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Purpose of Review: The migraine postdrome is the least studied and least understood phase of migraine. This article covers the salient features of the migraine postdrome and provides insight into the history, clinical symptoms, and future implications of this phase of migraine., Recent Findings: Prospective electronic diary studies have shown that patients are left disabled with various nonheadache symptoms in the migraine postdrome, and 81% of patients report at least one nonheadache symptom in the postdrome. Hence, it is important to understand this phase better and ensure that more effective treatments become available in the future to lessen the morbidity associated with this phase. Functional imaging shows widespread reduction in brain-blood flow in the postdrome, which explains the multitudes of symptoms experienced by patients., Summary: The disability related to migraine is not exclusive to the headache phase but extends into the postdrome phase and is associated with several nonheadache symptoms that prolong the symptoms experienced by patients with migraine. Further research into the postdrome is crucial to improve our overall understanding of migraine mechanisms. This knowledge may also help to treat the concurrent nonheadache symptoms better in the future. Novel neuroimaging techniques provide a valuable noninvasive tool to push the frontiers in the understanding of migraine pathophysiology. These methods may help shed further light onto the possible links between key brain structures and networks that could be implicated in the pathophysiology of the various migraine phases.

Introduction: Central nervous system infections caused by Listeria monocytogenes usually manifest in the form of meningitis or meningoencephalitis, and are more common among immunosuppressed patients. Brainstem encephalitis (rhombencephalitis) is less common and fatal if not recognized and treated early., Case Report: We describe the case of a 40-year-old, immunocompetent male patient, who presented with initial symptoms of high fever and productive cough. Signs of brainstem involvement appeared later. A magnetic resonance imaging of the brain revealed a lesion of inflammatory appearance in the right medulla oblongata, and the cerebrospinal fluid test showed mononuclear pleocytosis. Blood and cerebrospinal fluid cultures were negative. He presented with a significant improvement with the start of ceftriaxone and subsequent association of corticosteroids, until he developed respiratory failure and died. The third blood cultures grew after his death and they were positive for L. monocytogenes. An autopsy was carried out, which showed necrotizing inflammation, with gram-positive bacilli in the brainstem and the cerebellum., Conclusions: A fatal delay in the diagnosis occurred, mainly because of the favorable clinical response to ceftriaxone and corticosteroids. This case reminds us that a febrile clinical presentation with brainstem involvement must generate the suspicion of a Listeria infection, and therefore ampicillin must be a part of the empirical treatment.

We describe a case of an 87-year-old woman with rheumatoid arthritis on etanercept who developed subacute encephalopathy. Magnetic resonance imaging and blood cultures led to the diagnosis of Listeria monocytogenes rhomboencephalitis, which proved to be fatal. Postmortem examination of the brain revealed abscesses with extensive necrosis. Gram stain showed small gram-positive rods in the necrotic tissue and the wall lining the abscesses. While rhomboencephalitis is a rare entity, and clinical recognition may be hampered by immunosuppression such as in this case, early blood cultures, lumbar puncture, and empiric treatment with ampicillin should be initiated in all patients with suspected rhomboencephalitis if suggested by imaging and by a clinical concern for infection.

Objective: Cardiovascular comorbidities associate with neurodegeneration in the elderly and may contribute to extranigral pathologies and medically refractory axial motor features in Parkinson disease (PD)., Methods: We explored differences in the estimated rate of axial motor feature accrual between patients with PD with and without elevated cardiovascular risk factors as estimated by the Framingham General Cardiovascular Disease risk-scoring algorithm in a cross-sectional cohort study. All participants underwent motor evaluations with the Movement Disorders Society revised Unified Parkinson's Disease Rating Scale (MDS-UPDRS), [(11)C]dihydrotetrabenazine (DTBZ) monoaminergic brain PET imaging, and MRI., Results: Participants with PD with elevated Framingham risk (FR) scores (n = 63, 74.1%) showed higher unadjusted rates of total MDS-UPDRS (t = 3.60, p = 0.0006) and axial motor scores (t = 3.98, p = 0.0001) per estimated year of motor symptoms compared to participants with normal-range risk scores (n = 22, 25.9%). After controlling for sex, Montreal Cognitive Assessment score, frontal leukoaraiosis severity, and striatal DTBZ activity, elevated risk factor status was associated with the rate of accrual of axial motor impairments (R(2) = 0.206; t = 2.62, p = 0.011) but not with total MDS-UPDRS motor score (R(2) = 0.198; t = 1.51, p = 0.135). Frontal leukoaraiosis was associated with the rate of axial and total MDS-UPDRS scores per year of symptoms and also with elevated systolic blood pressure (R(2) = 0.291; t = 2.30, p = 0.024) in a separate risk-factor model., Conclusion: Cardiovascular risk factors may contribute to axial motor features in PD. Early modification of cardiovascular risk factors, including hypertension, deserves further study as a novel disease-modifying strategy in PD.

The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of etiologies, some of them potentially severe and life threatening without proper early treatment. In this retrospective observational study, we reviewed the records of consecutively hospitalized patients at University Hospital of Bellvitge (Barcelona, Spain) from January 1990 to December 2008. Rhombencephalitis was defined as a brainstem and/or cerebellar condition demonstrated clinically or by neuroimaging, with pleocytosis (>4 cells/mm) in cerebrospinal fluid. Ninety-seven patients (48 female; mean age, 37 yr; range, 14-79 yr) fulfilled these criteria. We reviewed their clinical, cerebrospinal fluid, and radiologic characteristics. The mean follow-up was 5 years (range, 0-20 yr). The etiologies of rhombencephalitis were as follows: unknown cause (n = 31), multiple sclerosis (n = 28), Behçet disease (n = 10), Listeria monocytogenes infection (n = 9), paraneoplastic syndrome (n = 6) (3 cases associated with anti-Yo antibodies and 3 with anti-Tr antibodies), Epstein-Barr virus (n = 4), tuberculosis (n = 2), pneumococcal infection (n = 2), systemic lupus erythematosus (n = 1), lymphoma (n = 1), Brucella species infection (n = 1), JC virus (n = 1), and relapsing polychondritis (n = 1). Certain clinical, cerebrospinal fluid, and radiologic characteristics that are commonly seen in some of these etiologies can guide us in the first approach to the etiologic diagnosis of rhombencephalitis.

