Baastrup's Disease as a Rare Cause of Cauda Equina Syndrome: A Case Report.

CASE: "Baastrup's disease" is a relatively common anatomical condition, controversially recognized as a possible cause of back pain but frequently missed by clinicians of different specialties. We present a rare case of Baastrup's disease causing compression to the cauda equina nerves due to the formation of a large pseudotumoral epidural mass. The patient underwent a successful surgical decompression with remarkable improvement in her neurological presentation. The histopathological findings consisted of increased collagen deposition and chondroid metaplasia. CONCLUSION: Clinicians should be aware of possible cauda equina syndrome in rare cases of Baastrup's disease due to pseudotumoral mass compression in the lumbar spine.

[A case of neurolymphomatosis presented as cauda equine syndrome accompanied with M-proteinemia].

A 63-year-old man developed a syndrome of cauda equine, with the numbness which is a left lower extremity from the left buttocks, weakness of left leg, and a dysfunction of bladder and bowel. Enhanced MRI revealed the enhancement of lower cauda equine, and a nerve conduction test revealed decreased F-wave persistency in the tibial nerve and increased F-wave latency in the peroneal nerve on the both sides. M-proteinemia was admitted and myeloma was suspected. By a biopsy of a vertebral arch, we diagnosed with diffuse large B-cell lymphoma. We treated with dexamethasone and R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone (prednisolone)), then the symptom was improved. In case of caude equine syndrome with M-proteinemia, a possibility of the malignant lymphoma should also be considered.

Lumbar spinal canal MRI diameter is smaller in herniated disc cauda equina syndrome patients.

INTRODUCTION: Correlation between magnetic resonance imaging (MRI) and clinical features in cauda equina syndrome (CES) is unknown; nor is known whether there are differences in MRI spinal canal size between lumbar herniated disc patients with CES versus lumbar herniated discs patients without CES, operated for sciatica. The aims of this study are 1) evaluating the association of MRI features with clinical presentation and outcome of CES and 2) comparing lumbar spinal canal diameters of lumbar herniated disc patients with CES versus lumbar herniated disc patients without CES, operated because of sciatica. METHODS: MRIs of CES patients were assessed for the following features: level of disc lesion, type (uni- or bilateral) and severity of caudal compression. Pre- and postoperative clinical features (micturition dysfunction, defecation dysfunction, altered sensation of the saddle area) were retrieved from the medical files. In addition, anteroposterior (AP) lumbar spinal canal diameters of CES patients were measured at MRI. AP diameters of lumbar herniated disc patients without CES, operated for sciatica, were measured for comparison. RESULTS: 48 CES patients were included. At MRI, bilateral compression was seen in 82%; complete caudal compression in 29%. MRI features were not associated with clinical presentation nor outcome. AP diameter was measured for 26 CES patients and for 31 lumbar herniated disc patients without CES, operated for sciatica. Comparison displayed a significant smaller AP diameter of the lumbar spinal canal in CES patients (largest p = 0.002). Compared to average diameters in literature, diameters of CES patients were significantly more often below average than that of the sciatica patients (largest p = 0.021). CONCLUSION: This is the first study demonstrating differences in lumbar spinal canal size between lumbar herniated disc patients with CES and lumbar herniated disc patients without CES, operated for sciatica. This finding might imply that lumbar herniated disc patients with a relative small lumbar spinal canal might need to be approached differently in managing complaints of herniated disc. Since the number of studied patients is relatively small, further research should be conducted before clinical consequences are considered.

Neuroprotective Effects of Valproic Acid in a Rat Model of Cauda Equina Injury.

BACKGROUND: Histone deacetylase inhibitors, including valproic acid (VPA), are promising therapeutic interventions in neurological disorders and play an important role in synaptic activity and neuronal function. METHODS: A total of 30 rats were randomly allocated to 3 groups: sham, control, and VPA. The rats in the VPA and control groups received laminectomy at the L4 level of the vertebrae and silicone gel implantation into the epidural spaces L5 and L6. Rats in the sham group only received laminectomy at the L4 level of vertebrae without any implantation. VPA (300 mg/kg in saline) was administered 2 hours before the surgery. After the surgery, the VPA group received further VPA injections at 300 mg/kg twice a day for 1 week. The same volume of saline was injected in the control group. Neurobehavioral tests using the Basso, Beattie, Bresnahan scale and the oblique board test were performed for 1 week starting at 2 hours before surgery up to day 7 after surgery. At day 7 after surgery, tissues from the compressed cauda equina (L5-L6) were subjected to hematoxylin and eosin, luxol fast blue, or immunofluorescence staining, whereas the terminal deoxynucleotidyl transferase-mediated biotinylated UTP nick-end label assay staining was performed on the tissue from the dorsal root ganglions and the lumbar segment of the spinal cord proximal to the compressed cauda equina (L5-L6). RESULTS: The behavioral results suggested a significant improvement in the lower limb motor function in the VPA group compared with controls (P < 0.05). Furthermore, histologic assessment revealed a significant reduction in nerve fibers showing Wallerian degeneration and demyelinating lesions in the VPA group, in addition to an increased myelination compared with the control group (P < 0.05). The terminal deoxynucleotidyl transferase-mediated biotinylated UTP nick-end label assay staining revealed a significant decrease in the number of apoptotic neurons in the spinal cord anterior horn and dorsal root ganglions in the VPA group compared with controls (P < 0.05). CONCLUSIONS: Our data demonstrated that VPA could alleviate cauda equina injury, reduce apoptotic cells, and improve motor recovery, suggesting a neuroprotective effect in acute cauda equina syndrome.

