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1.
R I Med J (2013) ; 107(2): 13-15, 2024 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-38285744

RESUMEN

Subacute combined degeneration (SCD) is an acquired neurologic complication from prolonged vitamin B12 deficiency. As a result of dorsal and lateral spinal cord column degeneration, patients present with a range of neurological symptoms, including paresthesias, ataxia, and muscle weakness. Without prompt treatment, irreversible nerve damage occurs. Here we present a young man who developed progressive ascending paresthesias and lower extremity weakness after escalated nitrous oxide use. This case highlights the importance of considering SCD from nitrous oxide toxicity when patients present with progressive ataxia, paresthesia, and lower extremity weakness.


Asunto(s)
Enfermedades de la Médula Espinal , Degeneración Combinada Subaguda , Deficiencia de Vitamina B 12 , Masculino , Humanos , Óxido Nitroso/efectos adversos , Parestesia/inducido químicamente , Parestesia/complicaciones , Vitamina B 12/uso terapéutico , Deficiencia de Vitamina B 12/inducido químicamente , Deficiencia de Vitamina B 12/complicaciones , Deficiencia de Vitamina B 12/diagnóstico , Degeneración Combinada Subaguda/complicaciones , Enfermedades de la Médula Espinal/complicaciones , Ataxia/complicaciones
2.
Neurologist ; 29(2): 113-119, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37839087

RESUMEN

INTRODUCTION: Nitrous oxide (NO) abuse is increasing among young people. This can result in severe neurological disorders such as myelopathy and/or peripheral neuropathy. We report the clinical presentations, biological, radiologic and electrophysiological findings of 5 patients hospitalized with neurological symptoms consecutive to NO abuse. In addition, a literature review was conducted to describe the neurological characteristics and to identify factors associated with a poor recovery. CASE REPORT: Among the 5 patients included, 2 had a myeloneuropathy, 2 had a sensorimotor neuropathy, and 1 had a normal spinal cord magnetic resonance imaging and electromyography despite neurological manifestations consistent with myeloneuropathy. After vitamin B 12 supplementation, recovery was reported in 4 patients, and 1 was lost to follow-up.From the literature review, 154 patients were included [94 males; median age 22 (19 to 26) y; NO exposure 9 (3 to 18) mo]. A myelopathy was identified in 116 patients (75%) and a peripheral neuropathy was documented in 89 patients (58%). Compared with patients who recovered, those with sequelae were more likely to have a motor deficit at presentation ( P <0.001), to use NO regularly ( P <0.001), to have a lower vitamin B 12 level ( P =0.04), and a higher concentration of homocysteine ( P =0.04). A less extensive myelopathy was more frequently found in the group with favorable outcomes ( P =0.002). CONCLUSION: Neurological disorders caused by NO may be challenging with severe clinical patterns. We identified several factors associated with a poor recovery, to make clinicians aware of NO-induced neurotoxicity.


Asunto(s)
Enfermedades del Sistema Nervioso , Enfermedades del Sistema Nervioso Periférico , Enfermedades de la Médula Espinal , Trastornos Relacionados con Sustancias , Masculino , Humanos , Adolescente , Adulto Joven , Adulto , Óxido Nitroso/efectos adversos , Vitamina B 12/efectos adversos , Enfermedades del Sistema Nervioso/inducido químicamente , Enfermedades de la Médula Espinal/inducido químicamente , Enfermedades de la Médula Espinal/diagnóstico por imagen , Enfermedades de la Médula Espinal/complicaciones , Trastornos Relacionados con Sustancias/complicaciones , Enfermedades del Sistema Nervioso Periférico/complicaciones
3.
Rehabilitacion (Madr) ; 57(4): 100810, 2023.
Artículo en Español | MEDLINE | ID: mdl-37399638

RESUMEN

We present the case of a 68-year-old man with progressive brachial and crural hypoaesthesia with gait ataxia suggesting subacute myelopathy of the posterior cords, demonstrated by MRI. After blood tests, a diagnosis of copper deficiency was made following zinc intoxication, secondary to the use of denture glue containing zinc. Treatment was started with copper and the dental glue was removed. Rehabilitation treatment was started with physiotherapy, hydrotherapy and occupational therapy. Functional improvement was achieved, going from an ASIAD level C4 to an ASIAD level C7 spinal cord injury. Copper levels should be studied in all non-compressive myelopathies of subacute onset if there is clear involvement of the posterior cords. Copper deficiency in analysis would establish the diagnosis. Rehabilitative treatment, supplementary copper supplementation and zinc withdrawal are essential to prevent irreversible neurological damage.


