Optic Neuropathy and Myelopathy in a Teenager With Biotinidase Deficiency.

ABSTRACT: A 19-year-old man presented with 3 years of gradually progressive, painless vision loss in both eyes. The ophthalmic examination showed bilateral diminished visual acuity, dyschromatopsia, and temporal optic nerve pallor. The neurological examination was consistent with a mild myelopathy with decreased pin-prick sensation starting at T6-T7 and descending through the lower extremities. Hyperreflexia was also present in the lower more than upper extremities. Infectious, inflammatory, and nutritional serum workup and cerebrospinal fluid analysis were both unrevealing. MRI of the brain and spinal cord showed abnormal T2 hyperintensity of the fornix, corpus callosum, optic nerves, and lateral columns of the cervical and thoracic spine, with diffusion restriction in the inferior-posterior corpus callosum and fornix. Biotinidase serum enzyme activity was tested and showed a decreased level of activity. Biotinidase gene testing showed a homozygous pathogenic variant, c.424C>A (p.P142T), confirming the diagnosis of biotinidase deficiency and prompting oral biotin supplementation. Three months after starting treatment, the patient's visual acuity, color vision, visual fields, and MRI spine abnormalities all improved significantly. Biotinidase deficiency is an important diagnostic consideration in patients with unexplained optic neuropathy and/or myelopathy.

Neurological complications due to copper deficiency in the context of Wilson disease treatment: a case report with long-term follow-up and review of the literature.

The objective is to investigate the presentation, complications, management, and outcomes of copper deficiency-induced neurological pathologies due to Wilson disease (WD) overtreatment. We examined the case of a WD patient who developed a low thoracic dorsal myelopathy due to chronic hypocupremia from excessive zinc therapy. A comprehensive literature review was conducted to identify similar cases. Ten additional cases of neurological pathology resulting from copper deficiency in the context of WD over-treatment were identified, all occurring during therapy with zinc salts. Myelopathy and peripheral neuropathy were the most common complications, while two additional groups reported leukoencephalopathy. Early cytopenia was often associated with copper deficiency-related neurological pathology appearing early in the context of copper deficiency. WD patients undergoing treatment, especially with zinc salts, should be closely monitored to prevent over-treatment and the consequent copper deficiency. Regular complete blood counts could provide early detection of copper deficiency, avoiding irreversible neurological damage. Swift recognition of new neurological signs not consistent with WD and timely discontinuation of the decoppering therapy are critical for improving outcomes. The optimal management, including the potential benefit of copper supplementation in patients with WD and subsequent therapy adjustments, remains unclear and necessitates further investigation. Despite the general poor functional neurological outcomes, there were some exceptions that warrant further exploration.

Brain stem and spinal cord demyelination due to acute carbon monoxide poisoning.

Demyelination throughout the brain stem and spinal cord caused by acute carbon monoxide (CO) poisoning has not been previously reported. Magnetic resonance imaging (MRI) has revealed that acute CO poisoning primarily affects the subcortical white matter of the bilateral cerebral hemispheres and basal ganglia. Here we report the case of a patient with delayed neuropsychological sequelae (DNS) due to acute CO poisoning. A 28-year-old man was admitted to our department following a suicide attempt by acute CO poisoning. After a six-month pseudo-recovery period, he was diagnosed with DNS, with MRI evidence of demyelinating change of the bilateral cerebral peduncles. Demyelination was identified throughout the brain stem, expanding from the bilateral cerebral peduncles to the medulla oblongata, occurring approximately six months after poisoning. One and a half years after acute CO poisoning, demyelination of the cervical and thoracic spine was observed, most notable in the lateral and posterior cords. It is evident that previously published research on this topic is extremely limited. Perhaps in severe cases of acute CO poisoning the fatality rate is higher, leading to fewer surviving cases for possible study. This may be because a more severe case of acute CO poisoning would result in the higher likelihood of secondary demyelination. This research indicates that clinicians should be aware of the risk of secondary demyelination and take increased precautions such as vitamin B supplementation and administration of low-dose corticosteroids for an extended period of time in order to reduce the extent and severity of demyelination.

Cervical balance and clinical outcomes in cervical spondylotic myelopathy treated by three-level anterior cervical discectomy and fusion and hybrid cervical surgery: A CONSORT-compliant study with minimum follow-up period of 5 years.

