OBJECTIVE: To identify superior cervical sympathetic ganglion (SCSG) and describe their characteristic MR appearance using 3T-MRI. MATERIALS AND METHODS: In this prospective study, we recruited 53 consecutive patients without history of head and neck irradiation. Using anatomic location based on literature review, both sides of the neck were evaluated to identify SCSGs in consensus. SCSGs were divided into definite (medial to internal carotid artery [ICA] and lateral to longus capitis muscle [LCM]) and probable SCSGs based on relative location to ICA and LCM. Two readers evaluated signal characteristics including intraganglionic hypointensity of all SCSGs and relative location of probable SCSGs. Interrater and intrarater agreements were quantified using unweighted kappa. RESULTS: Ninety-one neck sites in 53 patients were evaluated after exclusion of 15 neck sites with pathology. Definite SCSGs were identified at 66 (73%) sites, and probable SCSGs were found in 25 (27%). Probable SCSGs were located anterior to LCM in 16 (18%), lateral to ICA in 6 (7%), and posterior to ICA in 3 (3%). Intraganglionic hypointensity was identified in 82 (90%) on contrast-enhanced fat-suppressed T1-weighted images. There was no statistical difference in the relative location between definite and probable SCSGs of the right and left sides with intragnalionic hypointensity on difference pulse sequences. Interrater and intrarater agreements on the location and intraganglionic hypointensity were excellent (κ-value, 0.749-1.000). CONCLUSION: 3T-MRI identified definite SCSGs at 73% of neck sites and varied location of the remaining SCSGs. Intraganglionic hypointensity was a characteristic feature of SCSGs.
Carotid Artery, Internal ; Consensus ; Ganglia ; Ganglia, Sympathetic* ; Head ; Humans ; Magnetic Resonance Imaging ; Neck ; Pathology ; Prospective Studies

Basal Ganglia ; pathology ; Female ; Humans ; Rodenticides ; poisoning ; Young Adult

OBJECTIVE: To explore the clinical characteristics and genetic etiology of a patient with adolescent-onset hypomyelinated leukodystrophy with atrophy of basal ganglia and cerebellum (H-ABC). METHODS: A patient who was diagnosed with H-ABC in March 2018 at the First Affiliated Hospital of Nanjing Medical University was selected as the study subject. Clinical data was collected. Peripheral venous blood samples of the patient and his parents were collected. The patient was subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing. RESULTS: The patient, a 31-year-old male, had manifested with developmental retardation, cognitive decline and abnormal gait. WES revealed that he has harbored a heterozygous c.286G>A variant of the TUBB4A gene. Sanger sequencing confirmed that neither of his parents has carried the same variant. Analysis with SIFT online software indicated the amino acid encoded by this variant is highly conserved among various species. This variant has been recorded by the Human Gene Mutation Database (HGMD) with a low population frequency. The 3D structure constructed by PyMOL software showed that the variant has a harmful effect on the structure and function of the protein. According to the guidelines formulated by the American College of Medical Genetics and Genomics (ACMG), the variant was rated as likely pathogenic. CONCLUSION The c.286G>A (p.Gly96Arg) variant of the TUBB4A gene probably underlay the hypomyelinating leukodystrophy with atrophy of basal ganglia and cerebellum in this patient. Above finding has enriched the spectrum of TUBB4A gene variants and enabled early definitive diagnosis of this disorder.
Male ; Humans ; Adolescent ; Adult ; Magnetic Resonance Imaging ; Basal Ganglia/pathology* ; Cerebellum ; Atrophy/pathology* ; Mutation ; Tubulin/genetics*

OBJECTIVETo study pathologic features of glial cells in progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) and to explore their pathologic significance.

METHODSBrain tissues from 2 cases with PSP and 3 cases with CBD, all confirmed by autopsies, were examined by routine neuropathologic methods, Gallyas-Braak staining and tau immunostaining. Brain tissues from 6 Alzheimer's disease cases, 4 cases with Parkinson's disease and 6 elderly with no neurologic abnormality were used as controls.

RESULTSGallyas-Braak staining demonstrated tuft-shaped astrocytes and coiled-body oligodendroglial cells in the brain tissues of 2 cases with PSP and 3 cases with CBD. The tuft-shaped astrocytes appeared prominently in the frontal and parietal cortex, basal ganglia and grey matter of the brainstem. The coiled-body oligodendroglial cells were distributed widely in the white matter of the frontal and parietal lobes, basal ganglia, brainstem and cerebellum. However, astrocytic plaques, composed of degenerative stubby processes with radiating arrangement, only appeared in the frontal, parietal and cingular cortex, as well as in the striatum of 3 cases with CBD. The astrocytic plaques and tuft-shaped astrocytes coexisted in the same areas, including parietal and cingular cortex and striatum, in CBD. All these glial abnormalities showed tau-positive immunoreaction not found in control cases.