Asunto(s)
Enfermedades de la Médula Espinal , Traumatismos de la Médula Espinal , Masculino , Humanos , Anciano , Cobre , Zinc , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/diagnóstico , Traumatismos de la Médula Espinal/complicaciones
4.
Spinal Cord Ser Cases ; 8(1): 46, 2022 04 27.
Artículo en Inglés | MEDLINE | ID: mdl-35477695

RESUMEN

INTRODUCTION: Cervical spondylosis can predispose patients to central canal stenosis. In this setting, myelopathy through further flattening of the cord from extrinsic compression can be precipitated by relatively minor traumas. Arterial dissection is similarly considered a result of high velocity or momentum during trauma, commonly associated with fractures, cervical hyperflexion, or direct blunt force to the neck. Overall, precautions for both arterial dissection and myelopathy are rarely considered in low-velocity, static activities such as yoga. CASE PRESENTATION: The authors report the case of a 63-year-old man who suffered concurrent cervical myelopathy from multilevel spondylopathy, right vertebral artery dissection, and left cervical carotid artery dissection following a yoga session. Symptomatology consisted of acute onset neck pain, upper extremity sensory paresthesia, worsening gait and balance, and impaired dexterity for several weeks. Cervical MRI was obtained given myelopathic symptoms and revealed spondylosis with compression and T2 signal change at C3-C4. CT angiography of the neck revealed aforementioned dissections without flow limiting stenosis or occlusion. A therapeutic heparin infusion was started preoperatively until the patient underwent C3-C4 anterior cervical discectomy and fusion. Aspirin and Plavix were then started without incidence and the patient had significant but gradual improvement in myelopathic symptoms at 6-week follow-up. DISCUSSION: The static yet intensive poses associated with yoga present a rare etiology for arterial dissection and myelopathy, but patients with persistent and progressive symptoms should be screened with the appropriate imaging modality. Cervical decompression should be expedited before initiating an antiplatelet medication.


Asunto(s)
Enfermedades de la Médula Espinal , Traumatismos de la Médula Espinal , Espondilosis , Yoga , Constricción Patológica/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/cirugía , Traumatismos de la Médula Espinal/complicaciones , Espondilosis/complicaciones , Espondilosis/cirugía
5.
Neurol Sci ; 43(8): 5157-5159, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-35482156

RESUMEN

We present a case of a 23-year-old woman with a history of celiac disease who presented with a 2-month history of progressive gait unsteadiness and falls. Neurologic examination exhibited preserved motor strength, diffuse areflexia, and ataxic gait. Autoimmune and infectious workups were unremarkable, including vitamin B12. Electrodiagnostic testing showed absent diffuse sensory responses, consistent with sensory ganglionopathy. Total spine magnetic resonance imaging (MRI) revealed a non-enhancing, posterior cord, hyperintense signal from C1-T11. Partial improvement in her sensory ataxia was noted after 6 months of high-dose steroids without dorsal cord signals change on repeat MRI that suggests Wallerian degeneration of sensory axons.


Asunto(s)
Enfermedades de la Médula Espinal , Adulto , Femenino , Ataxia de la Marcha , Humanos , Imagen por Resonancia Magnética , Médula Espinal/diagnóstico por imagen , Médula Espinal/patología , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/diagnóstico por imagen , Vitamina B 12 , Adulto Joven
6.
Int Urol Nephrol ; 53(7): 1325-1330, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33743121

RESUMEN

PURPOSE: To compare the use of intraoperative ultrasound with X-ray fluoroscopy during sacral neuromodulation lead electrode placement in patients with neurogenic bladder secondary to spinal cord disease. METHODS: We reviewed the medical records of 52 patients who underwent sacral neuromodulation (SNM) lead electrode implantation under fluoroscopy or ultrasound guidance from July 2016 to July 2019. The operating time, number of electrode contacts with stimulus responses, minimum voltage that causes a stimulus response, and rate of standard lead electrode placement were used to assess the differences between the two methods. All patients were evaluated by recording bladder diaries, postvoid residual volumes before and during the testing period. Permanent SNM implantation is acceptable if symptoms improve by at least 50%. RESULTS: The operating time decreased from 87.1 ± 25.19 min in the X-ray group to 68.2 ± 25.20 min (p < 0.05) in the ultrasound group. The number of electrode contacts with stimulus responses, rate of standard lead electrode placement, and implantable pulse generator (IPG) placement rate were not significantly different between the two groups (p > 0.05). There was no radiation exposure during the operation in the ultrasound group. No incisional infections, hematomas, or other critical complications were reported in either groups. CONCLUSION: Ultrasound can be applied to safely place lead electrode for sacral neuromodulation and leads to no radiation exposure to the patient, surgeon, and operating room staff and a shortened operating time while maintaining the same efficacy as X-ray.