ABSTRACT: As the technology of combining with fusion and nonfusion procedure, cervical hybrid surgery (HS) is an efficacious alternative for treatment with cervical spondylotic myelopathy. While studies on cervical alignment between 3-level HS and anterior cervical discectomy and fusion (ACDF) were seldom reported. The effects of cervical imbalance on its related clinical outcomes are yet undetermined as well.Patients with cervical spondylotic myelopathy, who underwent 3-level ACDF or HS, were included to compare cervical alignment parameters after surgery and then explore the relationship between cervical balance and clinical outcomes.Forty-one patients with HS (HS group) and 32 patients who with ACDF (ACDF group) were reviewed from February 2007 to September 2013 with the mean follow-up of 90.3 ±â€Š25.5 (m) and 86.3 ±â€Š28.9 (m), respectively. Cervical alignments parameters including the C2 to C7 cervical lordosis (CL), C2 to C7 sagittal vertical axis, T1 slope. and T1SCL (T1 slope minus CL), and the clinical outcomes like neck disability index (NDI) and Japanese Orthopedic Association (JOA) score were measured and recorded preoperatively (PreOP), intraoperatively, and on the first preoperative day and the last follow-up (FFU). The balance and imbalance groupings were sorted based on the T1SCL: T1SCL≤20°,balance; T1SCL > 20°, imbalance.We found significant improvements (P < .001) in NDI and JOA at intraoperatively and FFU after ACDF and HS, and no difference on cervical alignment and clinical outcomes between the 2 procedures on the basis of intergroup comparisons. By between-subgroups comparisons, however, we found significant differences in CL and T1SCL at PreOP (P < .05). Nonetheless, there was no significant difference on the clinical outcomes between balance and imbalance subgroups at FFU at PreOP (P > .05), indicating that the change of T1SCL was not correlated to NDI and JOA at FFU.Both HS and ACDF groups showed significant clinical improvements after surgery. There was no correlation between cervical balance and clinical symptoms.

Copper deficiency-associated myelopathy in cryptogenic hyperzincemia: a case report.

Copper deficiency syndrome is an underestimated cause of posterior myelitis. We describe the case of a 41-year-old woman, who developed a subacute ataxic paraparesis associated with low back pain. Her 3T spine MRI showed a thin hyperintense FS-Echo T2 longitudinally extensive lesion involving the posterior columns of the cervical cord (from C2 to C6). An extensive diagnostic work-up excluded other causes of myelopathy and blood tests pointed out hypocupremia and mild hyperzincemia. Patients affected by this rare form of oligoelement deficiency typically develop progressive posterior column dysfunction with sensory ataxia and spasticity, sometimes associated with sensori-motor polyneuropathy. Clinical and radiological characteristics of posterior myelopathy due to copper deficiency are briefly reviewed. Physicians should be aware of this condition since a prompt introduction of copper supplementation can avoid progression of the neurological damage.

Clinical study on improving postoperative symptoms of cervical spondylotic myelopathy by Qishe pill.

BACKGROUND: Cervical spondylotic myelopathy (CSM) is the most serious type of cervical spondylosis, which is often treated surgically in patients with progressive neurological symptoms following ineffective conservative treatment. However, some patients have residual symptoms such as neck pain, stiffness, and C5 nerve palsy after surgery. The Qishe pill can effectively relieve the symptoms of neck pain and numbness, but there is no evidence showing the efficacy and safety of the Qishe pill in treating symptoms after spinal cord surgery. METHODS/DESIGN: A multicenter, randomized controlled clinical trial will be conducted to evaluate the efficacy and safety of the Qishe Pill. A total of 330 patients with CSM who receive surgical treatment will be randomly divided into 2 groups, treated for 12 weeks and with a 1-year follow-up. The primary outcome will be Japanese Orthopaedic Association score from the baseline to 4 weeks, 12 weeks, 24 weeks, and 48 weeks after surgery. Secondary outcomes will include Visual Analogue Scale score, Neck Disability Index, and imaging indicators (including magnetic resonance imaging and X-ray). Additionally, adverse reactions will be observed and recorded as safety indicators. DISCUSSION: Although the Qishe pill can effectively improve the discomfort of the neck and upper limbs in clinical applications, there is a lack of clinical research on postoperative patients. This study will investigate the efficacy and safety of the Qishe pill in treating postoperative symptoms of CSM. TRIAL REGISTRATION: Clinical Trials.gov ID: ChiCTR1900028173. Registered on 17 December 2019.

