Diastematomyelia and Diplomyelia

Bret, P; Patet, J. D.; Lapras, C

doi:10.1007/978-1-4613-8829-6_4

Cited by 6 publications

(4 citation statements)

References 57 publications

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“…Congenital malformations of the spinal cord are called myelodysplasia. Different types of myelodysplasia are among others: hydromyelia, syringomyelia, diastematomyelia, diplomyelia, and dimyelia [ 9 , 10 ]. Hydromyelia [ 1 ] is a condition in which the central canal is absent or dilated.…”

Section: Case Presentationmentioning

confidence: 99%

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Clinical, MRI, and histopathological findings of congenital focal diplomyelia at the level of L4 in a female crossbred calf

et al. 2020

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Background This case report describes the clinical signs of a calf with focal diplomyelia at the level of the fourth lumbar vertebra. Magnetic resonance imaging (MRI) images and histological findings of the affected spinal cord are included in this case report. This case differs from previously reported cases in terms of localization and minimal extent of the congenital anomaly, clinical symptoms and findings during further examinations. Case presentation The calf was presented to the Farm Animal Health clinic, Faculty of Veterinary Medicine, Utrecht University, with an abnormal, stiff, ‘bunny-hop’ gait of the pelvic limbs. Prominent clinical findings included general proprioceptive ataxia with paraparesis, pathological spinal reflexes of the pelvic limbs and pollakiuria. MRI revealed a focal dilated central canal, and mid-sagittal T2 hyperintense band in the dorsal part of the spinal cord at the level of the third to fourth lumbar vertebra. By means of histology, the calf was diagnosed with focal diplomyelia at the level of the fourth lumbar vertebra, a rare congenital malformation of the spinal cord. The calf tested positive for Schmallenberg virus antibodies, however this is not considered to be part of the pathogenesis of the diplomyelia. Conclusions This case report adds value to future clinical practice, as it provides a clear description of focal diplomyelia as a previously unreported lesion and details its diagnosis using advanced imaging and histology. This type of lesion should be included in the differential diagnoses when a calf is presented with a general proprioceptive ataxia of the hind limbs. In particular, a ‘bunny-hop’ gait of the pelvic limbs is thought to be a specific clinical symptom of diplomyelia. This case report is of clinical and scientific importance as it demonstrates the possibility of a focal microscopic diplomyelia, which would not be evident by gross examination alone, as a cause of hind-limb ataxia. The aetiology of diplomyelia in calves remains unclear.

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Section: Case Presentationmentioning

confidence: 99%

“…Diplomyelia [ 1 , 2 ] is defined as an isolated, true duplication of the spinal cord within one set of meninges. Dimyelia [ 9 ] is a generalized duplication of the spinal cord. Segmental hypoplasia is a condition in which some spinal cord segments are smaller than others.…”

Section: Case Presentationmentioning

confidence: 99%

Clinical, MRI, and histopathological findings of congenital focal diplomyelia at the level of L4 in a female crossbred calf

et al. 2020

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“…In our case the location of the defect at C4-C5 levels was atypical. Moreover, our patient demonstrated symmetrical lower paraparesis, what is also atypical because the side of the hypoplastic cord is usually consistent with the lateralization of the neurologic symptoms or signs of the "orthopaedic syndrome" such as scoliosis or foot deformity [5].…”

Section: Discussionmentioning

confidence: 50%

Diplomyelia in a patient with a clinical suspicion of autosomal recessive spastic ataxia of Charlevoix-Saguenay type (ARSACS)

Dziewulska

2020

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Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a form of cerebellar ataxia related to mutations in the SACS gene on chromosome 13q12.12 encoding sacsin protein. Characteristic clinical features are ataxia, spasticity, distal muscle wasting, neuropathy, dysarthria, nystagmus, and finger or feet deformities. The presented case concerns a 32-year-old man with clinical diagnosis of ARSACS. Magnetic resonance imaging (MRI) scans of the brain revealed cerebellar atrophy typical of the disease while neuroimaging of the C1-C3 and C6-Th12 segments showed only the thin thoracic spinal cord. The patient died suddenly and a gross examination of the spinal cord revealed extraspinal tumour at the C4-C5 levels, which turned out to be an additional spinal cord. Microscopic examination showed an extensive ischemic necrosis involving C6-Th5 segments of the proper spinal cord, and disturbed intrinsic structure containing many pathological vessels of the extra spinal cord. Lack of visualization of C4-C5 spinal cord segments on MRI scans made diagnosis of diplomyelia in vivo impossible. However, diplomyelia does not exclude coexistence of ARSACS because of the occurrence of such clinical symptoms as dysarthria or nystagmus which cannot be explained by the presence of the spinal cord defect. The possibility of congenital malformations of the spinal cord in adults should be remembered as their early identification and surgical correction can improve neurological symptoms.

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“…This abnormality can be associated with other forms of spinal dysraphism or can be an isolated condition. 1 The lesion can be very subtle with no obvious outward signs or can present with cutaneous or neurologic deficits in the lower extremities that can be secondary to a tethered cord. 2 Neurologically, these lesions can produce abnormalities varying from no loss of nerve function to extensive upper or lower motor neuron dysfunction.…”

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confidence: 99%

Urodynamic and Neurophysiologic Evaluation of Patients With Diastematomyelia

Kothari

Bauer

1997

J Child Neurol

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Diastematomyelia is a form of an occult dysraphism, which can present with lower urinary tract dysfunction. We present the findings in 21 patients with diastematomyelia evaluated at the Children's Hospital in Boston. Seventy-six percent of the patients were female. Eighty-six per cent had a cutaneous lesion present overlying the spine. Urodynamic and electrophysiologic studies showed abnormalities in 17 (81%) of 21 patients, of whom 13 (62%) had upper motor neuron dysfunction. Seventeen patients underwent corrective spinal surgery and none of the patients deteriorated on a follow-up urodynamic and electrophysiologic study. We recommend that patients with cutaneous, midline lower back lesions with or without lower urinary tract dysfunction be evaluated with a urodynamic and electrophysiologic study to characterize the extent of neurologic and urologic dysfunction.

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Diastematomyelia and Diplomyelia

Cited by 6 publications

References 57 publications

Clinical, MRI, and histopathological findings of congenital focal diplomyelia at the level of L4 in a female crossbred calf

Clinical, MRI, and histopathological findings of congenital focal diplomyelia at the level of L4 in a female crossbred calf

Diplomyelia in a patient with a clinical suspicion of autosomal recessive spastic ataxia of Charlevoix-Saguenay type (ARSACS)

Urodynamic and Neurophysiologic Evaluation of Patients With Diastematomyelia

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