Asunto(s)
Terapia por Estimulación Eléctrica/métodos , Electrodos Implantados , Implantación de Prótesis , Vejiga Urinaria Neurogénica/terapia , Adulto , Anciano , Femenino , Fluoroscopía , Humanos , Periodo Intraoperatorio , Plexo Lumbosacro , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Enfermedades de la Médula Espinal/complicaciones , Cirugía Asistida por Computador , Ultrasonografía , Vejiga Urinaria Neurogénica/etiología
7.
J Radiol Case Rep ; 14(4): 21-37, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33082920

RESUMEN

Cervical spondylolisthesis indicates instability of the spine and can lead to pain, radiculopathy, myelopathy and vertebral artery stenosis. Currently degenerative cervical spondylolisthesis is a wait-and-watch condition with no treatment guidelines. A literature review and discussion will be provided. 8 females presented with neck pain, disability, and history of motor vehicle collision. Radiographs revealed abnormal cervical alignment, spinal canal narrowing, and spondylolistheses. After 30 sessions of Chiropractic BioPhysics® care over 12 weeks, patients reported improved symptoms and disabilities. Radiographs revealed improvements in cervical alignment, spondylolistheses, and spinal canal diameter. Motor vehicle collision may cause instability and abnormal alignment of the cervical spine leading to cervical spondylolisthesis. Improving spinal alignment may be an effective treatment to reduce vertebral subluxation and cervical spondylolistheses and improve neck disability as a result of improved spinal alignment.


Asunto(s)
Lordosis , Estenosis Espinal , Espondilolistesis , Biofisica , Vértebras Cervicales , Quiropráctica , Femenino , Humanos , Persona de Mediana Edad , Cuello , Dolor de Cuello , Radiculopatía , Radiografía , Canal Medular , Enfermedades de la Médula Espinal/complicaciones , Estenosis Espinal/diagnóstico por imagen , Espondilolistesis/complicaciones
8.
Urology ; 142: 237-242, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32320790

RESUMEN

OBJECTIVES: To evaluate an alternative to clean intermittent catheterization (CIC) for individuals with neurogenic bladder for its effects on independence, privacy, and convenience. This prospective cohort study provides an initial assessment of quality of life, safety, and efficacy of closed diurnal indwelling catheterization (CDIC). MATERIALS AND METHODS: Individuals with spinal cord disorders using CIC were prospectively screened at multidisciplinary clinic appointments. During the 24-week intervention, a foley was placed each morning and capped between scheduled bladder drainage each 3-4 hours. After a maximum of 8 hours of CDIC use, CIC was resumed. Quality of life outcome measures (the Short Form Health Survey, King's Health Questionnaire, and Pediatric Quality of Life InventoryTM (PedsQL) questionnaires), clinic evaluations, labs, imaging, and urodynamics were obtained at specified interval visits planned after 4-, 12-, and 24 weeks of study participation and compared to baseline. RESULTS: A total of 11 subjects enrolled; 8 completed the 24-week intervention. No significant difference with CDIC was observed in the Short Form Health Survey or PedsQL summary scores as compared to baseline. For the King's Health Questionnaire, physical limitations secondary to bladder function decreased significantly from baseline to the 4-week and 12-week (P = .02) but not 24-week visits. All 8 subjects who completed the 24-week intervention requested continued use. Early discontinuation occurred in 3 male participants due to urethral trauma (1) and incontinence (2). No increase in bacteriuria, urinary tract infections, or renal anatomic changes was observed. CONCLUSION: This prospective study demonstrates that CDIC may be safe and effective for short-term use. This alternative to CIC for scheduled daytime bladder drainage for neurogenic bladder warrants further consideration.


Asunto(s)
Catéteres de Permanencia , Enfermedades de la Médula Espinal/complicaciones , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Neurogénica/terapia , Cateterismo Urinario/instrumentación , Adolescente , Catéteres de Permanencia/efectos adversos , Niño , Diseño de Equipo , Femenino , Humanos , Masculino , Proyectos Piloto , Estudios Prospectivos , Calidad de Vida , Resultado del Tratamiento , Cateterismo Urinario/efectos adversos , Adulto Joven
9.
Am J Emerg Med ; 37(6): 1216.e3-1216.e5, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-30940410