Encephalitis associated with human herpesvirus-7 infection in an immunocompetent adult.

BACKGROUND: Primary Human herpesvirus-7 (HHV-7) infection usually occurs during childhood and causes several clinical manifestations: mainly exanthem subitum (roseola infantum), followed by a lifelong latent state with possible reactivation in case of immunodeficiency. Nevertheless, some considerably different approaches exist regarding the natural history of HHV-7 and the possible consequences of HHV-7 infection in immunocompetent adults. In particular, little is known about its pathogenic role in central nervous system (CNS) disease in nonimmunosuppressed adults. Specifically, in case of encephalitis, it is important to distinguish between infectious encephalitis and postinfectious encephalomyelitis for the management of patients CASE PRESENTATION: We describe here a case of encephalitis associated to human herpesvirus-7 with associated polymyeloradiculopathy in an immunocompetent patient which may contribute to the delineation of the approach to a patient profile with a similar clinical presentation and evolution to those presented in the literature. CONCLUSIONS: This case may alert clinicians to consider this specific etiology in the differential diagnosis of encephalopathy in patients with suspected infectious encephalitis who do not respond to acyclovir or in patients who develop acute polymyeloradiculopathy, considering that HHV-7 may be a pathological factor and that a timely diagnosis is crucial for the early administration of specific treatment.

Definitions of traumatic conus medullaris and cauda equina syndrome: a systematic literature review.

STUDY DESIGN: A systematic review. OBJECTIVES: Conus medullaris syndrome (CMS) and cauda equina syndrome (CES) are well-known neurological entities. It is assumed that these syndromes are different regarding neurological and functional prognosis. However, literature concerning spinal trauma is ambiguous about the exact definition of the syndromes. METHODS: A MEDLINE, EMBASE and Cochrane literature search was performed. We included original articles in which clinical descriptions of CMS and/or CES were mentioned in patients following trauma to the thoracolumbar spine. RESULTS: Out of the 1046 articles, we identified 14 original articles concerning patients with a traumatic CMS and/or CES. Based on this review and anatomical data from cadaveric and radiological studies, CMS and CES could be more precisely defined. CONCLUSION: CMS may result from injury of vertebrae Th12-L2, and it involves damage to neural structures from spinal cord segment Th12 to nerve root S5. CES may result from an injury of vertebrae L3-L5, and it involves damage to nerve roots L3-S5. This differentiation between CMS and CES is necessary to examine the hypothesis that CES patients tend to have a better functional outcome.

The long term outcome of micturition, defecation and sexual function after spinal surgery for cauda equina syndrome.

BACKGROUND: Cauda equina syndrome (CES) is a rare neurologic complication of lumbar herniated disc for which emergency surgical decompression should be undertaken. Despite the common belief that the restoration of functions that are affected by CES can take several years postoperatively, follow up seldom exceeds the first year after surgery. Long term outcome of especially micturition, defecation and sexual function-which are by definition affected in CES-are unknown. The aim of this study is to evaluate 1) postoperative long term outcome of micturition, defecation and sexual function in CES patients 2) attitude of patients towards received hospital care with regard to (recovery of) these functions. METHODS: CES patients were selected by screening the records of all patients operated on lumbar herniated disc in our university hospital between 1995-2010. A questionnaire was sent to the selected CES patients evaluating current complaints of micturition, defecation and sexual function and attitude towards delivered care with focus on micturition, defecation and sexual function. RESULTS: Thirty-seven of 66 eligible CES patients were included (response rate 71%, inclusion rate 56%). Median time after surgery was 13.8 years (range 5.8-21.8 years). Dysfunction at follow up was highly prevalent: 38% micturition dysfunction, 43% defecation dysfunction and 54% sexual dysfunction. Younger age at presentation was associated with sexual dysfunction at follow up: for every year younger at presentation, odds ratio for sexual dysfunction at follow up was 1.11 (p = 0.035). Other associations with outcome were not identified. Two-third of the CES patients wished their neurosurgeon had given them more prognostic information about micturition, defecation and sexual function. CONCLUSION: The presented data demonstrate that dysfunction of micturition, defecation and sexual function are still highly prevalent in a large number of CES patients even years postoperatively. These alarming follow up data probably have a devastating effect on personal perceived quality of life, which should be studied in more detail. CES patients communicate a clear demand for more prognostic information. The presented figures enable clinicians to inform their CES patients more realistically about long term postoperative outcome of micturition, defecation and sexual function after surgical intervention.

