Hoffmann Sign Research Articles

Lateral Suboccipital Craniotomy With C1-C2 Hemilaminectomies and C1-C3 Fusion for the Treatment of a C1-C2 Synovial Cyst Causing Spinal Cord Compression: 2-Dimensional Operative Video.

Atlantoaxial synovial cysts are a rare cause of cervical myelopathy. Here we describe a case of a 26-yr-old woman who presented with progressively decreasing right arm and leg strength and associated gait imbalance. On examination, she had diffuse weakness in the right upper and lower extremities, a positive right-sided Hoffman sign, and clonus in the right lower extremity. Computed tomography demonstrated an os odontoideum and a retro-odontoid cyst. Magnetic resonance imaging demonstrated a T1 hypointense, T2 hyperintense, slightly rim-enhancing retro-odontoid cyst causing a marked narrowing of the spinal canal, with resultant flattening and leftward deviation of the spinal cord. The patient consented to undergo cyst fenestration via a right suboccipital craniotomy and right C1-C2 hemilaminectomies, along with a C1-C3 instrumented posterior spinal fusion. This approach was chosen because it allows for cyst fenestration and instrumentation of the hypermobile cervical spine within the same incision. After the dura was opened and the arachnoid was dissected, the cyst was visualized compressing the spinal cord. The cyst was fenestrated just inferior to the C1 nerve rootlets, resulting in immediate egress of a gelatinous content; thereafter, all accessible cyst wall portions were removed. Fusion was performed with lateral mass screws at C1 and C3 and pars screws at C2. Pathological analysis described the cyst content as reactive fibrovascular tissue with cholesterol deposition. There were no complications associated with the procedure, and the patient's right-sided weakness had nearly resolved by postoperative day 1. Patient consent was granted for publication.

A Novel Technique of Endoscopic Anterior Transcorporeal Approach with Channel Repair for Adjacent Segment Disease After Anterior Cervical Discectomy and Fusion

To first report the application of percutaneous full-endoscopic anterior transcorporeal cervical discectomy (PEATCD) with channel repair for a patient with adjacent segment disease (ASD) after anterior cervical discectomy and fusion. PEATCD with channel repair was performed for a patient with ASD at the cranial level adjacent to previous fusion at the C5-C6 level. The pre- and postoperative clinical symptoms were evaluated with Japanese Orthopedic Association and visual analog scale (VAS). The radiological examinations included magnetic resonance imaging, computed tomography, and plain radiographs, which were used to evaluate the cervical alignment, stability, intraoperative decompression, and bony channel. The procedure was successfully completed within 70 minutes. The drainage tube was unnecessary. No surgery-related complications were recorded. The postoperative neck pain immediately improved to VAS 3 from preoperative VAS 6. The Japanese Orthopedic Association scores also took a turn for the better gradually from preoperative 10 to final 16 (improvement rate 85.7%). The muscle power recovered completely, and the Hoffman sign turned to negative during follow-up periods. Magnetic resonance imaging 1 week postoperatively showed a total removal of the herniation. The bony channel was almost disappeared on computed tomography images 3 months postoperatively. During postoperative periods, no relapse, channel collapse, bone plug migration, or instability was observed. As a novel and supplemental procedure for ASD after anterior cervical discectomy and fusion, PEATCD combines the advantages of transcorporeal approach and endoscopy together, which decreases iatrogenic damage to disc, preserves the cervical motion segment, and reduces surgical trauma. As the limitations of 1 case show, the effectiveness and reliability of PEATCD for patients with ASD should be verified in further studies.

O0007. Delayed Presentation In Chiari Malformation In Pakistani Population

Ossification of posterior longitudinal ligament (OPLL) is a pathological process in which lamellar bone is deposited at the posterior longitudinal ligament and can lead to a limited range of cervical motion and spinal cord compression. A 64-year-old man presented with a 10-month history of worsening clumsiness in the hands and impaired gait, and he occasionally had a feeling of an electric shock in the limbs when the neck was flexed. Physical examination revealed atrophy of the intrinsic hand muscles, rapid reflexes in the lower extremities, and positive Hoffman sign and Babinski sign results. Seesaw-like OPLL was observed on hyperextension and hyperflexion x-rays, which also showed that the OPLL involved the spinal canal; laminoplasty and laminectomy were not recommended for this specific type of OPLL, even though the K-line was positive on both x-rays.

Fever as a rare combined symptom of degenerative cervical myelopathy: a case report and literature review

A 60-year-old male patient complained of weakness of both legs for one year, intermittent fever for two months, up to 38.0 °C. Physical examination showed bilateral hyperreflexia of knee tendon and positive Hoffman sign on the right side. MR imaging of the cervical spine showed central herniation of the cervical 5-7 disc and compression of the spinal cord. The WBC was normal, C-reactive protein was 24.42mg/l, ESR was 55mm/h, TB antibody, anti acid staining and T-SPOT were negative. Autoantibody was negative and thyroid function was normal. The JOA score was 9 points. During the operation, the herniated disc tissue was taken out for pathological examination and bacterial culture. The posterior longitudinal ligament was removed and no abscess was found. The symptom of asthenia in both legs was relieved and fever disappeared. No growth of aerobe, anaerobe or tubercle bacilli was found in the culture of resected tissue. One year after the operation, the fever did not recur, JOA score increased to 14 points, and MR imaging showed no protrusion in cervical spinal canal. In this case, the fever disappeared after the operation for cervical spondylosis, which may be a special manifestation of sympathetic nerve stimulation or autonomic dysfunction by chronic compression of spinal cord.