CONCLUSIONSThe tuft-shaped astrocytes and coiled-body oligodendroglial cells are common glial morphologic features of both PSP and CBD. Astrocytic plaques are also characteristically seen in CBD.

Aged ; Aged, 80 and over ; Astrocytes ; pathology ; Basal Ganglia ; pathology ; Brain Stem ; pathology ; Cerebral Cortex ; pathology ; Humans ; Male ; Neurodegenerative Diseases ; pathology ; Oligodendroglia ; pathology ; Supranuclear Palsy, Progressive ; pathology

A case of Leigh's disease (subacute necrotizing encephalomyelopathy) is reported with such noteworthy features as early onset, dystonia, paraparesis the presence of low attenuation areas in both basal ganglias on computerized tomography of the brain and the presence of a high signal intensity in both basal ganglias in T2 weighted image by MR. The electron microscopic findings of muscle biopsy are suggestive of pleoconial mitochondrial myopathy.
Basal Ganglia/pathology ; Case Report ; Dystonia/diagnosis/*etiology ; Energy Metabolism ; Human ; Infant ; Leigh Disease/*diagnosis/metabolism/pathology ; Male ; Muscles/pathology

OBJECTIVE: To investigate the effect of bilateral superior cervical sympathetic ganglion occlusion (SCG) on aortic dissection and its possible mechanism. METHODS: Forty-five SD rats were randomly divided into three groups with 15 in each:blank control group, sham operation group and SCG group. β-aminopropione (666 mg·kg·d) was given by subcutaneous injection for 4 weeks to establish the aortic dissection model. Rats in SCG group were given SCG before the injection of β-aminopropione. Blood pressure and heart rate of the rats were monitored using noninvasive tail artery blood pressure measuring instrument; sympathetic activity was monitored using drug block method; the structure of aortic wall was observed using HE staining; collagen fibers in aortic wall was observed using Sirius red staining; protein expression of Apelin was detected by immunohistochemistry; and the protein expression of matrix metalloproteinase (MMP)-2, 9 was detected by Western blotting. RESULTS: During the experiment, the body mass of the sham operation group and SCG group was smaller than that of the blank control group (all <0.05), and the body mass of the SCG group was larger than that of the sham operation group (all <0.05). The heart rate and sympathetic activity of the sham operation group were higher than those of the blank control group (all <0.05), while the SCG group were lower (all <0.05). Compared with the blank control group, the aortic wall in the sham operation group was thickening, while that in the SCG group was improved. A large number of collagen-1 in the aortic wall of the blank control group was stained brown by Sirius red, which was lighter in SCG group, and the staining in the sham operation group was the lightest. Compared with the blank control group, the expression of Apelin, MMP-2 and MMP-9 protein in the sham operation group increased (all <0.05), while those in the SCG group decreased (all <0.05). CONCLUSIONS SCG can effectively reduce the incidence and mortality of aortic dissection in rats, which may be related to the inhibition of sympathetic activity and the decrease of collagen-1, Apelin, MMP-2 and MMP-9 expression.
Aneurysm, Dissecting ; pathology ; surgery ; Animals ; Aorta ; pathology ; Collagen Type I ; Ganglia, Sympathetic ; Random Allocation ; Rats ; Rats, Sprague-Dawley

Intestinal neuronal dysplasia (IND) type B is a disease of the submucosal plexus of intestine manifesting chronic intestinal obstruction or severe chronic constipation. IND is one of intestinal dysganglionoses and clinically closely associated with Hirschsprung's disease. Until recently, it is not fully clear whether IND is a congenital malformation or an acquired secondary condition related to some gastrointestinal problems. However, recently published data and consensus reports have enhanced our understanding of the pathogenesis and management of IND. The aim of this paper was to review the current state of knowledge regarding the controversial issues of IND including the etiology, classification, diagnostic criteria, and available therapeutic intervention.
Child ; Colon/*innervation/radiography ; Constipation/etiology ; Enteric Nervous System/*abnormalities ; Ganglia/pathology ; Gastrointestinal Motility ; Hirschsprung Disease/pathology ; Humans ; Immunohistochemistry ; Intestinal Diseases/*diagnosis/pathology ; Intestinal Mucosa/pathology

