MRI of the brain and cervical spinal cord in rhizomelic chondrodysplasia punctata

Neurology ◽  
2006 ◽  
Vol 66 (6) ◽  
pp. 798-803 ◽  
Author(s):  
A. M. Bams-Mengerink ◽  
C. B.L.M. Majoie ◽  
M. Duran ◽  
R.J.A. Wanders ◽  
J. Van Hove ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Chondrodysplasia Punctata ◽  
Rhizomelic Chondrodysplasia Punctata ◽  
The Brain

scholarly journals A Case of Rhizomelic Chondrodysplasia Punctata in Newborn

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Nalan Karabayır ◽  
Gonca Keskindemirci ◽  
Erdal Adal ◽  
Orhan Korkmaz
Keyword(s):  
Soft Tissues ◽  
Clinical Symptoms ◽  
Current Knowledge ◽  
Chondrodysplasia Punctata ◽  
Long Bones ◽  
Rhizomelic Chondrodysplasia Punctata ◽  
Cervical Spinal Stenosis ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Severe Growth

Rhizomelic chondrodysplasia punctate (RCDP) is a rare autosomal recessive peroxisomal disease. The main features of the disease are shortening of the proximal long bones, punctate calcifications located in the epiphyses of long bones and in soft tissues around joints and vertebral column, vertebral clefting, dysmorphic face, and severe growth retardation, whereas cervical spinal stenosis may also rarely be present. Imaging of the brain and spinal cord in patients with this disorder may aid prognosis and guide management decisions. We report the newborn diagnosed as CDP with cervical stenosis. Our aim is to discuss current knowledge on etiopathogenesis as well as radiological and clinical symptoms of diseases associated with CDP.

scholarly journals Differential Response in Novel Stem Cell Niches of the Brain after Cervical Spinal Cord Injury and Traumatic Brain Injury

2018 ◽  
Vol 35 (18) ◽  
pp. 2195-2207 ◽  
Author(s):  
Aditi Falnikar ◽  
Jarred Stratton ◽  
Ruihe Lin ◽  
Carrie E. Andrews ◽  
Ashley Tyburski ◽  
...  
Keyword(s):  
Traumatic Brain Injury ◽  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Stem Cell ◽  
Brain Injury ◽  
Cervical Spinal Cord ◽  
Cervical Spinal Cord Injury ◽  
Cord Injury ◽  
The Brain ◽  
Stem Cell Niches

scholarly journals Temporospatial Development of Neuropathologic Findings in a Canine Model of Mucopolysaccharidosis IIIB

2020 ◽  
pp. 030098582096012
Author(s):  
Tyler A. Harm ◽  
Shannon J. Hostetter ◽  
Ariel S. Nenninger ◽  
Bethann N. Valentine ◽  
N. Matthew Ellinwood ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Canine Model ◽  
Brain Regions ◽  
Large Animal ◽  
Lysosomal Storage ◽  
Large Animal Models ◽  
Naturally Occurring ◽  
The Brain ◽  
Deficient Activity

Mucopolysaccharidosis (MPS) IIIB is a neuropathic lysosomal storage disease characterized by the deficient activity of a lysosomal enzyme obligate for the degradation of the glycosaminoglycan (GAG) heparan sulfate (HS). The pathogenesis of neurodegeneration in MPS IIIB is incompletely understood. Large animal models are attractive for pathogenesis and therapeutic studies due to their larger size, outbred genetics, longer lifespan, and naturally occurring MPS IIIB disease. However, the temporospatial development of neuropathologic changes has not been reported for canine MPS IIIB. Here we describe lesions in 8 brain regions, cervical spinal cord, and dorsal root ganglion (DRG) in a canine model of MPS IIIB that includes dogs aged from 2 to 26 months of age. Pathological changes in the brain included early microscopic vacuolation of glial cells initially observed at 2 months, and vacuolation of neurons initially observed at 10 months. Inclusions within affected cells variably stained positively with PAS and LFB stains. Quantitative immunohistochemistry demonstrated increased glial expression of GFAP and Iba1 in dogs with MPS IIIB compared to age-matched controls at all time points, suggesting neuroinflammation occurs early in disease. Loss of Purkinje cells was initially observed at 10 months and was pronounced in 18- and 26-month-old dogs with MPS IIIB. Our results support the dog as a replicative model of MPS IIIB neurologic lesions and detail the pathologic and neuroinflammatory changes in the spinal cord and DRG of MPS IIIB-affected dogs.