RESUMEN

The frequency of stroke mimics among stroke patients has been reported to be up to 30%, and that in patients who receive thrombolytic therapy ranges between 1% and 16%. Atlantoaxial dislocation with myelopathy mimicking stroke is extremely rare. An 83-year-old man with a history of old cerebellar infarction presented to the emergency department with acute left hemiplegia after a chiropractic manipulation of the neck and back several hours before symptom onset. Mild hypoesthesia was observed on his left limbs. No speech disturbance, facial palsy, or neck or shoulder pain was observed. Intravenous thrombolytic treatment was given 238 min after symptom onset. Brown-Sequard syndrome subsequently developed 6 h after thrombolysis with a hypoesthetic sensory level below the right C5 dermatome. An emergent brain magnetic resonance angiography did not reveal an acute cerebral infarct but rather an atlantoaxial dislocation causing upper cervical spinal cord compression. Clinical symptoms did not deteriorate after thrombolysis. He received successful decompressive surgery 1 week later, and his muscle power gradually improved, with partial dependency when performing daily living activities 2 months later. A literature review revealed that only 15 patients (including the patient mentioned here) with spinal disorder mimicking acute stroke who received thrombolytic therapy have been reported. Atlantoaxial dislocation may present as acute hemiplegia mimicking acute stroke, followed by Brown-Sequard syndrome. Inadvertent thrombolytic therapy is likely not harmful for patients with atlantoaxial dislocation-induced cervical myelopathy. The neurological deficits of patients should be carefully and continuously evaluated to differentiate between stroke and myelopathy.


Asunto(s)
Vértebras Cervicales/diagnóstico por imagen , Fibrinolíticos/uso terapéutico , Luxaciones Articulares/diagnóstico por imagen , Traumatismos del Cuello/complicaciones , Enfermedades de la Médula Espinal/complicaciones , Anciano de 80 o más Años , Encéfalo/diagnóstico por imagen , Isquemia Encefálica , Vértebras Cervicales/cirugía , Descompresión Quirúrgica , Errores Diagnósticos , Humanos , Luxaciones Articulares/cirugía , Angiografía por Resonancia Magnética , Masculino , Accidente Cerebrovascular
10.
Medicine (Baltimore) ; 97(49): e13541, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30544463

RESUMEN

RATIONALE: Nocardial spinal epidural abscess is rare. The diagnosis is often difficult to make and, if delayed, poses a high risk of long-term disability. Nocardial spinal epidural abscess with severe lumbar disc herniation has not previously been reported. PATIENT CONCERNS: A 50-year-old man presented with progressive lumbago and leg pain for 6 weeks after receiving acupuncture therapy, and then the patient suddenly occurred urine retention after walking. DIAGNOSES: Clinical examination revealed sign of cauda equina syndrome. Magnetic resonance imaging (MRI) revealed a Lumbar(L)4 to L5 disc herniation, L3 to Sacrum(S)1 epidural abscess, and L2 to S1 paravertebral abscess. The causative organism was Nocardia farcinica. INTERVENTIONS: An urgent paravertebral abscess debridement and right L4 to L5 laminectomy were performed. Simultaneously, the disc tissue protruding into the spinal canal was removed, as well as irrigation and drainage. And antimicrobial treatment was continued for 12 months. OUTCOMES: Fortunately, the patient was able to walk with a cane and urinate autonomously without a catheter, although this remained difficult 7 days after surgery. After 1 year of treatment, the patient has recovered completely and returned to work. LESSONS: Nocardial spinal epidural abscess with severe lumbar disc herniation is extremely rare. Pain from spinal degenerative diseases often masks the early symptoms of spinal infection. It's worth noting that invasive treatment of spine is a way of causing spinal nocardial infection.


Asunto(s)
Infecciones Bacterianas del Sistema Nervioso Central/complicaciones , Absceso Epidural/complicaciones , Degeneración del Disco Intervertebral/complicaciones , Desplazamiento del Disco Intervertebral/complicaciones , Nocardiosis/complicaciones , Nocardia , Enfermedades de la Médula Espinal/complicaciones , Infecciones Bacterianas del Sistema Nervioso Central/diagnóstico por imagen , Infecciones Bacterianas del Sistema Nervioso Central/terapia , Absceso Epidural/diagnóstico por imagen , Absceso Epidural/terapia , Humanos , Degeneración del Disco Intervertebral/diagnóstico por imagen , Degeneración del Disco Intervertebral/terapia , Desplazamiento del Disco Intervertebral/diagnóstico por imagen , Desplazamiento del Disco Intervertebral/terapia , Vértebras Lumbares/diagnóstico por imagen , Vértebras Lumbares/cirugía , Masculino , Persona de Mediana Edad , Nocardiosis/diagnóstico por imagen , Nocardiosis/terapia , Enfermedades de la Médula Espinal/diagnóstico por imagen , Enfermedades de la Médula Espinal/terapia
11.
Intern Med ; 56(1): 105-108, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28049986

RESUMEN

A patient with chronic alcoholism presented with myelopathy and low serum folate and cobalamin levels. A 42-year-old alcoholic man had gait disturbance for 4 months. A neurological examination revealed marked spasticity with increased deep tendon reflexes and extensor plantar responses of the lower limbs. His cobalamin level was decreased and his serum folate level was particularly low. His plasma ammonia level was not increased. Abstinence and folic acid and cobalamin supplementation stopped the progression of his neurological deficits. This case indicates that nutritional deficiency should be monitored closely in patients with chronic alcoholism who present with myelopathy.


