Solitary sclerosis: Progressive neurological deficit from a spatially isolated demyelinating lesion: A further report

Abstract

Context

Progressive myelopathy can be a manifestation of a variety of disorders including progressive multiple sclerosis. However it is extremely uncommon for a single lesion to cause a progressive myelopathy in MS. Such a myelopathy, i.e. a progressive neurological deficit from a solitary demyelinating lesion, not fulfilling the International diagnostic criteria for MS or Neuromyelitis Optica was first reported in 2012 and termed ‘solitary sclerosis’.

Method

We report 3 further cases of progressive myelopathy fulfilling the diagnostic criteria for solitary sclerosis.

Findings

Two patients had a single demyelinating lesion in the cervical cord and the third patient had it in the brain stem. All patients had serial MRI scans showing no dissemination or progression of lesions. Extensive diagnostic tests including aquaporin 4 antibodies were negative in all. At last follow-up at a median of 3.8 years, all patients continued to clinically progress despite immunosuppressive treatment.

Conclusion/Clinical Relevance

Solitary demyelinating lesions can cause a progressive myelopathy without clinical or radiological evidence of dissemination. Importantly, clinicians, both surgical and medical should be aware of such a diagnosis, to avoid invasive and often harmful tests particularly biopsies.     

A rare condition of anorectal dysfunction in a patient with multiple sclerosis: Coexistence of faecal incontinence and mechanical constipation: Report of case

INTRODUCTION

Multiple sclerosis is a chronic demyelinating neurological disease and causing a variety of neurological symptoms, including discomfort of anorectal function. Constipation and faecal incontinence present as anorectal dysfunction in MS and anal manometry, colonic transit time, electromyography, and defecography can be used for assessment.

PRESENTATION OF CASE

We presented a thirty-three years old woman with rare condition of anorectal dysfunction in multiple sclerosis. Anal manometry, defecography were done, and synchronously anal incontinence and mechanical constipation due to rectocele and anismus were detected in this patient.

DISCUSSION

Although anal incontinence and constipation are seen often in patients with multiple sclerosis, in the literature, coexistence of animus, rectocele and anal incontinence are quite rare.

CONCLUSION

Defecography and anal manometry are useful diagnostic methods for demonstration of anorectal dysfuntions in patients with MS.     

Functional electrical stimulation as a component of activity-based restorative therapy may preserve function in persons with multiple sclerosis

Objective

To examine the effect of functional electrical stimulation (FES) cycling on disability progression in persons with multiple sclerosis (MS).

Design

Retrospective cohort, 40 participants with mean follow-up of 15 months.

Setting

International Center for Spinal Cord Injury at Kennedy Krieger Institute in Baltimore, a rehabilitation referral center.

Participants

Forty consecutive persons with MS undergoing rehabilitation from 2007 to 2011, with at least two evaluations based on the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI).

Interventions

FES cycling as part of activity-based restorative therapy interventions.

Outcome measures

Change in Expanded Disability Status Scale (EDSS) and ISNCSCI motor, light touch, and pin prick scores from baseline to latest evaluation.

Results

In 71% of patients, activity-based rehabilitation included FES cycling. There was no disability progression on the EDSS. Lower extremity motor scores improved or stabilized in 75% of patients with primary progressive MS (PPMS), 71.4% with secondary progressive MS (SPMS), and 54.5% with relapsing remitting MS (RRMS). Among patients with improved or stabilized lower extremity motor function, PPMS recorded a mean 9% improvement, SPMS 3% and RRMS 6%. In PPMS, use of FES showed trend towards improvement in motor scores (P = 0.070).

Conclusions

FES as part of activity-based rehabilitation may help preserve or improve neurological function in patients with MS.     

Steroid-Responsive Myeloneuropathy Associated With Antithyroid Antibodies

Background/Objective:

To present information about 2 steroid-responsive, antithyroid antibody–positive patients with myeloneuropathy and myelopathy.

Methods:

Case reports.

