Presenting Internuclear Ophthalmoplegia with Peripheral Type Facial Palsy: Seven-and-a-Half Syndrome

A 49-year-old male presented with horizontal binocular diplopia without facial pain or skin lesion. Limitation of medial gaze in the left eye was revealed on neurological examination, which is accompanied by peripheral facial nerve palsy ipsilaterally. The diagnosis had been made based on the diffusion restriction lesion of left pontine tegmentum. We may denominate a “seven-and-a-half syndrome” and clinician should maintain a high level of awareness of the various syndromes associated with pontine lesions.

Brain-wide Mapping of Mono-synaptic Afferents to Different Cell Types in the Laterodorsal Tegmentum / 神经科学通报·英文版

The laterodorsal tegmentum (LDT) is a brain structure involved in distinct behaviors including arousal, reward, and innate fear. How environmental stimuli and top-down control from high-order sensory and limbic cortical areas converge and coordinate in this region to modulate diverse behavioral outputs remains unclear. Using a modified rabies virus, we applied monosynaptic retrograde tracing to the whole brain to examine the LDT cell type specific upstream nuclei. The LDT received very strong midbrain and hindbrain afferents and moderate cortical and hypothalamic innervation but weak connections to the thalamus. The main projection neurons from cortical areas were restricted to the limbic lobe, including the ventral orbital cortex (VO), prelimbic, and cingulate cortices. Although different cell populations received qualitatively similar inputs, primarily via afferents from the periaqueductal gray area, superior colliculus, and the LDT itself, parvalbumin-positive (PV) GABAergic cells received preferential projections from local LDT neurons. With regard to the different subtypes of GABAergic cells, a considerable number of nuclei, including those of the ventral tegmental area, central amygdaloid nucleus, and VO, made significantly greater inputs to somatostatin-positive cells than to PV cells. Diverse inputs to the LDT on a system-wide level were revealed.

Endoscopic Treatment of an Adult with Tegmental Astrocytoma Accompanied by Cerebrospinal Fluid Dissemination

Midbrain gliomas are relatively rare neoplasms with a generally benign prognosis, with dissemination or metastasis not previously reported. We describe here a woman, in whom magnetic resonance imaging scans showed hydrocephalus and a tegmental lesion in the upper aqueduct. Endoscopic third ventriculostomy and biopsy were performed; during surgery, a second small lesion was observed in the infundibular recess. Histologically, the two lesions had the characteristics of low grade astrocytoma, suggesting that the midbrain astrocytoma may have been disseminated via the cerebral spinal fluid to the infundibular recess. Postoperatively this patient received radiotherapy for nearly one month. Although patients with these tumors are not usually administered adjunctive therapy, radiation and, combined modality therapy, including surgery, radiotherapy, and chemotherapy, may be beneficial in patients with midbrain gliomas with dissemination.

Wilson?s disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment / Doença de Wilson apresentando-se com transtorno comportamental do sono REM: uma janela possível para instituição de tratamento precoce

Objective To describe characteristics of REM sleep behavior disorder in Wilson’s disease. Method Questionnaire-based interviews (patients and relatives), neurological examinations, two-week prospective dream-diary, video-polysomnography, transcranial sonography, MRI. Results Four Wilson’s disease cases with REM sleep behavior disorder were described; three had REM sleep behavior disorder as initial symptom. All showed mesencephalic tegmental/tectal sonographic hyperechogenicities and two presented ponto-mesencephalic tegmental MRI hyperintensities. Conclusion This first description of REM sleep behavior disorder in Wilson’s disease in literature documents REM sleep behavior disorder as a possible presenting symptom of Wilson’s disease and adds further evidence to the parallelism of Parkinson’s disease and Wilson’s disease in phenotype and brainstem topography, which ought to be further studied. REM sleep behavior disorder has prognostic relevance for neurodegeneration in α-synucleinopathies. In Wilson’s disease, usefulness of early diagnosis and treatment are already well established. REM sleep behavior disorder in Wilson’s disease offers a possible theoretical model for potential early treatment in this extrapyramidal and brainstem paradigm syndrome, previewing the possibility of neuroprotective treatment for REM sleep behavior disorder in “pre-clinical” Parkinson’s disease. .

Objetivo Descrever características do transtorno comportamental do sono REM (TCSR) na doença de Wilson (DW). Método Aplicação de entrevistas, vídeo-polissonografia, sonografia transcraniana (STC), ressonância magnética (RM), diário de sonhos. Resultados Descrevemos quatro casos de DW com TCSR. Três apresentaram o TCSR como primeira manifestação. Todos mostraram hiperecogenicidades mesencefálicas na STC, dois apresentaram hiperintensidades ponto-mesencefálicas na RM. Conclusão Esta é a primeira descrição do TCSR na DW. Relatamos o TCSR como um sintoma inicial da DW. Acrescentamos prova para o paralelismo entre a doença de Parkinson e DW, com relação aos fenótipos e localização das lesões cerebrais. Nas alfa-sinucleinopatias, o TCSR tem relevância prognóstica quanto à neurodegeneração. Na DW, já conhecemos a importância de diagnóstico e tratamento precoces. O TCSR na DW oferece um modelo para antecipar o tratamento desta síndrome de acometimento dos núcleos basais e tronco, vislumbrando a possibilidade de tratamento neuroprotetor para a fase “pré-clínica” da DP. .