Asunto(s)
Alcoholismo/complicaciones , Suplementos Dietéticos , Ácido Fólico/sangre , Desnutrición/etiología , Desnutrición/prevención & control , Enfermedades de la Médula Espinal/complicaciones , Vitamina B 12/sangre , Adulto , Humanos , Masculino
12.
J Spinal Cord Med ; 40(4): 489-491, 2017 07.
Artículo en Inglés | MEDLINE | ID: mdl-27841075

RESUMEN

CONTEXT: Acquired copper deficiency represents a rare cause of progressive myelopathy presenting with sensory ataxia and spastic gait. The time interval from neurological symptoms onset to diagnosis of myelopathy ranges from 2 months to several years in almost all cases, mimicking the clinical course of subacute combined degeneration due to vitamin B12 deficiency. FINDINGS: A 60-year-old man, without any gastrointestinal symptoms, developed over the course of one week rapidly progressive gait imbalance, tingling and numbness in his feet and ascending lower limb weakness. Spine magnetic resonance imaging revealed hyperintensity involving cervical and dorsal posterior columns of spinal cord. Blood analysis revealed undetectable serum copper levels, low serum ceruloplasmin and positive serum Immunoglobulin A anti-tissue transglutaminase. Upper gastrointestinal endoscopy was performed revealing duodenal villous atrophy consistent with a malabsorption pattern. A gluten-free diet in association with intravenous then oral copper supplementation prompted sustained normalization of serum copper levels and progressive clinical improvement. CONCLUSION/CLINICAL RELEVANCE: We report a rare case of myelopathy induced by copper deficiency secondary to undiagnosed celiac disease, peculiarly presenting with a subacute onset. This case expands the neurological presentation and clinical course of myelopathy due to acquired copper deficiency. We suggest investigation of copper deficiency in patients presenting with subacute or even acute sensory ataxia and spastic gait. Detection of hypocupremia in patients without a previous history of gastric surgery should lead to diagnostic testing for celiac disease even in the absence of any obvious gastrointestinal symptoms.


Asunto(s)
Enfermedad Celíaca/complicaciones , Cobre/deficiencia , Enfermedades de la Médula Espinal/etiología , Enfermedad Celíaca/diagnóstico , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/diagnóstico
13.
J R Coll Physicians Edinb ; 46(3): 160-162, 2016 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-27959349

RESUMEN

Nitrous oxide is commonly used as an analgesic and anaesthetic agent. Nitrous oxide is also in use in industry as an aerosol propellant and is now recognised as a recreational drug whose use is growing, especially among the young. Nitrous oxide from whipped cream canisters is inhaled to produce a dissociative, intoxicated state. Nitrous oxide is known to inactivate vitamin B12 via oxidation, which can precipitate a demyelinating myelopathy akin to the classical B12 deficiency syndrome, subacute combined degeneration of the spinal cord. This case describes a young woman with chronic pain and a poor nutritional state who took regular nitrous oxide as an opiate-sparing agent. She developed a progressive subacute myelopathy with a sensory level, profoundly impaired joint position sense, extensor plantars and required a wheelchair. Once diagnosed, she responded well to a regime of nitrous oxide withdrawal, high-dose B12 replacement and physiotherapy. The case illustrates the need for clinical teams to be able to dentify a nitrous oxide-precipitated myelopathy as its use as a drug of abuse increases; particularly in the case of malnourished patients who receive nitrous oxide surgically or obstetrically.


Asunto(s)
Accidentes por Caídas , Analgésicos no Narcóticos/efectos adversos , Dolor Crónico/tratamiento farmacológico , Enfermedades Desmielinizantes/inducido químicamente , Óxido Nitroso/efectos adversos , Enfermedades de la Médula Espinal/inducido químicamente , Vitamina B 12/metabolismo , Adulto , Analgésicos no Narcóticos/uso terapéutico , Anestésicos por Inhalación/efectos adversos , Anestésicos por Inhalación/uso terapéutico , Enfermedades Desmielinizantes/complicaciones , Enfermedades Desmielinizantes/metabolismo , Femenino , Humanos , Óxido Nitroso/uso terapéutico , Manejo del Dolor/efectos adversos , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/metabolismo , Deficiencia de Vitamina B 12
14.
Pediatr Neonatol ; 57(5): 431-435, 2016 10.
Artículo en Inglés | MEDLINE | ID: mdl-24486081