Results:

A 48-year-old woman and a 42-year-old man presented with acute onset tetraparesis and magnetic resonance imaging studies showing cervical spinal lesions. Nerve conduction and biopsy studies of the woman were suggestive of a demyelinating polyradiculoneuropathy. Detailed diagnostic workup turned out to be negative for both patients, except for highly elevated antithyroid antibodies with normal thyroid functions and imaging. Both patients responded remarkably well to high-dose steroid treatment, and their symptoms disappeared in a few months. Both patients'' antithyroid antibody levels were reduced shortly after steroid treatment and in parallel with the amelioration of symptoms.

Conclusions:

Antithyroid antibodies might be associated with acute demyelinating myeloneuropathy or myelopathy pathogenesis and might indicate a good response to steroid treatment in these syndromes.     

Progressive thoracic myelopathy caused by spinal calcium pyrophosphate crystal deposition because of proximal junctional vertebral compression fracture after lumbopelvic fusion

Purpose

We describe cases presenting with progressive thoracic myelopathy after lumbopelvic fusion attributed to proximal junctional vertebral compression fracture (PJF) followed by spinal calcium pyrophosphate dehydrate (CPPD) crystal deposition.

Methods

The study included six patients, ranging from 62 to 75 years. All patients had been treated previously with lumbopelvic fusion. The mean period from the detection of PJF to the onset of myelopathy was 4.8 months. Notably, five patients demonstrated upper-instrumented vertebra (UIV) collapse.

Results

After revision surgery involving decompressive laminectomy and extension of the spinal fusion, all patients experienced significant improvement. Photomicrographs of the resected ligamentum flavum showed CPPD crystals and multinucleated giant cells.

Conclusions

The combination of mechanical stress plus PJF and CPPD crystal deposition followed by a foreign body reaction to the deposited crystals caused myelopathy. Patients with radiographic evidence of PJF, especially UIV collapse, after lumbopelvic fusion should be followed carefully for the emergence of myelopathy.     

Thoracic Myelopathy Secondary to Intradural Extramedullary Bronchogenic Cyst

Background/Objective:

To report a case of thoracic myelopathy secondary to intradural extramedullary bronchogenic cyst.

Study Design:

Case report.

Methods/Findings:

A 20-year-old man presented to the emergency department with increasing back pain and lower-extremity weakness. Magnetic resonance imaging demonstrated a cystic lesion at the T4 level with mass effect on the spinal cord.

Results:

The lesion was resected, and histopathologic evaluation showed a cyst lined by respiratory-type epithelium consistent with a bronchogenic cyst.

Conclusions:

Intradural extramedullary bronchogenic cysts of the thoracic spine have been reported previously but are extremely rare. The treatment of choice is surgical resection.     

Copper deficiency myelopathy: A report of two cases

Context

Copper deficiency myelopathy represents an often underdiagnosed, acquired neurological syndrome, clinically characterized by posterior column dysfunction. The main causes of copper deficiency are bariatric surgery, increased consumption of zinc, and malabsorption. However, even after a careful history taking and extensive laboratory researches, the etiology of copper deficiency remains undetermined in a significant percentage of cases. Patients affected by copper deficiency myelopathy usually present with sensory ataxia due to dorsal column dysfunction and sometimes with mild leg spasticity. In such patients, spinal cord magnetic resonance imaging (MRI) may show hyperintense lesions in T2-weighted sequences involving the posterior columns of cervical and thoracic cord. These MRI findings are not distinguishable from those of subacute combined degeneration associated with vitamin B12 deficiency.

Findings

Here, we describe two patients with gait ataxia and sensory symptoms in which a diagnosis of copper deficiency myelopathy was made. Both patients showed a significant clinical, neuroradiological, and neurophysiological improvement after proper supplementation therapy.

Conclusion

The patients herein described underline the importance to include serum copper and ceruloplasmin levels as part of the myelopathy diagnostic workup, especially in the cases of otherwise unexplained subacute myelopathy involving the posterior columns. Since copper deficiency myelopathy is a progressive syndrome, early diagnosis is mandatory in order to promptly provide a proper supplementation therapy and, thus, prevent an irreversible neurological damage.     

Cervical cord compressive myelopathy in a man with a primary complaint of knee pain

Background:

Diagnosing patients with cervical cord compressive myelopathy in a timely manner can be challenging due to varying clinical presentations, the absence of pathognomonic findings, and symptoms that are usually insidious in nature.