A randomized, double-blind, placebo-controlled trial for Yi-Qi Hua-Yu tong-sui granule in the treatment of mild or moderate cervical spondylotic myelopathy.

BACKGROUND: Neck pain, sensory disturbance and motor dysfunction in most patients suffered cervical spondylotic myelopathy (CSM). However, some conservative treatments are limited by their modest effectiveness. In the other hand, surgical treatment is necessary when symptoms are refractory to conservative treatments and neurological function of the patients has deteriorated. Many patients use complementary and alternative medicine, including traditional Chinese medicine, to address their symptoms. The purpose of the present study is to examine effectiveness and safety of Yiqi-Huayu-Tongsui (YQHYTS) granule, a compound traditional Chinese herbal medicine, on symptoms in patients with mild or moderate CSM. METHODS/DESIGN: A randomized, double blinded, placebo-controlled clinical trial to evaluate the efficacy and safety of YQHYTS granule is proposed. 72 patients in Longhua Hospital with the diagnosis of mild or moderate CSM will be randomly allocated into 2 groups, and treated with YQHYTS granule or placebo. The prescription of the trial drugs (YQHYTS granule/placebo) is 20 grams twice a day for 3 months. The primary outcome measurements include visual analog scale, Japanese Orthopedic Association, and Neck Disability Index score. The secondary outcome measurements are electromyogram and Pfirrmann classification. DISCUSSION: YQHYTS granule has been established and applied in Longhua Hospital for many years. As it has a potential benefit in treating mild or moderate CSM, we designed a double-blind, prospective, randomized controlled trial and would like to publish the results and conclusions later. If YQHYTS granule can alleviate neck pain, sensory disturbance, and even motor dysfunction without adverse effects, it may be a unique strategy for the treatment of mild or moderate CSM. TRIAL REGISTRATION: Chinese Clinical Trial Registry ID: ChiCTR1900028192. Registered 15 December 2019, Available at: http://www.chictr.org.cn/edit.aspx?pid=46913&htm=4.

Human recombinant erythropoietin improves motor function in rats with spinal cord compression-induced cervical myelopathy.

OBJECTIVE: Erythropoietin (EPO) is a clinically available hematopoietic cytokine. EPO has shown beneficial effects in the context of spinal cord injury and other neurological conditions. The aim of this study was to evaluate the effect of EPO on a rat model of spinal cord compression-induced cervical myelopathy and to explore the possibility of its use as a pharmacological treatment. METHODS: To develop the compression-induced cervical myelopathy model, an expandable polymer was implanted under the C5-C6 laminae of rats. EPO administration was started 8 weeks after implantation of a polymer. Motor function of rotarod performance and grip strength was measured after surgery, and motor neurons were evaluated with H-E, NeuN and choline acetyltransferase staining. Apoptotic cell death was assessed with TUNEL and Caspase-3 staining. The 5HT, GAP-43 and synaptophysin were evaluated to investigate the protection and plasticity of axons. Amyloid beta precursor protein (APP) was assessed to evaluate axonal injury. To assess transfer of EPO into spinal cord tissue, the EPO levels in spinal cord tissue were measured with an ELISA for each group after subcutaneous injection of EPO. RESULTS: High-dose EPO maintained motor function in the compression groups. EPO significantly prevented the loss of motor neurons and significantly decreased neuronal apoptotic cells. Expression of 5HT and synaptophysin was significantly preserved in the EPO group. APP expression was partly reduced in the EPO group. The EPO levels in spinal cord tissue were significantly higher in the high-dose EPO group than other groups. CONCLUSION: EPO improved motor function in rats with compression-induced cervical myelopathy. EPO suppressed neuronal cell apoptosis, protected motor neurons, and induced axonal protection and plasticity. The neuroprotective effects were produced following transfer of EPO into the spinal cord tissue. These findings suggest that EPO has high potential as a treatment for degenerative cervical myelopathy.

Acute Presentation of Cervical Myelopathy Following Manipulation Therapy.