Ejaculation and sperm characteristics in men with cauda equina and conus medullaris syndromes.

STUDY DESIGN: Retrospective. OBJECTIVES: The objective of the study was to describe the type of ejaculation in patients with cauda equina (CE) and conus medullaris (CM) lesions, and to analyse sperm quality. SETTING: France. METHODS: One hundred sixty-six patients with CE and CM lesions were included. Diagnosis was based on clinical (no motor responses, sensation or sacral reflexes) and urodynamic assessments (no detrusor activity). Vibromassage (VM) was used to induce ejaculation according to the recommendations for patients with spinal cord injury. If ejaculation did not occur, oral midodrine was administered in progressive doses. Retrograde ejaculation was systematically sought. Sperm parameters were analysed according to World Health Organisation recommendations (2010). RESULTS: Eighty-nine patients were included. Eleven ejaculated on the first VM trial (four anterograde (AE), six retrograde (RE) and one antero-retrograde (ARE)). Five patients continued trials of VM alone, two of whom ejaculated following a mean 1.9 trials (one RE, one ARE). Twenty-six patients underwent trials of VM+ midodrine, 18 of whom ejaculated following a mean 4.4 trials with a mean dose of 22.5 g of midodrine (2 AE, 13 RE and 5 ARE). Fifty-three ejaculates from 26 patients were analysed. Sperm concentration was low in 90.6% of samples; total necrospermia was found in 65% and asthenospermia in 95% of samples. CONCLUSION: Ejaculation is difficult to induce using VM in patients with CE and CM lesions, and requires high doses of midodrine. Sperm counts were generally low, and asthenospermia and necrospermia were found in the majority of specimens. Cryopreservation of sperm should be systematic in case of medically assisted procreation.

Neurological deterioration in cauda equina syndrome is probably progressive and continuous. Implications for clinical management.

Fifty-six human and animal studies of cauda equina syndrome (CES) were reviewed. The evidence from human studies was poor (level IV). Evidence from animal studies and limited evidence from human studies suggest that structural and functional neurological losses are a progressive, continuous process. The longer the cauda equina nerve roots are compressed the greater the harm and the poorer the extent of recovery. This should prompt diagnosis and surgery for all CES patients as soon as practicably possible.

[A case of conus medullaris infarction expanding to the vertebral bodies, major psoas and erector spinae muscles].

A 77-year-old woman presented with conus medullaris and cauda equina syndrome following a sudden pain in the bilateral lower abdomen and right buttock. Lumbar magnetic resonance imaging (MRI) showed not only a conus medullaris lesion, but also several lesions in the vertebral bodies (L1, L2), right major psoas muscle, right multifidus muscle and bilateral erector spinae muscles. As these areas receive blood supply from each branch of the same segmental artery, we considered all of the lesions as infarctions that were a result of a single parent vessel occlusion. It is known that a vertebral body lesion can be accompanied by a spinal cord infarction, but in combination with infarction of a muscle has not been reported. This is the first report of a concomitant spinal cord and muscle infarction revealed by MRI. It is noteworthy that a spinal cord infarction could expand not only to neighboring vertebral bodies, but also to muscles.

Innervation and function of rat tail muscles for modeling cauda equina injury and repair.

INTRODUCTION: The rat tail exhibits functional impairment after cauda equina injury. Our goal was to better understand the innervation and roles of muscles that control the tail. METHODS: Adult rats received either: (1) ventral root injury; (2) caudales nerve injury; or (3) mapping of sacrococcygeal myotomes. Activation of small muscles within the tail itself (intrinsics) was compared with that of larger lumbosacral muscles acting on the tail (extrinsics). Behavioral testing of tail movement was done 1 week later. RESULTS: Rats that received ventral root injury exhibited multiple behavioral deficits, whereas rats with injury to caudales nerves maintained more fully preserved tail movement. Mapping studies revealed much broader overlap of myotomes for extrinsic muscles. CONCLUSIONS: Extrinsic tail muscles play a greater role in tail movement in the rat than their intrinsic counterparts and are innervated by multiple neurological segments. These findings have major implications for future research on cauda equina injury.