Objective:

To describe the clinical course of a patient with primary complaint of left medial knee pain that was nonresponsive to surgical and conservative measures; the patient was subsequently diagnosed with cervical cord compressive myelopathy.

Design:

Case report.

Subject:

A 63-year-old man with a primary complaint of left medial knee pain.

Findings:

Physical examination of the left knee was normal except for slight palpable tenderness over the medial joint line. During treatment, he noted loss of balance during activities of daily living. Reassessment revealed bilateral upper extremity hyperreflexia, bilateral Babinski reflex, and positive bilateral Hoffman reflex. Magnetic resonance imaging of the cervical spine demonstrated moderately severe spinal stenosis at the C3-C4, C5-C6, and C6-C7 levels. After C3-C7 laminoplasty for cervical cord compressive myelopathy, he reported substantial improvement of his left medial knee. Three years later, he had no complaint of knee pain.

Conclusion:

Appropriate diagnosis and treatment of cervical cord compressive myelopathy may avoid unnecessary diagnostic imaging, medical evaluations, invasive procedures, and potential neurologic complications.     

Total spondylectomy for solitary bone plasmacytoma of the lumbar spine in a young woman: a case report and review of literature

Abstract

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Introduction

Solitary bone plasmacytoma (SP) is a rare diagnosis for which the primary treatment is local radiotherapy. There is no established consensus suggesting a total spondylectomy in spinal SP.

Materials and Methods

We report the case of a 43-year-old woman with solitary plasmacytoma of the lumbar spine treated with complete vertebral resection. Radiographs, CT scan and MRI showed a single osteolytic lesion of the third lumbar vertebra. Further diagnostics following recommended algorithm for tumour screening were negative. Two times, biopsy showed no histological pathologies. Due to the instability of the spine with suspicious unknown lesion, we decided to perform a dorsal lumbar approach and instrumentation with complete resection of the posterior parts to prepare for a complete resection if mandatory. Resamples were taken and the bone surfaces sealed. Consecutive findings were positive for plasma cell infiltration of the respective vertebra, however not on the first pass, but after diagnostic pathological reference. Surgery was completed by total spondylectomy. Reference histological findings with restaging and cytogenetic risk analysis confirmed a non-high-risk solitary bone plasmacytoma, and the patient was scheduled for localized radiotherapy with 40 Gy.

Results

Follow-up examinations (53 months) showed no local recurrence or disease progression.

Discussion

There is no consensus in the literature regarding appropriate surgical approach and perioperative strategies in the treatment of solitary plasmacytoma. The finding of a solitary plasmacytoma of the spine was the determining factor for our decision to perform radical surgery with subsequent radiotherapy. The rationale for the chosen approach was to minimize the risk of local recurrence and to avoid conversion into multiple myeloma. The follow-up with 53 months is limited. However, discussion remains, if radical surgery in addition to local radiotherapy could be an alternative therapeutic approach depending on paraclinical parameters, age and cytogenetic risk analysis.     

Posterior Surgical Treatment of Cervical Spondylotic Myelopathy: Review Article

Background

Cervical spondylosis is now recognised as the leading cause of myelopathy and spinal cord dysfunction worldwide. Chronic spinal cord compression results in chronic inflammation, cellular apoptosis, and microvacular insufficiency, which are thought to the biologic basis for cervical spondylotic myelopathy (CSM).

Questions/Purposes

Our purpose was to address the key principles of CSM, including natural history and presentation, pathogenesis, optimal surgical approach, results and complication rates of posterior surgical approaches for CSM so that the rationale for addressing CSM by a posterior approach can be fully understood.

Methods

We conducted a systematic search of PubMed/MEDLINE and the Cochrane Collaboration Library for literature published through February 2014 to identify articles that evaluated CSM and its management. Reasons for exclusion included patients with ossification of the posterior longitudinal ligament (OPLL), patients with degenerative disc disease without CSM, and patients with spine tumor, trauma and infection. Meeting abstracts/proceedings, white articles and editorials were additionally excluded.