RESUMEN

Maple syrup urine disease (MSUD) is an autosomal recessive aminoacidopathy secondary to an enzyme defect in the catabolic pathway of the branched-chain amino acids (BCAAs: leucine, isoleucine, and valine). Accumulation of their corresponding keto-acids leads to encephalopathy if not treated in time. A newborn male patient was suspected to have MSUD after tandem mass study when he presented symptoms and signs suggestive neonatal sepsis, anemia, and diarrhea. Food restriction of BCAAs was started; however, acrodermatitis enteropathica-like skin eruptions occurred at age 2 months. The skin rashes resolved after adding BCAAs and adjusting the infant formula. At age 7 months, he suffered from recurrent skin lesions, zinc deficiency, osteoporosis, and kyphosis of the thoracic spine with acute angulation over the T11-T12 level associated with spinal compression and myelopathy. After supplementation of zinc products and pamidronate, skin lesions and osteopenia improved gradually. Direct sequencing of the DBT gene showed a compound heterozygous mutation [4.7 kb deletion and c.650-651insT (L217F or L217fsX223)]. It is unusual that neurodegeneration still developed in this patient despite diet restriction. Additionally, brain and spinal magnetic resonance imaging, bone mineral density study, and monitoring of zinc status are suggested in MSUD patients.


Asunto(s)
Enfermedad de la Orina de Jarabe de Arce/complicaciones , Enfermedad de la Orina de Jarabe de Arce/diagnóstico , Enfermedades de la Médula Espinal/diagnóstico , Curvaturas de la Columna Vertebral/diagnóstico , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Enfermedad de la Orina de Jarabe de Arce/genética , Mutación , Enfermedades de la Médula Espinal/complicaciones , Curvaturas de la Columna Vertebral/complicaciones
15.
Neuropeptides ; 48(6): 371-8, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25455106

RESUMEN

The aim of the present study was to evaluate the therapeutic potential of local capsaicinoid (EMSPOMA(®) cream) treatment on chronic low back pain in patients with degenerative spine diseases and to investigate the possible mechanism of action of the therapy. The qualitative and quantitative analyses of capsaicinoids in EMSPOMA(®) cream were performed by liquid chromatography-tandem mass spectrometry (LC-MS/MS). In the clinical study 20 patients with degenerative spine diseases were involved in a self-controlled examination. During the 21 day therapy they received 30 min daily treatment with capsaicinoid (EMSPOMA(®)) cream to the lumbar region of the back. The pain (VASs, Oswestry Disability Index) and the mobility of the lumbar region of the spine (Schober's, Domján's L and R test) were detected at baseline and at the end of the 1st, 2nd and 3rd weeks. The plasma level of somatostatin-like immunoreactivity (SST-LI) was measured by radioimmunoassay (RIA) before and after the treatment on the first and the last day of the therapy. Nonivamide (0.01%) was identified as the only capsaicinoid molecule in the cream. In the clinical study the 21 day local nonivamide treatment reduced the pain sensation. Oswestry Disability Index decreased from 39 ± 3.9% to 32.5 ± 4.4%. VASs showed 37.29%-59.51% improvement. In the plasma level of SST-LI threefold elevation was observed after the first nonivamide treatment. We conclude that nonivamide treatment exerts analgesic action in chronic low back pain and causes the release of the antinociceptive and anti-inflammatory neuropeptide somatostatin which may play pivotal role in the pain-relieving effect.


Asunto(s)
Analgésicos/administración & dosificación , Capsaicina/análogos & derivados , Dolor de la Región Lumbar/tratamiento farmacológico , Somatostatina/sangre , Administración Cutánea , Anciano , Capsaicina/administración & dosificación , Femenino , Humanos , Dolor de la Región Lumbar/etiología , Masculino , Persona de Mediana Edad , Dimensión del Dolor , Enfermedades de la Médula Espinal/complicaciones
16.
J Neurol Sci ; 336(1-2): 273-5, 2014 Jan 15.
Artículo en Inglés | MEDLINE | ID: mdl-24215944

RESUMEN

BACKGROUND: Folate deficiency is known to be associated with subacute combined degeneration of the spinal cord; however, reports of long-standing cases are rare. Although neurological deficits due to folate deficiency have been reported to respond to folic acid supplementation, the functional outcomes have not been fully elucidated. OBJECTIVE: The aim of the study was to evaluate the clinical features and response to folate supplementation in a patient with folate deficiency manifested over 10 years as a slowly progressive myelopathy. METHODS: We performed comprehensive clinical screening, electrophysiological testing, and posturography before and after folate supplementation. RESULTS: A 49-year-old man had a slowly progressive gait disturbance for 10 years. He had not eaten fresh green vegetables for more than 10 years. Neurological examination revealed spastic paraplegia and absence of any vibration sense in the lower limbs accompanied by a positive Romberg's sign. Serum folate level was low, and plasma homocysteine level was elevated. Levels of blood thiamine and serum cobalamin were normal. We diagnosed the patient with myelopathy due to folate deficiency. Folic acid supplementation led to improvement of his symptoms; posturography and walking speed tests showed partial improvement, while the somatosensory-evoked potentials and central motor conduction time remained unchanged. CONCLUSIONS: Folate deficiency should be considered as a differential diagnosis of chronic slowly progressive myelopathy. The present case suggests the importance of early diagnosis and treatment before the adverse neurological manifestations of folate deficiency become irreversible.


