Steroid-Responsive Encephalopathy,Dropped Head Syndrome,and Hypertension in a Toddler: Is There a Clue?

BackgroundParaneoplastic manifestations may provide an early clue to underlying malignancies in children.MethodsWe describe a 3-year-old girl with an unusual neurological paraneoplastic syndrome of steroid-responsive encephalopathy and dropped head syndrome associated with paraspinal neuroblastoma.ResultsCranial neuroimaging, metabolic screen, and thyroid functions were normal. Abdominal imaging identified a paraspinal neuroblastoma. Encephalopathy and neck extensor weakness responded to pulse corticosteroids followed by tumor resection.ConclusionsThis is a unique child with paraneoplastic steroid-responsive encephalopathy and dropped head syndrome in association with neuroblastoma.     

Cauda equina syndrome caused by a migrated bullet in dural sac

Cauda equina syndrome (CES) is a serious neurological condition and the most common cause is a central disc herniation. Migration of a bullet down the spinal canal is uncommon. In this report, the authors present an unusual case of cauda equina syndrome caused by a migrated bullet in dural sac. The patient's cauda equina syndrome was related to possible compression of cauda equina resulting from both bone fragments in L1 level and bullet itself in S2 level. The patient's symptoms and neurological deficits resolved considerably after surgery. In our opinion, it is important to pay attention to diversity of injury when confronted with a gunshot wound. It is essential to perform early and sufficient surgical decompression of the cauda equina to provide a better postoperative neurological recovery.     

Treatment of neuroblastoma with intraspinal extensions.

Neuroblastoma is the most common malignant cause of spinal compression in the paediatric population. Chemotherapy is commonly considered as the first-line treatment for these patients. The role of neurosurgical decompression and radiotherapy are still controversial. Thirteen children diagnosed as having neuroblastoma with intraspinal extension were included in this report. All patients presented with neurological deficits and were treated with chemotherapy initially, after which 3 patients recovered, 4 improved and 6 were aggravated into paraplegia. Two of the 6 aggravated patients received emergent laminectomy with removal of intraspinal tumour and recovered satisfactorily. Although spread of tumour into the spinal canal indicates an advanced disease, aggressive treatments such as chemotherapy and surgical resection can often improve neurological symptoms and life quality. Neurological decompression is recommended for patients with intraspinal neuroblastoma and rapid neurological deterioration during chemotherapy.     

Spinal cord compression by secondary epi- and intradural metastases in childhood

Over a 9-year period, 35 out of 614 children with malignant tumours who were treated at the Centre Léon Bérard developed spinal metastases. Of these, 18 with known malignancies before the development of spinal cord compression are reviewed. The most common tumours causing spinal metastases were Ewing's sarcoma, neuroblastoma and renal tumours. Cord compression occurred 5–88 months after the diagnosis of systemic cancer. The median interval from first symptoms to the diagnosis of compression was 17 days. There were 16 patients with neurological deficit, including 5 with paraplegia. Specific imaging procedures were performed in 16 patients. Treatment included operation in 8 patients, followed by chemotherapy (6 patients) and/or radiotherapy (4 patients); 9 of the 10 non-operated patients received radiotherapy. Only 6 patients had a significant neurological improvement. All patients but 1 died within a median time of 2 months. Early diagnosis might prevent permanent disability in these children with a short survival expectancy. Received: 12 March 1997     

Neurologic paraneoplastic syndrome with anti-CV2/CRMP5 antibodies revealing a small cell lung cancer. Effectiveness of the lung cancer treatment

IntroductionParaneoplastic neurological syndrome associated with anti-CV2/CRMP5 antibodies are rare. Various clinical manifestations can occur, cerebellar ataxia, polyneuropathy, optic neuritis with NORB or uveitis. Small cell lung carcinoma is generally responsible.Case reportWe report the case of a 64-year-old man who developed visual symptoms with papilledema, cerebellar signs, polyneuropathy confirmed with a neurophysiological studies. Anti-CV2/CRMP5 antibodies were present. A small cell lung carcinoma was responsible for this paraneoplastic syndrome revealing the cancer. The paraneoplastic syndrome improved with radio chemotherapy of the cancer alone.ConclusionA paraneoplastic neurological syndrome must be evoked in case of an atypic neurological syndrome. This diagnostic can be confirmed by the presence of anti-neuronal antibodies. In this case, a small cells cancer of the lung must be research.     

Sudden hypotonic paraparesis caused by tophaceous gout of the lumbar spine.