Results

The search strategy yielded 1,292 articles, which was reduced to 52 articles, after our exclusion criteria were introduced. CSM is considered to be a surgical disorder due to its progressive nature. There is currently no consensus in the literature whether multilevel spondylotic compression is best treated via an anterior or posterior surgical approach.

Conclusion

Multilevel CSM may be safely and effectively treated using a posterior approach, either by laminoplasty or with a laminectomy and fusion technique.

Electronic supplementary material

The online version of this article (doi:10.1007/s11420-014-9425-5) contains supplementary material, which is available to authorized users.     

A giant adrenal cyst difficult to diagnose except by surgery

Introduction

Adrenal cysts represent rare clinical entities. Although surgical indications are well defined, pitfalls arise from the failure to establish an accurate preoperative diagnosis. Cystic lesions of other abdominal organs especially the pancreas complicate the diagnostic field.

Presentation of case

We present the case of a giant adrenal cyst in a young female causing diagnostic dilemma. Imaging studies revealed a large cystic lesion of uncertain origin located between the spleen and the tail of the pancreas. It was decided to perform a laparotomy which confirmed the presence of an adrenal cyst and enucleation of the cyst was performed. Examination at one year confirmed no complications.

Discussion

Adrenal cysts should always be included in the differential diagnosis of cystic abdominal lesions.

Conclusion

When the preoperative diagnosis is uncertain, surgical intervention can be both diagnostic and therapeutic.     

Solitary plasmacytoma of L3 vertebral body treated by minimal access surgery: Common problem different solution!

Introduction

Solitary plasmacytoma of bone is a local primary bone tumour consisting of malignant plasma cells without systemic involvement.These tumours are known for large amount of blood loss, and the use of electrocautery is helpful in reducing blood loss and performing surgery in a relatively bloodless field; however, use of unipolar cautery in patients with indwelling cardiac pacemaker is known to cause arrhythmias and cardiac events.Minimally invasive techniques offer potential advantages over open techniques particularly in patients with spinal tumours, where massive amount of blood loss is expected, if open procedure is performed.Here, we present a case of solitary L3 plasmacytoma with progressive neurological deficit with chronic refractory anaemia with indwelling cardiac pacemaker treated by minimally invasive technique.

Materials and methods

A 71-year-old male presented with increasing back pain with Left L3 radiculopathy since 6 months and progressive left lower limb weakness since 5 days.The patient is a known case of chronic renal failure with chronic refractory anaemia. The patient has indwelling cardiac pacemaker for cardiac arrhythmias.Radiology was suggestive of L3 body plasmacytoma. L3 corpectomy and anterior column reconstruction with expandable cage and posterior stabilization by minimally invasive techniques were performed.

Results

Two years of follow-up showed no local recurrence. The patient is ambulatory unaided with no neurological deficit and backache.

Discussion

There is no consensus regarding appropriate surgical approach and perioperative strategies in treatment of solitary plasmacytoma. A solitary plasmacytoma was found in the spine of a patient with cardiac pacemaker where anaesthetic consideration, blood loss and the use of electrocautery were the limiting factors. Minimally invasive approach is a good option.     

Malignant fibrous histiocytoma presenting with complete opacification of the hemithorax: A case report

BACKGROUND

Malignant fibrous histiocytoma, a subtype of primary lung sarcoma is a very rare disease. It usually presents as a lung nodules and the final diagnosis is made by immunohistochemical studies.

METHODS

A 45-year-old patient presented with progressive dyspnea, dry cough and right shoulder pain. Chest X-ray revealed complete opacification of the right hemithorax. Chest computed tomography confirmed the presence of a heterogeneous lesion occupying the whole right hemithorax causing a mass effect on the trachea. Ultrasound guided biopsy was done and final pathology was suggestive of malignant fibrous histiocytoma.

CONCLUSION

Progressive dyspnea in young otherwise healthy patients should be investigated early on. In our case the presence of right shoulder pain indicates advance disease illustrated by the singular imaging findings.     

