types based on the direction of pneumatization: sphenoid body, lateral, clival, lesser wing, anterior, and combined. The recesses and prominences, formed by pneumatization of the sinus, act as "windows" opening from the sinus in different areas of the cranial base and may facilitate minimally invasive access to lesions in the corresponding areas. CONCLUSION: The variations in the extensions of pneumatization of the sphenoid sinus may facilitate entry into areas bordering the sphenoid sinus and play a role in the selection of a surgical approach to lesions bordering the sinus.