Gouty arthritis of the axial skeleton is rare, and neurological complications due to spinal cord or nerve root compression secondary to tophaceous deposits have been seldom reported. We describe a patient with chronic gouty arthritis who developed an acute paraparesis caused by tophaceous cauda equina compression. Surgical removal of the mass resulted in complete recovery.     

Primary B-cell lymphoma of the cauda equina, successfully treated with high-dose methotrexate plus high-dose cytarabine: a case report with MRI findings

Primary malignant lymphoma of the cauda equina is an extremely rare disease. Previously, there have been only 12 reported cases of malignant lymphoma of the cauda equina, and most cases relapsed early in the clinical course. So, the optimal treatment strategy for this condition has not been established yet and the prognosis is thought to be poor. We experienced a case of B-cell malignant lymphoma of the cauda equina, with rapid progression of the muscle weakness of the bilateral lower extremities, successfully treated with high-dose methotrexate plus high-dose cytarabine (Ara-C) chemotherapy, followed by radiotherapy and in complete remission without any recurrence signs, 1.5 years after the initial diagnosis. Intrathecal chemotherapy with MTX, Ara-C, and predonisolone was simultaneously performed. We should carefully continue to monitor the clinical course of our case, with the examinations of magnetic resonance imaging and cerebrospinal fluid in order not to overlook any subtle neurological changes or other clinical symptoms.     

The clinical features and outcome of scan-negative and scan-positive cases in suspected cauda equina syndrome: a retrospective study of 276 patients

Background

The majority of patients presenting with suspected clinical cauda equina syndrome (CES) have no identifiable structural cause for their symptoms (‘scan-negative’ CES). Understanding these patients aids clinical differentiation and management in CES.

Methods

A retrospective electronic note review was undertaken of patients presenting with suspected CES, defined as ≥?1 of acute bladder, bowel, sexual dysfunction or saddle numbness, to a regional neurosciences centre. We investigated radiology, clinical features, psychiatric and functional disorder comorbidities and outcome of patients with ‘scan-negative’ CES and patients with MRI confirmed compression of the cauda equina (‘scan-positive’ CES).

Results

276 patients were seen over 16 months. There were three main radiologically defined patient groups: (1) ‘scan-positive’ CES (n?=?78, mean age 48 years, 56% female), (2) ‘scan-negative’ CES without central canal stenosis but with lumbosacral nerve root compression not explaining the clinical presentation (n?=?87, mean age 43 years, 68% female) and (3) ‘scan-negative’ CES without neural compromise (n?=?104, mean age 42 years, 70% female). In the two ‘scan-negative’ groups (no neural compromise and nerve root compression), there were higher rates of functional disorders (37% and 29% vs. 9%), functional neurological disorders (12% and 11% vs 0%) and psychiatric comorbidity (53% and 40% vs 20%). On follow-up (mean 13–16 months), only 1 of the 191 patients with ‘scan-negative’ CES was diagnosed with an explanatory neurological disorder (transverse myelitis).

Conclusions

The data support a model in which scan-negative cauda equina syndrome arises as an end pathway of acute pain, sometimes with partly structural findings and vulnerability to functional disorders.

     

Short- and long-term outcome of patients with symptoms of spinal cord compression by neuroblastoma

Aim Prospective trials on neuroblastoma‐induced myelopathy are lacking. Therefore, we retrospectively analysed patients in four national neuroblastoma trials. Method Neuroblastoma patients diagnosed between August 1989 and December 2008 were included. Clinical and molecular data were available in the national trials database. Additional details on neurological findings, treatment, and outcome were collected using a questionnaire. Results Among 2603 patients, 122 (61 males and 61 females) had symptoms of spinal cord compression (SCC), and 99 of these were included in the final long‐term analysis. The survival of patients with symptoms of SCC was better than that of patients without symptoms. Patients first presented with lower extremity motor impairment (95%), impaired cutaneous sensibility (58%), neuropathic pain (56%), bladder dysfunction (44%), and/or constipation (34%). Symptoms improved after first‐line neurosurgery in 36 out of 52 patients and after first line chemotherapy in 30 out of 47 patients (p=0.77). After a median observation time of 8 years 1 month (range 1mo–19y 6mo), 71 out of 99 patients still had residual impairments: motor impairment (43%), scoliosis (31%), impaired bladder function (26%), constipation (19%), impaired cutaneous sensibility (17%), growth delay (14%), and neuropathic pain (5%). The initial treatment had no clear impact on the frequency of late effects. Interpretation This retrospective analysis showed no clear advantage of either first‐line neurosurgery or chemotherapy and that most patients still exhibit residual symptoms and require specialized care.     