The cardiac neural crest is a subpopulation of neural crest cells (NCCs) that originates from the dorsal neural tube and later migrates to the heart and pharyngeal arch (PA), where they contribute to distinct aspects of cardiogenesis and PA patterning. Wnt family members have been implicated in general neural crest induction; however, the molecular mechanisms underlying the specification of distinct cell types such as cardiac NCCs remain unclear. Here, we have identified cysteine-rich intestinal protein 2 (crip2), a gene encoding for a LIM domain-containing protein, as a novel marker for cardiac NCCs in zebrafish. We observed a dynamic expression pattern for crip2, with expression being detected in the premigratory NCCs in rhombomere 6 (r6), migrating NCCs, ventricular cardiomyocytes, and aortic vessels in PAs 3 to 6. Interestingly, expression of wnt3a was detected in regions of the dorsal neural tube encompassing r6, and reduction of wnt3a led to disrupted expression of crip2 in r6, abnormal migration of NCCs, and defective cardiac function, as well as PA development. Furthermore, these phenotypes could be recapitulated by reduction of crip2 and rescued by overexpression of crip2. In summary, our data suggest a specific role for Wnt3a in the development of cardiac NCCs. We propose that this function of wnt3a in r6 is partially mediated by crip2 expression in the premigratory cardiac NCCs, which subsequently affects cardiac function and PA patterning.

Recent neuropathologic studies suggest that Parkinson disease (PD) does not begin in the substantia nigra compacta (SNc) but only involves this region later in the course of the disease. It is proposed that once the SNc is affected by the original pathobiological process (for example, protein aggregation), additional processes more specific to dopaminergic neurons are triggered (including sources of oxidative stress such as increased dopamine turnover, reduced levels of reduced glutathione, increased iron, and the presence of neuromelanin, as well as altered calcium homeostasis and excitotoxicity). This results in an acceleration of cell loss in the SNc, causing nigrostriatal degeneration to both reach a threshold for symptoms in advance of earlier affected brain areas and progress more rapidly than other aspects of the disease. Neuroprotective therapy directed solely at more general biologic processes may not have sufficient effects on this accelerated degeneration in the SNc, while neuroprotective therapy designed exclusively to slow the progression of dopaminergic cell loss will not alter the progression of the nondopaminergic symptoms that contribute the greatest disability in the later stages of the disease. Effective disease-modifying therapy may require a cocktail combining treatments designed to address the basic mechanisms of the neurodegeneration and the additional biologic processes specific to the dopaminergic SNc. This hypothesis has implications for the development of disease-modifying therapy and the interpretation of endpoints of clinical trials evaluating the efficacy of such treatments.

Rhombencephalosynapsis is a rare cerebellar malformation that can be associated with anomalies of the cerebral hemispheres and variable degrees of neurodevelopmental delay. A syndromic association, comprising rhombencephalosynapsis, developmental delay, scalp alopecia and trigeminal anaesthesia (Gomez-López-Hernández syndrome) has been described in seven individuals. We report the case of a 2-year-old boy with rhombencephalosynapsis, and review the evidence for a possible diagnosis of Gomez-López-Hernández syndrome. We also discuss other malformations reported in combination with rhombencephalosynapsis, and consider the possibility that a genetic aetiology for syndromic and nonsyndromic forms of rhombencephalosynapsis may be established with more detailed clinical and genetic studies.

Study Design: Magnetic resonance imaging (MRI) was used to compare quantitatively the position of the cerebellar tonsil in neurologically normal adolescents with that in idiopathic scoliosis (AIS) patients and age-matched healthy controls., Objectives: To redefine the normal reference level of the cerebellar tonsil in healthy adolescents and to compare the differences in tonsillar level with AIS of different severities., Summary of Background Data: Asymptomatic Chiari I malformation has been reported not infrequently in children and adolescents with AIS. The definition of tonsillar herniation and its variations with sex, age, and curve severities have not been clarified., Methods: Sagittal MRI of hindbrain was performed on 170 adolescents, including 117 patients with AIS and 53 age-matched healthy controls., Results: The cerebellar tonsillar tip in healthy patients was found at an average of 2.8 mm above the basion-opisthion reference line (BO line) (range, 0-10.5 mm), in contrast to the finding that 17.9% of the AIS patients had tonsillar tip below the BO line. The position of tonsil in the AIS group was lower than that in the healthy controls (P < 0.05), and the differences also increased with the severity of the curve (P = 0.027)., Conclusions: Using the current MRI reference standards, the incidence of tonsillar herniation could be significantly underestimated. According to our experience, any inferior displacement of a tonsil below the BO line in adolescents should be regarded as abnormal. Scoliosis could be an important manifestation of subclinical tonsillar herniation.

Rhombencephalosynapsis is an unusual disorder characterized predominantly by agenesis/hypogenesis of the cerebellar vermis and fusion of the cerebellar hemispheres. Three cases are reported with emphasis on the MRI and clinical findings. Discussion of the relative importance of the cerebellar anomalies and associated supratentorial abnormalities is included.