BACKGROUND: Spinal manipulation therapy (SMT) is commonly used as an effective therapeutic modality for a range of cervical symptoms. However, in rare cases, cervical manipulation may be associated with complications. In this review we present a series of cases with cervical spine injury and myelopathy following therapeutic manipulation of the neck, and examine their clinical course and neurological outcome. We conducted a search for patients who developed neurological symptoms due to cervical spinal cord injury following neck SMT in the database of a spinal unit in a tertiary hospital between the years 2008 and 2018. Patients were assessed for the clinical course and deterioration, type of manipulation used and subsequent management. A total of four patients were identified, two men and two women, aged 32-66 years. In three patients neurological deterioration appeared after chiropractic adjustment and in one patient after tuina therapy. Three patients were managed with anterior cervical discectomy and fusion while one patient declined surgical treatment. Assessment for subjective and objective evidence of cervical myelopathy should be performed prior to cervical manipulation, and suspected myelopathic patients should be sent for further workup by a specialist familiar with cervical myelopathy (such as a neurologist, a neurosurgeon or orthopedic surgeon who specializes in spinal surgery). Nevertheless, manipulation therapy remains an important and generally safe treatment modality for a variety of cervical complaints. This review does not intend to discard the role of SMT as a significant part in the management of patients with neck related symptoms, rather it is meant to draw attention to the need for careful clinical and imaging investigation before treatment.

A Case Report of a 4-Year-Old Boy with Intradural Spinal Cord Abscess Successfully Treated with Adjuvant Hyperbaric Oxygen Therapy.

Intradural spinal cord abscesses are rare infections in early childhood and usually result from pre-existing congenital anomalies of the spinal column. The formation of abscess may be the result of hematogenous spread. It is treated by surgical and parenteral antibiotic treatment, but some special cases may require additional treatments. This article presents a 4-year-old male patient who was previously operated on for spina bifida (meningocele and tethered cord syndrome) at another hospital. Upon complaints of not being able to walk after one month, he was operated on with the diagnosis of spinal intradural abscess and referred to our clinic to continue his treatment. The patient underwent an emergency operation when his new spinal magnetic resonance imaging (MRI) at our hospital showed progression of intradural abscess. Due to no regression of neurological deficits in the follow-up and the risk of another operation, antimicrobial treatment as well as hyperbaric oxygen therapy (HBOT) was planned. At the end of 20 HBOT treatment sessions, the patient started to walk with support and the antibiotic treatment was completed in six weeks. In cases where surgical and antimicrobial treatments have failed, HBOT should be considered as an additional treatment method in children with spinal abscess.

NuNec™ Cervical Disc Arthroplasty Improves Quality of Life in Cervical Radiculopathy and Myelopathy: A 2-yr Follow-up.

BACKGROUND: Anterior cervical disc replacement is an alternative to fusion for the treatment of selected cases of radiculopathy and myelopathy. We report clinical and radiological outcomes after disc replacement with the NuNec™ artificial cervical disc (Pioneer® Surgical Technology, Marquette, Michigan) with subgroup analysis. OBJECTIVE: To review clinical and radiological outcomes after anterior cervical disc replacement with the NuNec™ artificial cervical disc. METHODS: A consecutive case series of patients undergoing cervical disc replacement with the NuNec™ artificial disc was conducted. Clinical outcomes were assessed by questionnaires preoperatively and up to 2 yr postoperatively including neck and arm pain, Neck Disability Index, Euroqol 5-dimensions, and Short Form-36; x-rays from the same period were analyzed for range of movement and presence of heterotopic ossification. RESULTS: A total of 44 NuNec™ discs were implanted in 33 patients. Clinical improvements were seen in all outcomes; significant improvements on the Neck Disability Index, Euroqol 5-dimensions, and physical domain of the Short Form-36 were maintained at 2 yr. There was a mean of 4° range of movement at the replacement disc level at 2 yr, a significant reduction from baseline; there was also progression in levels of heterotopic ossification. Complications included temporary dysphagia (10%) and progression of disease requiring foraminotomy (6%); no surgery for adjacent level disease was required. There was no significant difference in the outcomes of the radiculopathy and myelopathy groups. CONCLUSION: Clinical outcomes using the NuNec™ disc replacement are comparable with other disc replacements. Although the range of movement is reduced, the reoperation rate is very low.

Subacute copper-deficiency myelopathy in a patient with occult celiac disease.

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.