Spontaneous regression of posterior epidural migrated lumbar disc fragments: case series.

BACKGROUND CONTEXT: Posterior epidural migrated lumbar disc fragments is an extremely rare disorder. Surgical treatment was performed in all reported cases. To the best of our knowledge, there are no reported cases of the use of conservative treatment for posterior epidural migrated lumbar disc fragments. PURPOSE: To report the possibility of a spontaneous regression of posterior epidural migrated lumbar disc fragments. STUDY DESIGN: Case series. METHODS: Four patients with posterior epidural migrated lumbar disc fragments were treated at Karatsu Red Cross Hospital between April 2008 and August 2010. Spontaneous regression of the posterior epidural migrated lumbar disc fragments with relief of symptoms was observed on magnetic resonance imaging (MRI) in three cases. Another patient underwent surgical treatment. The present and previously reported cases of posterior epidural migrated lumbar disc fragments were analyzed with respect to patient age, imaging features on MRI, the level of the lesion, clinical symptoms, treatment, and outcomes. RESULTS: Conservative treatment was successful, and spontaneous lesion regression was seen on MRI with symptom relief in three cases. CONCLUSIONS: Although posterior epidural migrated lumbar disc fragment cases are generally treated surgically, the condition can regress spontaneously over time, as do sequestrated disc fragments. Spontaneous regression of lumbar disc herniations is a widely accepted observation at present. Posterior epidural migrated lumbar disc fragments fall under the sequestrated type of disc herniation. In fact, the course of treatment for posterior epidural migrated lumbar disc fragments should be determined based on the symptoms and examination findings, as in cases of ordinary herniation. However, providing early surgical treatment is important if the patient has acute cauda equina syndrome or the neurologic symptoms worsen over time.

Vertebral body infarct and ventral cauda equina enhancement: two confirmatory findings of acute spinal cord infarct.

Two valuable confirmatory MRI findings of acute spinal cord infarct are highlighted and discussed: concomitant vertebral body infarct and ventral cauda equina nerve root enhancement.

Acute myelopathy or cauda equina syndrome in HIV-positive adults in a tuberculosis endemic setting: MRI, clinical, and pathologic findings.

BACKGROUND AND PURPOSE: Cape Town is the center of an HIV-tuberculosis coepidemic. This study's aim was to highlight the importance and to describe the MR imaging features of tuberculosis in acute myelopathy and cauda equina syndrome in HIV-positive adults. To accomplish this we retrospectively reviewed the MR imaging and clinico-pathologic findings of HIV-positive patients presenting to our hospital with recent onset paraplegia and sphincter dysfunction over a 4-year period, 2008-2011. MATERIALS & METHODS: MR imaging, CD4 count, and CSF analysis and pathology were correlated in 216 cases. RESULTS: Fifty-eight percent (127) of subjects were female. The mean age was 37 years. The median CD4 count was 185 cells/µL. Twenty-five percent (54) of patients were on antiretroviral therapy. MR imaging showed spondylitis in 30% (65). The median CD4 count in these patients was significantly higher than in the remainder. Disk destruction was common and 10% had synchronous spondylitis elsewhere in the spinal column. Thirty percent (64) had features of myelitis/arachnoiditis. Twenty-five percent (55) had no MR imaging abnormality. In 123 (57%) of cases with a definitive etiology on CSF culture or biopsy, 84 (68%) were attributable to tuberculosis including all spondylitis cases and 40% of nonspondylitis cases. Twelve (10%) were due to nontuberculous infection and 12 (10%) had HIV-associated tumors including 2 rare Epstein-Barr-related tumors. CONCLUSIONS: In our setting, acute onset myelopathy/cauda equina syndrome in HIV-positive patients is largely attributable to tuberculosis with nonspondylitic forms being more common than spondylitis and associated with a lower CD4 count.

A contrast-enhancing lumbar ligamentum flavum haematoma.

We present a case of lower back pain with lumbar nerve compromise due to a ligamentum flavum haematoma which was successfully treated surgically. A 62-year-old man was evaluated for lower back pain with associated leg pain and early signs of cauda equina syndrome. MRI of the lumbar spine demonstrated a contrast-enhancing mass adjacent to the lamina of L3 which was causing severe canal stenosis. Surgical excision of the lesion was recommended. The patient underwent an L3 laminectomy with excision of the epidural lesion. Histopathology showed it to be a haematoma of the ligamentum flavum with no untoward features. The patient recovered without complication.