Asunto(s)
Progresión de la Enfermedad , Deficiencia de Ácido Fólico/complicaciones , Deficiencia de Ácido Fólico/diagnóstico , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/diagnóstico , Humanos , Masculino , Persona de Mediana Edad
17.
Spinal Cord ; 51(7): 571-8, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23628893

RESUMEN

OBJECTIVES: To compare the efficacy of intravesical electrostimulation (IVES) versus sacral neuromodulation (SNM) in patients with incomplete spinal cord lesions (SCL) and neurogenic non-obstructive urinary retention (N-NOR). METHODS: In this retrospective study, 77 N-NOR patients underwent IVES (minimum 28 sessions), then after returning to voiding baseline symptoms, percutaneous first stage of SNM (lasting for minimum 4 weeks). After the two neuromodulation treatments, responders were categorized as patients experiencing both a 50% reduction of volume per catheterization per ml and a 50% reduction in number of catheterizations per day when comparing the 7-day voiding diaries at the end of both procedures to baselines. New urodynamics were performed subsequently. Responders to first stage of SNM underwent permanent SNM. RESULTS: Forty-eight patients responded to neither of the treatments, whereas 29 responded to both IVES and first-stage SNM. No significant statistical differences (P>0.05) were detected in the voiding diaries. Following the two procedures, the first sensation of bladder filling was either maintained or recovered by all responders, whereas the same 11 patients reached a bladder contractility index of >100. The 29 IVES responders lost their clinical benefits in a mean follow-up of 9.6 months. Only 10 out of the 29 patients became nonresponsive to permanent SNM, in a mean follow-up of 54 months. CONCLUSION: A strict correlation in terms of clinical and urodynamic patterns was demonstrated in patients with incomplete SCL and N-NOR, following IVES and first stage of SNM. However, voiding improvement through IVES was short-term when compared with the effects of permanent SNM.


Asunto(s)
Terapia por Estimulación Eléctrica/métodos , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/rehabilitación , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Neurogénica/rehabilitación , Retención Urinaria/etiología , Retención Urinaria/rehabilitación , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Sacro/inervación , Enfermedades de la Médula Espinal/diagnóstico , Resultado del Tratamiento , Obstrucción Ureteral/diagnóstico , Obstrucción Ureteral/etiología , Obstrucción Ureteral/rehabilitación , Vejiga Urinaria/inervación , Vejiga Urinaria Neurogénica/diagnóstico , Retención Urinaria/diagnóstico
18.
J Neurol Sci ; 329(1-2): 11-6, 2013 Jun 15.
Artículo en Inglés | MEDLINE | ID: mdl-23562297

RESUMEN

BACKGROUND: Vitamin B12 deficiency is a well recognized cause of posterolateral myelopathy. In Indian subcontinent, it may coexist with nutritional copper deficiency producing partial response of patients to B12 supplementation. Hence the study was planned to look for association of hypocupremia and B12 deficiency. METHODS: Twenty-three patients with posterolateral myelopathy (Romberg sign positive) were enrolled and investigated for levels of vitamin B12, copper and zinc and followed up for six months. RESULT: In three patients, copper deficiency alone was found to be the cause. In another three, both copper and vitamin B12 were deficient. In all these six patients, ceruloplasmin and 24h urinary copper were found to be low suggesting dietary copper deficiency. Hyperzincemia was found in four of these patients. Magnetic resonance imaging of spine was normal in lone Cu deficient patients but showed T2 hyperintensity of posterior column in lone B12 or combined B12 and copper deficiency. CONCLUSION: In cases of B12 deficiency myelopathy not responding to supplementation, copper deficiency must be sought at the earliest to avoid and treat persistent neurological disability.


