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Idiopathic ventral spinal cord herniation: an increasingly recognized cause of thoracic myelopathy.

Berg-Johnsen J, Ilstad E, Kolstad F, Züchner M, Sundseth J - J Cent Nerv Syst Dis (2014)

Bottom Line: ISCH is probably a more common cause of thoracic myelopathy than previously recognized.The patients usually develop progressive myelopathy for several years before the correct diagnosis is made.Early diagnosis is important in order to treat the patients before the myelopathy has become advanced.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Oslo University Hospital, Rikshospitalet, Oslo, Norway. ; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

ABSTRACT
Idiopathic spinal cord herniation (ISCH), where a segment of the spinal cord has herniated through a ventral defect in the dura, is a rarely encountered cause of thoracic myelopathy. The purpose of our study was to increase the clinical awareness of this condition by presenting our experience with seven consecutive cases treated in our department since 2005. All the patients developed pronounced spastic paraparesis or Brown-Séquard syndrome for several years (mean, 4.7 years) prior to diagnosis. MRI was consistent with a transdural spinal cord herniation in the mid-thoracic region in all the cases. The patients underwent surgical reduction of the herniated spinal cord and closure of the dural defect using an artificial dural patch. At follow-up, three patients experienced considerable clinical improvement, one had slight improvement, one had transient improvement, and two were unchanged. Two of the four patients with sphincter dysfunction regained sphincter control. MRI showed realignment of the spinal cord in all the patients. ISCH is probably a more common cause of thoracic myelopathy than previously recognized. The patients usually develop progressive myelopathy for several years before the correct diagnosis is made. Early diagnosis is important in order to treat the patients before the myelopathy has become advanced.

No MeSH data available.


Related in: MedlinePlus

Preoperative MRI studies obtained in the thoracic spine of a 56-year-old woman who presented with a Brown-Séquard syndrome (case 4). (A) sagittal T2-weighted image shows that the spinal cord is ventrally dislocated in the spinal canal at the level of T5 with a spacious CSF volume posteriorly. (B) axial T2-weighted image at the level of T5 demonstrating a ventral adhesion with soft tissue outside the dura strongly indicating herniation of the spinal cord.
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f1-jcnsd-6-2014-085: Preoperative MRI studies obtained in the thoracic spine of a 56-year-old woman who presented with a Brown-Séquard syndrome (case 4). (A) sagittal T2-weighted image shows that the spinal cord is ventrally dislocated in the spinal canal at the level of T5 with a spacious CSF volume posteriorly. (B) axial T2-weighted image at the level of T5 demonstrating a ventral adhesion with soft tissue outside the dura strongly indicating herniation of the spinal cord.

Mentions: Seven consecutive patients operated for ISCH at the Oslo University Hospital, Rikshospitalet, in the time period 2005–2011 were included in the study. The following background data were obtained from the patients’ medical journals: age, sex, time to diagnosis, and operated level. The patients were evaluated with full neurological examination before surgery and at 12 months follow-up. Radiological assessment was conducted with MRI preoperatively and at 12 months follow-up. In all the seven patients, the diagnosis was made on MRI of the thoracic spine that demonstrated a focal ventral displacement of the spinal cord, which appeared abnormally thinned in the anteroposterior dimension (Fig. 1A and B). Dorsal to this kink of the spinal cord was a capacious space with cerebrospinal fluid (CSF) signal characteristics on MRI. The herniation was situated in the mid-thoracic region (T4–8) in all the seven patients. No bone scalloping, major degeneration of the disk, or local angular kyphosis at the site of herniation was observed.


Idiopathic ventral spinal cord herniation: an increasingly recognized cause of thoracic myelopathy.

Berg-Johnsen J, Ilstad E, Kolstad F, Züchner M, Sundseth J - J Cent Nerv Syst Dis (2014)

Preoperative MRI studies obtained in the thoracic spine of a 56-year-old woman who presented with a Brown-Séquard syndrome (case 4). (A) sagittal T2-weighted image shows that the spinal cord is ventrally dislocated in the spinal canal at the level of T5 with a spacious CSF volume posteriorly. (B) axial T2-weighted image at the level of T5 demonstrating a ventral adhesion with soft tissue outside the dura strongly indicating herniation of the spinal cord.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4196882&req=5

f1-jcnsd-6-2014-085: Preoperative MRI studies obtained in the thoracic spine of a 56-year-old woman who presented with a Brown-Séquard syndrome (case 4). (A) sagittal T2-weighted image shows that the spinal cord is ventrally dislocated in the spinal canal at the level of T5 with a spacious CSF volume posteriorly. (B) axial T2-weighted image at the level of T5 demonstrating a ventral adhesion with soft tissue outside the dura strongly indicating herniation of the spinal cord.
Mentions: Seven consecutive patients operated for ISCH at the Oslo University Hospital, Rikshospitalet, in the time period 2005–2011 were included in the study. The following background data were obtained from the patients’ medical journals: age, sex, time to diagnosis, and operated level. The patients were evaluated with full neurological examination before surgery and at 12 months follow-up. Radiological assessment was conducted with MRI preoperatively and at 12 months follow-up. In all the seven patients, the diagnosis was made on MRI of the thoracic spine that demonstrated a focal ventral displacement of the spinal cord, which appeared abnormally thinned in the anteroposterior dimension (Fig. 1A and B). Dorsal to this kink of the spinal cord was a capacious space with cerebrospinal fluid (CSF) signal characteristics on MRI. The herniation was situated in the mid-thoracic region (T4–8) in all the seven patients. No bone scalloping, major degeneration of the disk, or local angular kyphosis at the site of herniation was observed.

Bottom Line: ISCH is probably a more common cause of thoracic myelopathy than previously recognized.The patients usually develop progressive myelopathy for several years before the correct diagnosis is made.Early diagnosis is important in order to treat the patients before the myelopathy has become advanced.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Oslo University Hospital, Rikshospitalet, Oslo, Norway. ; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

ABSTRACT
Idiopathic spinal cord herniation (ISCH), where a segment of the spinal cord has herniated through a ventral defect in the dura, is a rarely encountered cause of thoracic myelopathy. The purpose of our study was to increase the clinical awareness of this condition by presenting our experience with seven consecutive cases treated in our department since 2005. All the patients developed pronounced spastic paraparesis or Brown-Séquard syndrome for several years (mean, 4.7 years) prior to diagnosis. MRI was consistent with a transdural spinal cord herniation in the mid-thoracic region in all the cases. The patients underwent surgical reduction of the herniated spinal cord and closure of the dural defect using an artificial dural patch. At follow-up, three patients experienced considerable clinical improvement, one had slight improvement, one had transient improvement, and two were unchanged. Two of the four patients with sphincter dysfunction regained sphincter control. MRI showed realignment of the spinal cord in all the patients. ISCH is probably a more common cause of thoracic myelopathy than previously recognized. The patients usually develop progressive myelopathy for several years before the correct diagnosis is made. Early diagnosis is important in order to treat the patients before the myelopathy has become advanced.

No MeSH data available.


Related in: MedlinePlus