Spinomuscular Coherence in Monkeys Performing a Precision Grip Task

2008 ◽  
Vol 99 (4) ◽  
pp. 2012-2020 ◽  
Author(s):  
Tomohiko Takei ◽  
Kazuhiko Seki
Keyword(s):  
Spinal Cord ◽  
Sensorimotor Integration ◽  
Unique Feature ◽  
Cervical Spinal Cord ◽  
Precision Grip ◽  
Muscle Physiology ◽  
Field Potentials ◽  
Spinal Interneurons ◽  
Arm Muscles ◽  
The Brain

We recorded local field potentials (LFPs) from cervical spinal cord (C5–C8) in monkeys performing a precision grip task and examined their coherence with electromyographic (EMG) activities (spinomuscular coherence) recorded from hand and arm muscles. Among 164 LFP-EMG pairs, significant coherence was found in 34 pairs (21%). We classified the coherence into two groups based on its frequency range, narrowband coherence, and broadband coherence. The narrowband coherence was restricted to discrete frequencies in the range of 14–55 Hz and was widespread throughout the superficial and deep gray matter. In contrast, the broadband coherence distributed between 10 and 95 Hz and was found only in the ventral half of the spinal cord. The narrowband coherence suggests that oscillations, which have been described in many motor control areas of the brain, could also pass though spinal interneurons to affect motor output and sensorimotor integration. On the other hand, the broadband coherence could be a unique feature of spinal motoneuron-muscle physiology.

Cervical spinal cord in experimental hydrocephalus

1972 ◽  
Vol 37 (5) ◽  
pp. 538-542 ◽  
Author(s):  
George J. Dohrmann
Keyword(s):  
Spinal Cord ◽  
Brain Stem ◽  
Cervical Spinal Cord ◽  
Central Canal ◽  
Content Type ◽  
Experimental Hydrocephalus ◽  
Physiological Pressure ◽  
The Brain ◽  
Shunting Procedure ◽  
Fine Print

✓ Adult dogs were rendered hydrocephalic by the injection of kaolin into the cisterna magna. One group of dogs was sacrificed 1 month after kaolin administration, and ventriculojugular shunts were performed on the other group. Hydrocephalic dogs with shunts were sacrificed 1 day or 1 week after the shunting procedure. All dogs were perfused with formalin at physiological pressure, and the brain stem and cervical spinal cord were examined by light microscopy. Subarachnoid granulomata encompassed the superior cervical spinal cord and dependent surface of the brain stem. Rarefaction of the posterior white columns and clefts or cavities involving the gray matter posterior to the central canal and/or posterior white columns were present in the spinal cords of both hydrocephalic and shunted hydrocephalic dogs. Predominantly in the dogs with shunts, hemorrhages were noted in the spinal cord in association with the clefts or cavities. A mechanism of ischemia followed by reflow of blood is postulated to explain the hemorrhages in the spinal cords of hydrocephalic dogs with shunts.

scholarly journals Morphologic and Immunoperoxidase Study of Neurologic Lesions in Naturally Acquired Rabies of Raccoons

1992 ◽  
Vol 4 (4) ◽  
pp. 369-373 ◽  
Author(s):  
A. N. Hamir ◽  
G. Moser ◽  
C. E. Rupprecht
Keyword(s):  
Spinal Cord ◽  
Brain Stem ◽  
Ganglion Cells ◽  
Cervical Spinal Cord ◽  
Fluorescent Antibody ◽  
Antibody Test ◽  
Immunoperoxidase Method ◽  
Spinal Cord Regions ◽  
Trigeminal Nerves ◽  
The Brain

Histopathologic (hematoxylin and eosin [HE]) and immunoperoxidase (streptavidin-biotin complex) methods were used for examination of formalin-fixed tissues of rabid raccoons from an enzootic area of Pennsylvania. Extensive morphologic lesions of rabies encephalitis were present in the cerebrum and the brain stem regions. Negri bodies were detected by both methods and were present in the brain (cerebral cortex, hippocampus, brain stem, cerebellum, and cervical spinal cord) and in the ganglia of the trigeminal nerves. The viral inclusions were also seen in ganglion cells in the tongue, parotid salivary glands, pancreas, intestines, and adrenal glands. These sites were not associated with any inflammatory cellular infiltrate. The immunoperoxidase method was superior to HE for the detection of Negri bodies. Because lesions of rabies encephalitis were consistently observed in the cerebrum, brain stem, and cervical spinal cord regions, these areas of the brain should be included when raccoons are examined by the fluorescent antibody test for rabies.

scholarly journals Multiple nocardial abscesses of the brainstem and spinal cord diagnosed after an open biopsy through a cervical partial central corpectomy: case report

2015 ◽  
Vol 23 (3) ◽  
pp. 290-293 ◽  
Author(s):  
Ian Peeters ◽  
Jan W. Casselman ◽  
Stefaan J. Vandecasteele ◽  
Alexander Janssen ◽  
Bart Regaert ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Emission Tomography ◽  
Open Biopsy ◽  
Difficult Diagnosis ◽  
Positron Emission ◽  
The Central Nervous System ◽  
Walking Difficulty ◽  
Brain And Spinal Cord ◽  
The Brain

Nocardiosis of the central nervous system is a challenging and difficult diagnosis for the clinician. The combination of infections of the brain and spinal cord is even more rare. The authors report on a patient with multiple lesions in the brainstem and cervical spinal cord. This 81-year-old immunocompetent woman presented with symptoms of progressive walking difficulty and ataxia. The results of an extensive workup with laboratory investigation, MRI, lumbar puncture, positron emission tomography (PET), and bone marrow biopsy remained inconclusive. Only after an open biopsy of a cervical lesion by an anterior approach through a partial central corpectomy of the cervical spine, was the diagnosis of nocardiosis made, allowing for specific antibiotic treatment.

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