Asunto(s)
Ataxia/complicaciones , Cobre/sangre , Enfermedades de la Médula Espinal/sangre , Enfermedades de la Médula Espinal/complicaciones , Vitamina B 12/sangre , Zinc/sangre , Potenciales de Acción/fisiología , Adulto , Análisis de Varianza , Ataxia/sangre , Encéfalo/patología , Evaluación de la Discapacidad , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Médula Espinal/patología , Estadísticas no Paramétricas , Deficiencia de Vitamina B 12/complicaciones
19.
Acta Neurol Taiwan ; 20(2): 129-37, 2011 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-21739392

RESUMEN

PURPOSE: Case reports with a comprehensive review of the current literature concerning subacute combined degeneration induced by nitrous oxide inhalation. A differential diagnosis should be considered when young patients present with progressive myelopathy because that the misuse of nitrous oxide has potentially serious outcomes. CASES REPORT: Three young patients aged from 18 to 24, one male and two females, were diagnosed with progressive ascending numbness in four limbs or both legs and ataxia. They all had been inhaling nitrous oxide from whipped-cream containers for several months. A cervicothoracic magnetic resonance imaging scan revealed long segmental hyperintensity changes at the posterior column of the spinal cord. Serological examination showed a low level of vitamin B12. Subacute combined degeneration of the spinal cord was diagnosed and the etiology was considered related to nitrous oxide misuse. Their neurological status, neuroimage, and neurophysiologic condition improved after vitamin B12 supplementation and cessation of nitrous oxide inhalation. CONCLUSION: Iatrogenic usage of nitrous oxide apparently resulted in subacute combined degeneration in our three patients. Recently, nitrous oxide misuse has increased among young people. Subacute combined degeneration of the spinal cord should be considered as a possible outcome of such abuse.


Asunto(s)
Óxido Nitroso/envenenamiento , Enfermedades de la Médula Espinal/inducido químicamente , Degeneración Combinada Subaguda/inducido químicamente , Adolescente , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Enfermedades de la Médula Espinal/complicaciones , Degeneración Combinada Subaguda/complicaciones , Vitamina B 12/metabolismo , Adulto Joven
20.
Brain ; 134(Pt 5): 1277-92, 2011 May.
Artículo en Inglés | MEDLINE | ID: mdl-21490053

RESUMEN

Although cervical spondylotic myelopathy is a common cause of chronic spinal cord dysfunction in humans, little is known about the molecular mechanisms underlying the progressive neural degeneration characterized by this condition. Based on animal models of cervical spondylotic myelopathy and traumatic spinal cord injury, we hypothesized that Fas-mediated apoptosis and inflammation may play an important role in the pathobiology of human cervical spondylotic myelopathy. We further hypothesized that neutralization of the Fas ligand using a function-blocking antibody would reduce cell death, attenuate inflammation, promote axonal repair and enhance functional neurological outcomes in animal models of cervical spondylotic myelopathy. We examined molecular changes in post-mortem human spinal cord tissue from eight patients with cervical spondylotic myelopathy and four control cases. Complementary studies were conducted using a mouse model of cervical spondylotic myelopathy (twy/twy mice that develop spontaneous cord compression at C2-C3). We observed Fas-mediated apoptosis of neurons and oligodendrocytes and an increase in inflammatory cells in the compressed spinal cords of patients with cervical spondylotic myelopathy. Furthermore, neutralization of Fas ligand with a function-blocking antibody in twy/twy mice reduced neural inflammation at the lesion mediated by macrophages and activated microglia, glial scar formation and caspase-9 activation. It was also associated with increased expression of Bcl-2 and promoted dramatic functional neurological recovery. Our data demonstrate, for the first time in humans, the potential contribution of Fas-mediated cell death and inflammation to the pathobiology of cervical spondylotic myelopathy. Complementary data in a murine model of cervical spondylotic myelopathy further suggest that targeting the Fas death receptor pathway is a viable neuroprotective strategy to attenuate neural degeneration and optimize neurological recovery in cervical spondylotic myelopathy. Our findings highlight the possibility of medical treatments for cervical spondylotic myelopathy that are complementary to surgical decompression.


Asunto(s)
Apoptosis/fisiología , Vértebras Cervicales/patología , Inflamación/etiología , Enfermedades de la Médula Espinal/complicaciones , Osteofitosis Vertebral/complicaciones , Receptor fas/metabolismo , Proteína de la Poliposis Adenomatosa del Colon/metabolismo , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Animales , Anticuerpos/uso terapéutico , Peso Corporal , Caspasa 9/metabolismo , Cicatriz/etiología , Modelos Animales de Enfermedad , Proteína Ligando Fas/inmunología , Femenino , Proteína Ácida Fibrilar de la Glía/metabolismo , Humanos , Etiquetado Corte-Fin in Situ/métodos , Inflamación/tratamiento farmacológico , Masculino , Ratones , Ratones Endogámicos ICR , Ratones Mutantes , Persona de Mediana Edad , Neuronas/metabolismo , Neuronas/patología , Oligodendroglía , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo , Desempeño Psicomotor , Enfermedades de la Médula Espinal/tratamiento farmacológico , Osteofitosis Vertebral/tratamiento farmacológico , Factores de Tiempo
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