Laterodorsal Tegmentum and Pedunculopontine Tegmental Nucleus Circuits Regulate Renal Functions: Neuroanatomical Evidence in Mice Models / 华中科技大学学报（医学）（英德文版）

Neurons in the laterodorsal tegmentum (LDTg) and pedunculopontine tegmental nucleus (PPTg) play important roles in central autonomic circuits of the kidney.In this study,we used a combination of retrograde tracers pseudorabies virus (PRV)-614 and fluorescence immunohistochemistry to characterize the neuroanatomic substrate of PPTg and LDTg innervating the kidney in the mouse.PRV-614-infected neurons were retrogradely labeled in the rostral and middle parts of LDTg,and themiddle and caudal parts of PPTg after tracer injection in the kidney.PRV-614/TPH double-labeled neurons were mainly localized in the rostral of LDTg,whereas PRV-614/TH neurons were scattered within the three parts of LDTg.PRV-614/TPH and PRV-614/TH neurons were located predominantly in the caudal of PPTg (cPPTg).These data provided direct neuroanatomical foundation for the identification of serotonergic and catecholaminergic projections from the mid-brain tegmentum to the kidney.

The CK1 gene family: expression patterning in zebrafish development

Protein kinase CK1 is a ser/thr protein kinase family which has been identified in the cytosol cell fraction, associated with membranes as well as in the nucleus. Several isoforms of this gene family have been described in various organisms: CK1 , CK1á, CK1δ, CK1å and CK1γ. Over the last decade, several members of this family have been involved in development processes related to wnt and sonic hedgehog signalling pathways. However, there is no detailed temporal information on the CK1 family in embryonic stages, even though orthologous genes have been described in several different vertebrate species. In this study, we describe for the first time the cloning and detailed expression pattern of five CK1 zebrafish genes. Sequence analysis revealed that zebrafish CK1 proteins are highly homologous to other vertebrate orthologues. Zebrafish CK1 genes are expressed throughout development in common and different territories. All the genes studied in development show maternal and zygotic expression with the exception of CK1å. This last gene presents only a zygotic component of expression. In early stages of development CK1 genes are ubiquitously expressed with the exception of CK1å. In later stages the five CK1 genes are expressed in the brain but not in the same way. This observation probably implicates the CK1 family genes in different and also in redundant functions. This is the first time that a detailed comparison of the expression of CK1 family genes is directly assessed in a vertebrate system throughout development.

Automatic Stepping in Patients with Progressive Basilar Artery Infarction

Automatic stepping is an involuntary disorder with cyclic alternating leg movements which causes patients to appear as if walking. We report automatic stepping in patients with progressive basilar artery territory infarcts. These movements consisted of spontaneous knee flexions and ankle dorsiflexions with intermittent hip flexions, and alternating leg movements without provocation. Intervals between the movements were various from 3 to 30 seconds. Serial diffusion- weighted MRI revealed that progressive lesions were mainly located in the pontine tegmental areas.

AFFERENT CONNECTIONS OF THE PONTINE TEGMENTUM IN GUINEA PIGS HRP RETROGRADE TRACING METHOD / 解剖学报

The present study was undertaken to identify some afferent connections of the ventral and dorsal parts of pontine tegmentum in guinea pigs by using horseradish peroxidase retrograde microelectrophoretic technique.After introducing HRP into the ventral part of pontine tegmentum, numerous labeled cells were observed in the mesencephalic central gray and related reticular formation, which is equivalent to the MSR (Mesencephalic Stepping Region) ventral to the surface of the superior colliculus. Labeled cells could also be identified at the following sites: nucleus cuneiformis ventral to the surface of inferior colliculus, nucleus tractus spinalis nervi trigemini, nucleus reticularis gigantocellularis, nucleus medialis vestibulares, nucleus lateralis vestibulares, nucleus loci coerulei and its ventral part, gray matter of the cervical enlargement of spinal cord.Following injection of HRP into the dorsal part of pontine tegmentum, substantial amount of labeled cells were observed in the nucleus reticularis pontis caudalis and nucleus reticularis gigantocellularis Labeled cells gould also be identi- fied in the nucleus medialis vestibulares, nucleus lateralis vestibulares and nucleus cuneatus. Only a few HRP cells appeared around the nucleus tuber in the midbrain and no labeled cells have been seen in the nucleus loci coerulei, as well as the gray matter of cervical spinal cord.

SPINAL PROJECTIONS TO THE PONTINE NUCLEUS AND THE CAUDAL PONTINE VENTROLATERAL TEGMENTUM IN RABBITS / 解剖学报

The cervical, thoracic, or lumbar spinal gray was injected with HRP or WGAHRP unilaterally in 18 adult rabbits and the anterograde labeling was traced in the pontine nucleus and the caudal pontine ventrolateral tegmentum.In the pontine nucleus labeled terminal arborizations were found in its caudal 1/3, distributing in the paramedian nucleus, the dorsal part of peduncular nucleus and the dorsolateral nucleus. In the ventrolateral tegmentum four small cell groups were found labeled which were temporarily denominated as VLPT_(1～4) groups. In all locations labelings were found bilaterally and no apparent somatotopical localization could be identified.The cytoarchitecture of VLPT_(1～4) was studied on Nissl sections and their relationship with Meessen and Olszewski's (M-O's) "k" and "m" groups and with pontobulbar body was discussed. VLPT_2 (corresponding to the ventral part of M-O's "k") and VLPT_3 (corresponding to a part of M-O's "m") join each other to form one group which then merges into the dorsolateral nucleus of the pontine gray. They apparently form a part of the pontobulbar body.