A subset of patients of amyotrophic lateral sclerosis (ALS) present with mutation of Cu/Zn superoxide dismutase 1 (SOD1), and such mutants caused an ALS-like disorder when expressed in rodents. These findings implicated SOD1 in ALS pathogenesis and made the transgenic animals a widely used ALS model. However, previous studies of these animals have focused largely on motor neuron damage. We report herein that the spinal cords of mice expressing a human SOD1 mutant (hSOD1-G93A), besides showing typical destruction of motor neurons and axons, exhibit significant damage in the sensory system, including Wallerian-like degeneration in axons of dorsal root and dorsal funiculus, and mitochondrial damage in dorsal root ganglia neurons. Thus, hSOD1-G93A mutation causes both motor and sensory neuropathies, and as such the disease developed in the transgenic mice very closely resembles human ALS.
Amyotrophic Lateral Sclerosis/enzymology/*pathology ; Animals ; Axons/*pathology ; Disease Models, Animal ; Ganglia, Spinal/pathology ; Humans ; Mice ; Mice, Transgenic ; Mitochondria/pathology ; Motor Neurons/metabolism/pathology ; Mutation ; Nerve Degeneration/*pathology ; Sensory Receptor Cells/*pathology ; Spinal Cord/*pathology ; Superoxide Dismutase/genetics/*physiology

The cell body or soma in the dosal root ganglion (DRG) is normally excitable and this excitability can increase and persist after an injury of peripheral sensory neurons. In a rat model of radicular pain, an intraforaminal implantation of a rod that chronically compressed the lumbar DRG ("CCD" model) resulted in neuronal somal hyperexcitability and spontaneous activity that was accompanied by hyperalgesia in the ipsilateral hind paw. By the 5th day after onset of CCD, there was a novel upregulation in neuronal expression of the chemokine, monocyte chemoattractant protein-1 (MCP-1 or CCL2) and also its receptor, CCR2. The neurons developed, in response to topically applied MCP-1, an excitatory response that they normally do not have. CCD also activated non-neuronal cells including, for example, the endothelial cells as evidenced by angiogenesis in the form of an increased number of capillaries in the DRG after 7 days. A working hypothesis is that the CCD induced changes in neurons and non-neuronal cells that may act together to promote the survival of the injured tissue. The release of ligands such as CCL2, in addition to possibly activating nociceptive neurons (maintaining the pain), may also act to preserve injured cells in the face of ischemia and hypoxia, for example, by promoting angiogenesis. Thus, somal hyperexcitability, as often said of inflammation, may represent a double edged sword.
Animals ; Chemokine CCL2 ; metabolism ; Ganglia, Spinal ; cytology ; pathology ; Hyperalgesia ; pathology ; Neuroglia ; cytology ; Nociceptors ; cytology ; Pain ; pathology ; Rats ; Rats, Sprague-Dawley ; Spinal Cord Compression ; physiopathology ; Up-Regulation

BACKGROUNDNucleus pulposus of intervertebral discs has proinflammatory characteristics that play a key role in neuropathic pain in lumbar herniated intervertebral disc. One of the most commonly used animal models (the traditional model) of non-compressive lumbar herniated intervertebral disc is created by L4-L5 hemilaminectomy and the application of autologous nucleus pulposus to cover the left L4 and L5 nerve roots in rats. However, such procedures have the disadvantages of excessive trauma and low success rate. We proposed a modified model of non-compressive lumbar herniated intervertebral disc in which only the left L5 dorsal root ganglion is exposed and transplanted with autologous nucleus pulposus following incision of epineurium. We aimed to compare the modified model with the traditional one with regard to trauma and success rate.

METHODSThirty Sprague-Dawley male rats were randomized into three groups: sham operation group (n = 6), traditional group (n = 12), and modified group (n = 12). The amount of blood loss and operative time for each group were analyzed. The paw withdrawal threshold of the left hind limb to mechanical stimuli and paw withdrawal latency to heat stimuli were examined from the day before surgery to day 35 after surgery.

RESULTSCompared with the traditional group, the modified group had shorter operative time, smaller amount of blood loss, and higher success rate (91.7% versus 58.3%, P < 0.05). There was no decrease in paw withdrawal latency in any group. The sham operation group had no decrease in postoperative paw withdrawal threshold, whereas the modified and traditional groups had significant reduction in paw withdrawal threshold after surgery (mechanical hyperalgesia).

CONCLUSIONSTransplantation of nucleus pulposus onto the L5 dorsal root ganglion following incision of epineurium in rats established an improved animal model of non-compressive lumbar herniated intervertebral disc with less trauma and more stable pain ethology.

Animals ; Disease Models, Animal ; Ganglia, Spinal ; pathology ; Intervertebral Disc Degeneration ; pathology ; Intervertebral Disc Displacement ; pathology ; Male ; Random Allocation ; Rats ; Rats, Sprague-Dawley