Anti-AQP(4) antibody in idiopathic acute transverse myelitis with recurrent clinical course: frequency of positivity and influence in prognosis

Background

The anti-aquaporin4 (anti-AQP4) antibody is specific for neuromyelitis optica (NMO), but is also found in limited forms. The presence of this antibody in acute transverse myelitis (ATM) has been associated with recurrence and conversion to NMO, but the influence on disability has not yet been described.

Objective

To describe the frequency of anti-AQP4 in ATM and analyze the influence in long-term prognosis.

Design

Cross-sectional and retrospective study.

Methods

Consecutive ATM cases in a multiple sclerosis center in Rio de Janeiro, Brazil, from 2000 through 2009 were reviewed. Recurrent cases tested for anti-AQP4 were selected. ATM with magnetic resonance imaging spinal cord lesions extending over three or more vertebral segments was classified as longitudinally extensive transverse myelitis (LETM); Kurtzke scale was applied at last evaluation.

Outcome measures

Frequency of anti-AQP4; severity of spinal cord dysfunction at last follow-up.

Results

Twenty six patients (21 female:5 male; 17 white:9 African descent) were studied. The first ATM occurred at 38.04 ± 12.7 years. The interval between the first and the second ATM was eight months (1–150) and the number of ATM varied from two to seven. After 40.5 months (12–192) of disease, the median Expanded Disability Status Scale (EDSS) score was three (0–9). Anti-AQP4 antibody was positive in 26.9%. LETM was found in 65.4%. LETM presented later onset, higher disability and higher positivity to anti-AQP4 (LETM 41.2% versus no-LETM 0%, P = 0.024). Dysfunction at long-term follow-up was similar in anti-AQP4 positive and negative cases.

Conclusion

The frequency of anti-AQP4 in recurrent ATM was 26.9%, increasing to 41.2% among LETM. Presence of the antibody had no influence on morbidity.     

Kienbock��s disease in a varsity football player: a case report and review of the literature

Objective:

To present the diagnostic, clinical features, and management of Kienbock’s disease and create awareness of the differential diagnosis of this condition in patients presenting with insidious, progressive dorsal wrist pain.

Clinical Features:

A 23-year old male varsity football player presented with insidious progressive dorsal sided wrist pain with reduced wrist flexion and extension. A diagnosis of Kienbock’s disease was made based on radiographs and magnetic resonance imaging.

Intervention and Outcome:

A 3mm ulnar-minus variance was found and a joint leveling procedure to shorten the radius was performed. Conservative therapy was provided pre and post surgical management.

Summary:

This case report demonstrates the importance of findings on radiographs, MRI, and clinical examination in the accurate diagnosis and management of a patient with wrist pain.     

Benign skull lesions

Objective

To document the epidemiologic and clinical features of benign skull lesions.

Design

A case series.

Setting

St. Michael’s Hospital, a tertiary care facility affiliated with the University of Toronto.

Patients

Thirty-one patients who had a neurosurgical consultation and were discharged from hospital after excision of a benign skull lesion during a 10-year period.

Main outcome measures

Patient demographics, clinical signs and symptoms, radiographic and pathological tumour characteristics, surgical procedure, length of hospital stay, outcome and follow-up.

Results

The 31 patients (6 men, 25 women) had 32 lesions excised. The mean age of the patients was 41.9 years. Osteomas accounted for 63% of the tumours. The most frequent location was the parietal bone. Neurologic symptoms were absent in the majority of calvarial tumours. Useful diagnostic studies included plain skull radiography and computed tomography. Nuclear bone scanning was done in 7 patients. All patients underwent craniectomy, with cranioplasty in most cases. Three patients had new neurologic symptoms postoperatively and 1 patient had incomplete resolution of symptoms.

Conclusions

Benign skull lesions are infrequent, but they require neurosurgical intervention. When necessary, surgical excision can serve to confirm the diagnosis, improve cosmesis and retard the progression of neurologic dysfunction. Of primary importance is the recognition of such lesions by primary care physicians and referral to the surgeon so that an appropriate treatment plan can be made.     

Duodenal seromyectomy in the management of adherent colonic carcinoma in elderly patients

Objective

To determine if partial denudation of the duodenum by seromyectomy can achieve tumour clearance in elderly patients with adherent primary colonic carcinoma.

Design

A case series.

