Bowel dysfunction in patients with cauda equina lesions

Despite their serious sequels on bowel function, lesions of the cauda equina have not been previously systematically studied in larger patient populations. This was the aim of the present report. From the registrars of a diagnostic and rehabilitation centres 67 patients with clinical, electrodiagnostic and radiological findings supportive of the cauda equina lesions were recruited. The Slovene versions of the standard questionnaires for anal incontinence and constipation were used. The responses were scored, impairments categorized and previous treatments noted. Neurological examination, electromyography (EMG) of lumbo-sacral myotomes, quantitative anal sphincter EMG and electromyographic evaluation of the sacral reflex were performed. Severe anal incontinence/constipation was reported by 18%/0%, moderate by 36%/33%, and slight by 28%/43% of our patients. Twenty-one per cent of patients wore pads continuously and 14% occasionally. More than half of the patients (60%) reported changes in their lifestyle due to anal incontinence. No patient had completely normal findings on neurological examination. Perianal sensory loss correlated ( P  < 0.05) with anal incontinence and gender with constipation (women >men). Only two patients had received medical attention for bowel dysfunction. Study thus demonstrated significant bowel impairment in patients with lesions of the cauda equina, which has received insufficient medical attention.     

Increased tone of the rectal wall in response to feeding persists in patients with cauda equina syndrome

The aim was to study fasting and postprandial rectal tone in patients with cauda equina injury. Electromechanical barostat measurement of rectal tone was made in 13 healthy volunteers and in five patients during a 10 min recording, while fasting and for 1 h after a 1000 kCal intake. A prompt decrease of rectal volume was observed in all control subjects and patients. The delay between the end of the meal and the onset of the rectal response was always less than 3 min in the five patients as well as in the control group. The rapidity of the rectal response to feeding observed in our five patients suggests that the rectal response was mediated via a neural or neurohumoral pathway despite severe injury of the sacral parasympathetic supply.     

Selective cauda equina hypertrophy with idiopathic inflammation

A 20-year-old woman with selective cauda equina hypertrophy presented with muscle weakness and severe pain in the lower extremities. Serial immunotherapy was not effective. We performed biopsy of the cauda equina, and laminectomy and duraplasty of the thoracolumbar region. Biopsy revealed marked infiltration of small lymphocytes and foamy macrophages in the endoneurium. Three years after decompression surgery, her symptoms have improved slightly without progression or relapse. This is the first case of selective cauda equina hypertrophy with idiopathic inflammation. We propose that this is a new disease entity.     

Lower motor neuron syndrome due to cauda equina hypertrophy with onion bulbs

Introduction: Hypertrophy of the nerve roots of the cauda equina may occur with both acquired and inherited neuropathies. Although selective nerve root involvement of the sensory roots has been described and termed chronic inflammatory sensory polyradiculoneuropathy (CISP), selective involvement of the proximal motor roots has not been described. Methods: Clinical, electrophysiological, MRI, and pathological findings are reported. Results: Here, we report a patient with cauda equina hypertrophy presenting with a pure lower motor neuron syndrome without clinical or electrophysiological evidence of sensory fiber involvement. Bowel and bladder functions were spared. Nerve root biopsy demonstrated abundant onion bulb formations. The patient experienced improvement in motor function with immunomodulatory treatment. Conclusions: We suggest the term chronic immune demyelinating motor polyradiculopathy (CIMP) to describe this particular form of CIDP, thereby expanding the clinical spectrum of CIDP. Muscle Nerve, 48: 301–305, 2013     

Hemangioblastoma of the cauda equina

A very rare hemangioblastoma of the filum terminale with sciatic pain and partial cauda syndrome in a 36-year-old man is presented. The difference between solitary hemangioblastoma, hemangioblastomatosis and von Hippel-Lindau disease is stressed. The literature is reviewed and the diagnosis of cauda equina neoplasms is discussed. Even nowadays often the final diagnosis is made late and only after operation and microscopical tissue examination.     

Epidemiology of cauda equina and conus medullaris lesions

The epidemiology of cauda equina and conus medullaris lesions is not well known, and this study aimed to provide further information on this topic. In the period 1996-2004, patients fulfilling the clinical, electrodiagnostic, and radiological criteria for such lesions were identified. For cauda equina/conus medullaris lesions an annual incidence rate of 3.4/1.5 per million, and period prevalence of 8.9/4.5 per 100,000 population were calculated. The values obtained are probably valid estimates of the incidence and prevalence of these lesions in developed countries.     