Antineuronal Nuclear Autoantibody Type 1/Anti-Hu–Associated Opsoclonus Myoclonus and Epilepsia Partialis Continua: Case Report and Literature Review

BackgroundOpsoclonus-myoclonus syndrome is a rare clinical condition that has been associated with neuroblastoma. There are few reported examples of ANNA-1/anti-Hu antibodies in children with neuroblastoma and opsoclonus-myoclonus, all in children aged less than three years of age.MethodsWe describe the new onset of focal seizures without alteration of consciousness and opsoclonus-myoclonus in an 11-year-old girl with ANNA-1/anti-Hu positivity and a paraspinal ganglioneuroblastoma. A systematic review of the literature of children with ANNA-1/anti-Hu positivity and malignancy was also performed.ResultsFourteen patients were identified, eight of whom had opsoclonus-myoclonus. Although epilepsia partialis continua has been described in association with several neuronal autoantibodies, association with ANNA-1/anti-Hu has not been reported.ConclusionsWe describe epilepsia partialis continua in a child with ANNA-1/anti-Hu antibodies and neuroblastoma. Testing for antineuronal antibodies should be considered in children presenting with either opsoclonus-myoclonus or epilepsia partialis continua.     

Visualization of the electrical activity of the cauda equina using a magnetospinography system in healthy subjects

ObjectiveTo establish a method to measure cauda equina action fields (CEAFs) and visualize the electrical activities of the cauda equina in a broadly aged group of healthy adults.MethodsUsing a 124-channel magnetospinography (MSG) system with superconducting interference devices, the CEAFs of 43 healthy volunteers (22–64 years of age) were measured after stimulation of the peroneal nerve at the knee. Reconstructed currents were obtained from the CEAFs and superimposed on the X-ray image. Conduction velocities were also calculated from the waveform of the reconstructed currents.ResultsThe reconstructed currents were successfully visualized. They flowed into the L5/S1 foramen about 8.25–8.95 ms after the stimulation and propagated cranially along the spinal canal. In 32 subjects (74%), the conduction velocities of the reconstructed currents in the cauda equina could be calculated from the peak latency at the L2–L5 level.ConclusionsMSG visualized the electrical activity of the cauda equina after peroneal nerve stimulation in healthy adults. In addition, the conduction velocities of the reconstructed currents in the cauda equina could be calculated, despite previously being difficult to measure.SignificanceMSG has the potential to be a novel and noninvasive functional examination for lumbar spinal disease.     

Ependymal cyst in the lumbar spine associated with cauda equina compression.

There are many reports of extradural ependymal cysts in the literature; however, reports of intradural ependymal cysts are very rare and there has been no prior mention of an ependymal cyst originating from the filum terminale. In this report we present the case of a 31-year-old woman with an ependymal cyst that caused cauda equina compression, and discuss the clinical profile of the case in terms of symptoms, diagnostic images, pathohistological findings, and surgical procedures. To our knowledge, this is the first report of an ependymal cyst that caused cauda equina compression. The cyst was successfully treated by excision of the cyst during careful intraoperative monitoring to prevent neurological damage to the conus medullaris and cauda equina.     

Cauda equina syndrome with total myelographic block (author's transl)]

Twenty-seven cases of complete subarachnoid block discovered through myelography are analysed with regard to the causes of obstruction, the neurologic symptoms, and the cerebrospinal fluid. The neurologic signs in cases of cauda equina compression, most often caused by massive disc protrusions (20 patients), vary considerably and rarely correspond to the gross myelographic findings. Bilateral neurologic symptoms of any degree with or without sphincter disturbance were observed in 21 among 27 cases and are signs of cauda compression. In contrast to cases of sciatica with unilateral root compression and radicular symptoms neurological symptoms are not reliable in revealing the site of the lesion in cases of massive central disc protrusions. Therefore radiculography (myelography of the cauda equina) is necessary for diagnosis. In addition to the well-known elevation of the spinal fluid protein and pleocytosis, the presence of neutrophile granulocytes is frequently found (11 of 20 cases) in cytological analysis.     