Descripción de una serie hospitalaria de pacientes con fístula espinal / Description of a series of hospital patients with a spinal fistula

Introducción. Aunque las fístulas espinales suponen el 70% de las malformaciones arteriovenosas espinales, son una entidad infradiagnosticada. El shunt arteriovenoso produce una congestión vascular que da lugar a una mielopatía progresiva, en ocasiones irreversible si no se trata de forma precoz. Objetivo. Describir las características clinicorradiológicas de una serie de pacientes con fístula espinal. Pacientes y métodos. Se realizó una búsqueda retrospectiva de pacientes con diagnóstico de fístula espinal ingresados en el área de neurociencias de un hospital de tercer nivel asistencial. Resultados. Se identificaron 19 pacientes (7 mujeres y 12 varones) con una edad media de 56 años. La fístula espinal fue de tipo I en un 79% de los pacientes y la localización dorsal fue la más frecuente. La mayoría de los casos presentó un curso progresivo (90%). Un 74% de los pacientes se diagnosticó mediante resonancia magnética. En cuatro casos fue necesaria la realización de una angiografía para llegar al diagnóstico, y en uno de ellos se precisó una biopsia intraoperatoria. Se realizaron tres punciones lumbares, en dos de las cuales se objetivó pleocitosis linfocitaria e hiperproteinorraquia. El retraso diagnóstico medio fue de nueve meses. Se trató a un 79% de los pacientes, y de ellos sólo mejoró el 10%. Conclusiones. Ante una clínica sugestiva de fístula espinal, debe realizarse una angiografía espinal diagnóstica aunque el paciente estudiado pueda presentar características licuorales atípicas y normalidad en la resonancia magnética medular (AU)

Introduction. Although spinal fistulas account for 70% of all spinal arteriovenous malformations, they are an underdiagnosed condition. The arteriovenous shunt produces vascular congestion that gives rise to a progressive myelopathy, sometimes irreversible if it is not treated in the early stages. Aim. To describe the clinicoradiological characteristics of a series of patients with spinal fistulas. Patients and methods. A retrospective search was conducted for patients diagnosed with a spinal fistula who were hospitalised in the neuroscience area of a tertiary care hospital. Results. A total of 19 patients (7 females and 12 males) were identified, with a mean age of 56 years. The spinal fistula was type I in 79% of patients, and a dorsal location was the most frequent. Most of the cases (90%) presented a progressive course. Magnetic resonance imaging was used in the diagnosis in 74% of the patients. In four cases angiography was required to reach a diagnosis, and in one of them it was necessary to perform an intraoperative biopsy. Three lumbar punctures were performed, two of which revealed lymphocytic pleocytosis and high protein levels in cerebrospinal fluid. The average diagnostic delay was nine months. Seventy-nine per cent of the patients were treated and only 10% of them improved. Conclusions. When faced with a clinical picture suggestive of a spinal fistula, a diagnostic spinal angiography must be carried out, although the patient under study may present atypical cerebrospinal fluid characteristics and normal results in magnetic resonance imaging of the spinal cord (AU)

Retrospective study of the clinical effects of acupuncture on cervical neurological diseases in dogs.

This study was conducted to evaluate new acupuncture protocols for the clinical treatment of cervical spinal cord diseases in 19 dogs. Three treatment options containing Jing-jiaji (cervical jiaji) were developed to treat neck pain, hemiparesis, and tetraparesis depending on the severity. The interval between the neurological disease onset and treatment (duration of signs), time to improvement after treatment, and recovery time were compared in dogs by body weight, age, and dry needle acupuncture (AP) with or without electro-AP (EAP). The duration of signs was longer in dogs weighing greater than 10 kg than in those weighing less than 10 kg (p＜ 0.05). Improvement and recovery times did not vary by body weight. Additionally, improvement and recovery times did not vary by age. The improvement and recovery times were longer in the AP+EAP group than the AP group (p＜ 0.05). Acupuncture with Jing-jiaji was effective in cervical spinal cord diseases in different sized dogs and in middle-aged and senior dogs. This report standardized AP treatment containing Jing-jiaji for canine cervical problems and evaluated its effects. The newly standardized AP methodology offers clinical practitioners an effective way to improve the outcomes of cervical neurological diseases in dogs.

Nitrous Oxide Abuse and Vitamin B12 Action in a 20-Year-Old Woman: A Case Report.

Herein, we report a case of a 20-year-old (ethnicity not reported) woman with a history of nitrous oxide abuse and clinical symptoms consistent with spinal cord subacute combined degeneration with associated low serum concentrations of vitamin B12, elevated methylmalonic acid levels, and radiologic evidence of demyelination of the dorsal region of the spinal column. The health of the patient improved dramatically with B12 supplementation. In this case, we discuss the interaction of nitrous oxide with the enzymatic pathways involved in the biochemistry of vitamin B12.

Optic neuropathy, myelopathy, anemia, and neutropenia caused by acquired copper deficiency after gastric bypass surgery.