Lumbosacral epidural lipomatosis causing rapid onset cauda equina syndrome

Spinal epidural lipomatosis (SEL) is a rare cause of cauda equina syndrome (CES), which must be diagnosed with MRI in conjunction with a high level of clinical suspicion. Most reported cases are associated with obesity, steroid use or are secondary to endocrinopathies, frequently present subacutely or chronically, and have been managed with both surgical decompression and non-operative measures. We describe an obese 55-year-old man with rapid onset CES secondary to idiopathic lumbosacral SEL which was managed successfully with surgical decompression. Although often thought to be a trivial radiological finding, it is important not to be dismissive of patients presenting with compressive neuropathy and MRI evidence of space-occupying SEL.     

Tumors in the cauda equina: A SEER analysis of tumor types and predictors of outcome

Cauda equina tumors are histologically diverse. International Classification of Diseases for Oncology (ICD-O3) confers dedicated site code (C72. 1) for cauda equina. This code is excluded during analyses of other primary spinal cord tumors. In this retrospective study, the Surveillance, Epidemiology and End Results (SEER) data for primary cauda equina tumors (PCET, C72. 1) excluding the tumors of spinal meninges (C70. 1) from 1992 to 2015 were reviewed. Demographic characteristics, tumor types, and clinical outcomes were analyzed using univariable analysis. Overall survival was estimated using Kaplan-Meier methods and compared for age, histology and treatment type. 293 patients with PCET met inclusion criteria. The most common tumors comprised schwannoma (32%), myxopapillary ependymoma (21%), malignant ependymoma (22%). The median age at diagnosis was 50 years (range < 1 year to 98 years), 57% of patients were males. 77% of the patients underwent surgery. Median follow up time for these patients was 70 months. Of the 293 patients, 250 (85%) were living at the end of 2015. The cause of death was tumor or CNS related in 15 patients. 136 patients were followed for <5 years, of which 102 were censored and 34 died (11.6%) before 5 years. Using univariable analysis, age at diagnosis (Hazard Ratio, HR 1.05; confidence interval, CI 1.03–1.07; p < 0.001), malignant tumor type (HR 2.88, CI 1.15–7.19, p = 0.0239) and absence of surgical intervention (HR 2.54, CI1.26–5.11, p = 0.0092) were predictors of increased mortality. Although most patients did well, older age and lack of surgical intervention were associated with worse survival.     

Postoperative spinal adhesive arachnoiditis presenting with hydrocephalus and cauda equina syndrome

To our knowledge, the association between hydrocephalus and postoperative spinal adhesive arachnoiditis (SAA) has never been reported. Herein we describe an unusual case of a 45-year-old man with spinal adhesive arachnoiditis (SAA) who developed delayed-onset hypertensive hydrocephalus and cauda equina syndrome (CES) after multiple low-back surgeries. The patient's clinical presentation, imaging findings, surgical management, and the possible mechanisms are discussed in the light of the present literature.     

Saddle sensation is preserved in a few patients with cauda equina or conus medullaris lesions1

Although saddle sensory deficit seems the most useful clinical sign in the diagnosis of a cauda equina or conus medullaris lesion, findings of previous studies were controversial. The aim of the present study was to try to resolve this issue. The data from the author's series of patients with clinical, electrodiagnostic and radiological findings compatible with a cauda equina lesion were reviewed. Of the 117 patients in the series, 11 (10 men) did not have a saddle sensory deficit. These 11 patients had less severe sacral dysfunction than the others, and none of them needed urgent surgical intervention. They all had electromyographic (EMG) signs of a significant motor fibre lesion, and in seven men the sacral (penilo‐cavernosus) reflex was clinically abnormal. The study revealed normal saddle sensation in approximately 10% of patients with cauda equina or conus medullaris lesions. Dissociation between preserved touch sensation and abnormal EMG findings, as well as dissociation between preserved touch sensation and a non‐elicitable penilo‐cavernosus reflex might be explained by preservation of the thinner sensory nerve fibres, which are more resistant to compression. Although, saddle sensory loss seems to identify patients who might benefit from urgent spinal imaging and surgery, further diagnostic evaluation is also indicated in patients with normal saddle sensation, particularly due to the increased frequency of spinal tumours found in this subgroup.     

Minimally invasive discectomy for the treatment of disc herniation causing cauda equina syndrome

Cauda equina syndrome (CES) is a condition associated with significant morbidity that requires definitive surgical decompression of the nerve roots to prevent permanent disability. Traditionally, wide open decompression has been advocated to obtain optimal decompression with minimal complications. Some have been reluctant to employ minimally invasive strategies to treat urgent conditions. The authors present a small series of four patients who presented with CES and were treated with minimally invasive discectomy (MID). Operative length times (mean ± standard deviation, 102.8 ± 30.9 minutes) were comparable to reported operative length for open laminectomies. All patients had either stabilization or resolution of symptoms at the 6-week follow-up examination. No patients suffered a cerebrospinal fluid (CSF) leak post-operatively. This series of patients presenting with CED at a single institution treated with MID demonstrates that MID can be used to successfully treat CES. Even large fragments can be safely removed without increased risk of a CSF leak.     

Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease

A patient with coeliac disease developed a progressive cauda equina syndrome in the 2 months prior to his death, for which no cause could be identified. At post-mortem examination this proved to be due to disseminated enteropathy-type T-cell lymphoma. Although rare, disseminated enteropathy-type T-cell lymphoma should enter the differential diagnosis in any patient with coeliac disease developing new neurological signs.     

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. Muscle Nerve 52 : 94–102, 2015     

Chronic inflammatory demyelinating polyradiculoneuropathy presenting as cauda equina syndrome in a diabetic

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may occur in association with diabetes mellitus (DM). We report a case of a poorly controlled diabetic patient who presented with rapid onset of bilateral lower extremity weakness and sensory loss associated with sacral and posterior thigh paresthesias and urinary and bowel incontinence, indicative of cauda equina syndrome (CES). Subsequent evaluation was consistent with CIDP. Monthly infusions with intravenous immunoglobulins (IVIg) with strict glycemic control using insulin resulted in remarkable clinical and electrophysiological recovery. This case report describes a rare presentation of CIDP and emphasizes the importance of early utility of electrodiagnostic (EDX) studies in the clinical evaluation of diabetic patients presenting with rapidly progressive lower extremity weakness and sensory loss associated with diminished reflexes.     

Changes in compressed neurons from dogs with acute and severe cauda equina constrictions following intrathecal injection of brain-derived neurotrophic factor-conjugated polymer nanoparticles

This study established a dog model of acute multiple cauda equina constriction by experimental constriction injury (48 hours) of the lumbosacral central processes in dorsal root ganglia neurons. The repair effect of intrathecal injection of brain-derived neurotrophic factor with 15 mg encapsulated biodegradable poly(lactide-co-glycolide) nanoparticles on this injury was then analyzed. Dorsal root ganglion cells (L7) of all experimental dogs were analyzed using hematoxylin-eosin staining and immunohistochemistry at 1, 2 and 4 weeks following model induction. Intrathecal injection of brain-derived neurotrophic factor can relieve degeneration and inflammation, and elevate the expression of brain-derived neurotrophic factor in sensory neurons of compressed dorsal root ganglion. Simultaneously, intrathecal injection of brain-derived neurotrophic factor obviously improved neurological function in the dog model of acute multiple cauda equina constriction. Results verified that sustained intraspinal delivery of brain-derived neurotrophic factor encapsulated in biodegradable nanoparticles promoted the repair of histomorphology and function of neurons within the dorsal root ganglia in dogs with acute and severe cauda equina syndrome.     

Angiotropic lymphoma (intravascular large cell lymphoma) presenting with cauda equina syndrome

A 50-year-old man developed cauda equina syndrome of unknown etiology that was stable for 20 months. Two months prior to sudden death, he experienced new back pain, confusion, seizures, and multiple cranial nerve palsies. Neuropathologic examination revealed angiotropic lymphoma without parenchymal involvement or infarcts in the brain, spinal cord, and muscle. In addition, nerve roots in the cauda equina contained angiotropic lymphoma and infarcts of various ages. Angiotropic lymphoma should be considered as a cause of cauda equina syndrome and of disorders that affect the central and peripheral nervous systems concurrently.     

Post-hemorrhagic hydrocephalus presenting as cauda equina syndrome in a patient with spinal dysraphism

Hydrocephalus has varied presentations in patients with a history of spinal dysraphism. This is a unique case of post-subarachnoid hemorrhage hydrocephalus presenting as cauda equina syndrome. We report on a 32-year-old woman with remotely repaired spinal defect who experienced subarachnoid hemorrhage and underwent anterior communicating artery aneurysm clipping. Post-operatively, she developed urinary and fecal incontinence as the sole presenting symptom of communicating post-hemorrhagic hydrocephalus. New neurological deficits in this population can also be attributed to recurrent cord tethering or syrinx, both of which were demonstrated on her lumbar spine MRI, but her incontinence resolved with external ventricular drain placement and cerebrospinal fluid diversion. There are few case reports of patients with closed neural tube defects and hydrocephalus and none in the adult population to our knowledge. Neurological change in patients with any history of spinal dysraphism may reflect altered cerebrospinal fluid dynamics affecting either end of the neuraxis.