Cauda equina syndrome due to recurrent malignant lymphoma of the spinal cord. A case report]

A 50-year-old man noticed a mass in the right cervical region and presented to our hospital. He underwent biopsy of a cervical lymph node, which revealed non-Hodgkin's lymphoma (diffuse large B cell, lymphoblastic type) histologically. He was treated with chemotherapy (CHOP) and radiation, and achieved complete remission. Two months later, he was admitted because of distal pain and extensive numbness of the lower limb as well as weakness of the left leg. Lumbar MRI showed an area of abnormal intensity in the cauda equina. Cytological examination of cerebrospinal fluid showed class V (lymphoma cells), so he was diagnosed as having recurrent malignant lymphoma of the spinal cord. He was treated with intrathecal chemotherapy and irradiation. After the treatment the mass in the cauda equina disappeared and the neurological symptoms in his legs resolved. It is rare for malignant lymphoma to recur in the spinal cord, particularly the cauda equina. It is well known that cauda equina syndrome can be caused by vertebral lesions and primary spinal cord tumors, but it is also necessary to keep malignant lymphoma of the cauda equina in mind.     

Fluorescence-guided resection of primary and recurrent malignant gliomas with 5-aminolevulinic acid. Preliminary results

Background and purposeExtent of resection plays a key role in the treatment of malignant gliomas (MGs). Patients with complete glioma removal, followed by chemoradiation, obtain the longest overall and progression-free survival. Fluorescence-guided resection of MGs enables intraoperative visualization of glioma tissue and increases control of the resection. The authors present preliminary results of 5-aminolevulinic acid (5-ALA) application during the resection of primary and recurrent MGs.Material and methodsSix patients with either a suspected malignant glioma based on magnetic resonance imaging (MRI) or with recurrent glioblastoma multiforme were enrolled in the study. The extent of resection was calculated according to the postoperative MRI performed within 72 hours. Preoperative and early postoperative neurological status and Karnofsky Performance Scale (KPS) were compared.ResultsFluorescence of tumour tissue was observed in 5/6 patients (five with the histopathological diagnosis of glioblastoma multiforme and one with neurotoxoplasmosis and AIDS). Complete tumour resection was achieved in 5 patients. Postoperative KPS and neurological status deteriorated in 2 cases. Radiotherapy and chemotherapy did not interfere with the sensitivity of the fluorescence guided tumour visualization.ConclusionsFluorescence-guided resection of primary and recurrent MGs with 5-ALA improves control of the tumour resection. It enables the cytoreduction to be maximized but experience in neuro-oncological surgery is required to avoid serious, postoperative neurological deficits.     

Lumbar spinal stenosis: Assessment of cauda equina involvement by electrophysiological recordings

Abstract The objective of this study was to investigate the relationship between electrophysiological recordings and clinical as well as radiological findings in patients suggestive to suffer from a lumbar spinal stenosis (LSS). We hypothesise that the electrophysiological recordings, especially SSEP, indicate a lumbar nerve involvement that is complementary to the neurological examination and can provide confirmatory information in less obvious clinical cases. In a prospective cohort study, 54 patients scheduled for surgery due to LSS were enrolled in an unmasked, uncontrolled trial. All patients were assessed by neurological examination, electrophysiological recordings, and magnetic resonance imaging (MRI) of the lumbar spine. The electrophysiological recordings focused on spinal lumbar nerve involvement. Results About 88% suffered from a multisegmental LSS and 91% of patients respectively complained of chronic lower back pain and/or leg pain for more than 3 months, combined with a restriction in walking distance. The neurological examination revealed only a few patients with sensory and/or motor deficits while 87% of patients showed pathological electrophysiological recordings (abnormal tibial SSEP in 78% of patients, abnormal H-reflex in 52% of patients). Conclusions Whereas the clinical examination, even in severe LSS, showed no specific sensory-motor deficit, the electrophysiological recordings indicated that the majority of patients had a neurogenic disorder within the lumbar spine. By the pattern of bilateral pathological tibial SSEP and pathological reflexes associated with normal peripheral nerve conduction, LSS can be separated from a demyelinating polyneuropathy and mono-radiculopathy. The applied electrophysiological recordings, especially SSEP, can confirm a neurogenic claudication due to cauda equina involvement and help to differentiate neurogenic from vascular claudication or musculo-skeletal disorders of the lower limbs. Therefore, electro-physiological recordings provide additional information to the neurological examination when the clinical relevance of a radiologically-suspected LSS needs to be confirmed.     