Malabsorptive bariatric surgery is rapidly becoming a major cause of copper deficiency given the increasing prevalence of these procedures for morbid obesity. Acquired copper deficiency can present with clinically significant hematologic and neurological manifestations. Although hematologic manifestations of copper deficiency are rapidly reversible, significant neurological improvement after copper supplementation therapy is unusual and many patients remain debilitated and may only experience, at best, stabilization of the neurological manifestations. Here we present a case of an undiagnosed copper deficiency several years after bariatric gastric bypass surgery, in a patient who concomitantly used zinc-containing denture cream for several years, associated with anemia, neutropenia, myelopathy, respiratory failure, and bilateral optic neuropathy, which caused major vision loss. This patient was also a heterozygote carrier of the 5,10-methylenetetrahydrofolate reductase A1298C gene polymorphism, which may affect copper metabolism. Intravenous copper repletion resulted in rapid correction of hematologic indices. However, neurological manifestations, including vision loss responded only modestly to copper supplementation, despite achieving normal blood copper concentrations. Clinicians should consider copper deficiency in patients at risk, as in this case, as a delayed diagnosis can lead to irreversible disability due to neurological manifestations.

Biotinidase deficiency presenting as recurrent myelopathy in a 7-year-old boy and a review of the literature.

Biotinidase deficiency may produce variable neurologic manifestations. Brainstem and spinal cord disease comprises an uncommon presentation of biotinidase deficiency. We describe a 7-year old boy with subacute progressive quadriplegia and "sighing" respirations. Severe biotinidase deficiency was established, and the patient demonstrated complete recovery with biotin supplementation. Genetic studies revealed presence of homozygous mutation in the BTD gene [c.133C>T (p.H447Y)]. Biotinidase deficiency should be considered in the differential diagnosis for subacute, long segment myelopathy, particularly with brainstem involvement. This entity is treatable; a high index of suspicion can be life-saving. We also review the literature on biotinidase deficiency presenting as spinal cord demyelinating disease.

[Peripheral neuropathy, myelopathy, cerebellar ataxia, and subclinical optic neuropathy associated with copper deficiency occurring 23 years after total gastrectomy].

We report a 61-year-old man with slowly progressive gait disturbance and paresthesia in the lower extremities following a total gastrectomy for gastric cancer 23 years previously. The patient presented with hyperreflexia, peripheral sensory neuropathy, and cerebellar ataxia. Magnetic resonance imaging showed atrophy of the cerebellum, and electrophysiological findings suggested the presence of disorder in both sides of the pyramidal tract, dorsal column, peripheral nerves, and optic nerve. Laboratory findings revealed anemia, neutropenia, and a remarkably low serum copper level (10 microg/dl; normal: 68-128). His serum vitamin E was slightly low and his serum vitamin B12 was within the normal limits. After administering an oral copper supplement, his symptoms improved with normalization of the serum copper level. We need to pay attention to myeloneuropathy caused by copper deficiency if the patient has a past history of total gastrectomy.

[Effect of the direct electric stimulation of the spinal cord in the treatment of neurologic complications of cervical osteochondrosis].

The results of the treatment of 92 patients with neurologic complications of cervical osteochondrosis (26 patients with isolated persistent lesions of roots C4-C7 and 66 patients with the combination of root syndromes and cervical myelopathy) are presented. The combination of direct electric stimulation of the spinal cord with different methods of surgical treatment was used. The efficacy of treatment was assessed with the scale for measuring severity of neurologic complications of cervical osteochondrosis and by electromyographic data before treatment and 1 and 6 months after the surgery. The significantly higher (p<0,05) effect of surgical treatment in the combination with direct electric stimulation of the spinal cord was shown.

Difficulty in walking 10 years after gastric surgery.

A 65-year-old gentleman presented with a history of abdominal distension and difficulty in walking 10 years after a Polya partial gastrectomy. Clinical history and neurological examination suggested an axonal sensory neuropathy. A computed tomographic scan of the abdomen showed a large afferent jejunal loop, and a hydrogen breath test confirmed small-bowel bacterial overgrowth secondary to the blind loop syndrome. Serological tests revealed low copper levels, which are a cause of a myeloneuropathy. The trace element deficiency occurred as a consequence of small-bowel bacterial overgrowth, and with antibiotic treatment of the bacterial overgrowth and copper supplementation his symptoms markedly improved.