Setting

An urban tertiary care centre.

Patients

Seven elderly patients with Dukes’ class C primary adenocarcinoma of the ascending colon adherent to the duodenum but without distant metastases. The follow-up ranged from 29 to 41 months.

Interventions

Right hemicolectomy and seromyectomy of the duodenum at the site of adhesion.

Main outcome measures

Patient survival and tumour recurrence.

Results

One patient died 29 months postoperatively of myocardial infarction but without tumour recurrence. Another patient had a solitary metastasis in the right liver lobe 7 months postoperatively. She was disease free 34 months after a right hemihepatectomy. The other 5 patients were alive and disease free at their last follow-up.

Conclusion

Duodenal seromyectomy with postoperative chemotherapy for locally advanced adherent colonic cancer seems to be an acceptable management strategy for elderly patients in whom major en bloc resections present a greater than average risk of death.     

Autonomic Dysreflexia in a Man With Multiple Sclerosis

Background/Objective:

To report manifestation of autonomic dysreflexia (AD) in a man with multiple sclerosis (MS).

Design:

Case report.

Findings:

A young man presented with a history of several admissions to the emergency department with complaints of hypertensive attacks, palpitations, difficulty in breathing, headaches, and flushing. The attacks were attributed to a previously diagnosed anxiety disorder. Onset of numbness of the left leg numbness prompted a more thorough study, which showed evidence of MS. AD was suspected as the cause of his recurrent attacks of hypertension. Bladder distension was identified as the cause of AD, and his hypertensive attacks were controlled by management of neurogenic bladder.

Conclusions:

This report emphasizes that AD can occur in MS. Somatic symptoms warrant thorough investigation before attributing them to psychosomatic causes.     

Considerations for Prophylactic Surgery in Asymptomatic Severe Cervical Stenosis: Review Article

Background

Cervical spondylotic myelopathy (CSM) is a devastating pathology that can severely impair quality of life. The symptoms in CSM progress slowly and often do not manifest until they become severe and potentially irreversible. There is a consensus that surgical intervention is warranted in symptomatic patients. The recovery of the neurologic deficit after surgical decompression of the spinal cord varies, and halting the progression of the disease remains the principle aim of surgery.

Questions/Purposes

The aim of this review is to address the key question of whether or not to intervene in cases that have radiographic evidence of significant cervical stenosis yet are asymptomatic or exhibit minimal symptoms?

Methods

The PubMed databases for publications that addressed asymptomatic cervical spondylotic myelopathy were reviewed. The relevant articles were selected after screening all the resulting abstracts. The references of the relevant articles were then reviewed, and cross references with titles discussing CSM were picked up for review.

Results

The search identified 14 papers which were reviewed. Seven articles were found to be relevant to the subject in question. Going through the references of the relevant articles, three articles were found to be directly related to the topic in study.

Conclusion

There is paucity of evidence to support for or against surgery in the setting of asymptomatic cervical spondylotic myelopathy despite radiographic evidence of severe stenosis. Patient factors such as age, level of activity, and risk of injury should be considered in formulating a management plan. Moreover, the patient should play an integral role in the process of decision making.

Electronic supplementary material

The online version of this article (doi:10.1007/s11420-014-9426-4) contains supplementary material, which is available to authorized users.     

Urological vignette: Satellite lesion in the bladder from urachal enteric adenocarcinoma

INTRODUCTION

We present, to the best of our knowledge, the first published case report of a satellite lesion within the bladder from enteric type urachal adenocarcinoma (UA).

PRESENTATION OF CASE

Our case report involves a 38-year-old man from the Solomon Islands who underwent open partial cystectomy for UA. However, resection margins were positive due to the novel finding of a satellite lesion on histopathological assessment. Salvage cystectomy was subsequently performed and the patient had an uncomplicated post-operative recovery.

DISCUSSION

This case highlights the importance of achieving negative soft tissue and bladder margins on initial resection of UA, as the consequences of incomplete resection can place significant additional morbidity on the patient.

CONCLUSION

We aim to highlight the possibility of satellite lesions within the bladder in UA and suggest that further studies looking at this phenomenon are required to establish its incidence and overall impact on management of UA.