Association of electrochemical skin conductance with neuropathy in chemotherapy-treated patients

Purpose

Chemotherapy-induced peripheral neuropathy (CIPN) is an adverse event of cancer treatment that can affect sensory, motor, or autonomic nerves. Assessment of autonomic neuropathy is challenging, with limited available tools. Accordingly, it is not routinely assessed in chemotherapy-treated patients. In this study, we aimed to examine whether electrochemical skin conductance (ESC) via Sudoscan, a potential measure of autonomic function, associates with subjective and objective measures of CIPN severity and autonomic neuropathy.

Methods

A cross-sectional assessment of patients who completed neurotoxic chemotherapy 3–24 months prior was undertaken using CIPN patient-reported outcomes (EORTC-QLQ-CIPN20), clinically graded scale (NCI-CTCAE), neurological examination score (TNSc), autonomic outcome measure (SAS), and Sudoscan. Differences in CIPN severity between participants with or without ESC dysfunction were investigated. Linear regression analyses were used to identify whether ESC values could predict CIPN severity.

Results

A total of 130 participants were assessed, with 93 participants classified with CIPN according to the clinically graded scale (NCI-CTCAE/grade ≥ 1), while 49% demonstrated hands or feet ESC dysfunction (n = 46). Participants with ESC dysfunction did not significantly differ from those with no dysfunction on multiple CIPN severity measures (clinical-grade, patient-report, neurological examination), and no differences on the autonomic outcome measure (SAS) (all p > 0.0063). Linear regression analyses showed that CIPN could not be predicted by ESC values.

Conclusions

The inability of ESC values via Sudoscan to predict clinically-graded and patient-reported CIPN or autonomic dysfunction questions its clinical utility for chemotherapy-treated patients. The understanding of autonomic neuropathy with chemotherapy treatment remains limited and must be addressed to improve quality of life in cancer survivors.

     

Subacute reversible toxic encephalopathy related to treatment with capecitabine: A case report with literature review and discussion of pathophysiology

IntroductionCapecitabine, a 5-fluorouracil (5FU) pro-drug, is increasingly used in breast and gastrointestinal cancers due to its more convenient oral route of administration when compared to 5FU. Despite its widespread use, there are only a few reports on capecitabine CNS toxicity, while the pathogenic basis of such toxicity remains unclear.CaseA 69-year-old male presented with recurrent generalized seizures 2.5 months after preoperative chemoradiotherapy with capecitabine in locally advanced rectal cancer. Brain MRI revealed a diffuse, subcortical white matter alteration suggestive of vasogenic edema. The diagnosis of toxic encephalopathy was supported after elimination of alternative causes of the neurological dysfunction and complete resolution of clinical and imaging findings after 3 months of no further chemotherapy.ConclusionsGiven the expanding use of capecitabine, physicians should be aware of this potential complication when a neurological worsening occurs during or after treatment with this chemotherapeutic agent. In our case, as in previously described cases encephalopathy was characterized by a favorable course after cessation of the drug. Vasogenic edema rather than cytotoxic edema may play a pivotal pathogenetic role in this form of encephalopathy.     

Hemodynamic alterations following a cerebellar arteriovenous malformation resection: Case report and densitometric quantitative analysis from CT imaging

BackgroundCerebellar arteriovenous malformations (cAVMs) are rare and challenging lesions with an aggressive natural history. The mechanisms whereby a patient can worsen clinically after a supratentorial AVM resection include an acute alteration in cerebral hemodynamics, which is a known cause of postoperative hyperemia, edema and/or hemorrhage. These phenomena has not been described for cAVMS. Moreover, the underlying pathophysiology of edema and hemorrhage after AVM resection still remains controversial.MethodsWe report a patient that presented an abrupt neurological deterioration after cAVM surgical resection. Emergent external ventricular drainage to treat incipient hydrocephalus only partially reverted the patient's deterioration. Consecutive post-surgery CT images revealed fourth ventricle compression secondary to cerebellar swelling that concurred with a new neurological deterioration. Densitometric analysis was performed in these CT images to reveal the nature of these changes as well as their evolution over time.ResultsImportantly, we demonstrated a dynamic increase in the cerebellum mean density at the interval of Hounsfield values which correspond to hyperemia values. These changes were dynamic, and when hyperemia resolved and cerebellar density returned to basal levels, the fourth ventricle re-expanded and the patient neurologically recovered.ConclusionsThis study demonstrated the utility of quantitative CT image analysis in the context of hemodynamic alterations following cAVM resection. Densitometric CT analysis demonstrated that hyperemic changes, but not ischemic ones, were time-dependent and were responsible for swelling and hemorrhage that conditioned neurological status and patient's evolution.