Seesaw-Like Cervical Ossification of Posterior Longitudinal Ligament

Ossification of posterior longitudinal ligament (OPLL) is a pathological process in which lamellar bone is deposited at the posterior longitudinal ligament and can lead to a limited range of cervical motion and spinal cord compression. A 64-year-old man presented with a 10-month history of worsening clumsiness in the hands and impaired gait, and he occasionally had a feeling of an electric shock in the limbs when the neck was flexed. Physical examination revealed atrophy of the intrinsic hand muscles, rapid reflexes in the lower extremities, and positive Hoffman sign and Babinski sign results. Seesaw-like OPLL was observed on hyperextension and hyperflexion x-rays, which also showed that the OPLL involved the spinal canal; laminoplasty and laminectomy were not recommended for this specific type of OPLL, even though the K-line was positive on both x-rays.

Treatment of a Patient with Ossification of Ligamentum Flavum of Cervical Spine and Literature Review

<i>Background</i>: The ossification of ligamentum flavum occurs frequently in the thoracic vertebra, and the incidence of ossification of cervical ligamentum flavum is relatively low. It is easy to cause misdiagnosis and mistreatment due to lack of understanding in clinical practice. <i>Object</i>: To understand the diagnosis and treatment of cervical ossification of ligamentum flavum through the treatment of one case. <i>Material</i>: A 65-year-old female patient was hospitalized due to "lumbar discomfort with numbness of both lower limbs for 1 year, and aggravation with numbness and weakness of both upper limbs for the last 3 months". Physical examination showed cervical flexion, extension and lateral flexion activities were limited, post extension limitations were obvious, limb muscle strength was grade 4, right knee tendon reflex was active, and bilateral Hoffman sign and right Babinski sign were positive. Cervical JOA score was 8 points. Magnetic resonance imaging of cervical spine showed that there were pressure objects protruding to the spinal canal between C3/4, C5/6, and C6/7 laminae. Sagittal MRI showed segmental or focal nodular space occupying and protruding into the spinal canal from the rear of the spinal canal. Cross sectional MRI showed nodular or "M" shaped occupying space. T1 weighted image was equal signal, and T2 weighted image showed low signal shadow, suggesting high-density ossification and severe spinal cord compression. The effective sagittal diameter was less than 8mm. <i>Method</i>: Hemilaminectomy was performed for a single segmental OLF of C3/4, and a total laminectomy was performed for bilateral and multi-segmental OLF of C5/6, 6/7. According to the range of intraoperative decompression, C5 and 6 posterior lateral mass screws and C7 pedicle screws were fixed. <i>Result</i>: After the operation, the patient indicated that his upper limbs were relaxed, the umbilical horizontal band disappeared, and the muscle strength of his limbs was stronger than that before the operation. She could move freely on the ground, and her holding was more flexible and powerful than before. The postoperative JOA score was 12 points. The postoperative pathological result was fibrous connective tissue and ossification. The follow-up JOA score was increased to 15 points 8 months after operation, and the improvement rate was 78%. The patient could completely take care of herself, and the treatment was satisfactory. <i>Conclusion</i>: Surgical resection of cervical olf and canal decompression is an effective method for the treatment of Cervical OLF and can achieve good clinical results.

NCMP-21. REVERSIBLE MOTOR NEURON SYNDROME IN THE SETTING OF CANCER

Abstract 60 year old female was admitted with difficulty swallowing, slurred speech and gait instability resulting in multiple falls worsening over 2-3 weeks. Her exam revealed subtle spastic dysarthric speech, spasticity in all four extremities (lower &amp;gt; upper and right &amp;gt; left), hyperreflexia, and bilateral Babinksi and Hoffman signs consistent with pure upper motor neuron syndrome. An MRI brain and total spine were without evidence of neurological pathology but revealed an incidental finding of lymphadenopathy concerning for neoplastic process. Her symptoms continued to worsen over the next few weeks and she developed severe, nearly continuous, extensor spasms of BLE (right &amp;gt; left) with stimulus sensitivity causing inability to walk. She was started on baclofen, tizanidine and diazepam for spasticity with only mild improvement. An MRI cervical spine was repeated (please see below) and revealed hyperintense T2 signal in lateral corticospinal tract (right &amp;gt; left) without enhancement and no cord compression. Given strong clinical concern for paraneoplastic process patient received 0.4 g/kg IVIG for 5 days and concurrent 1000 mg methylprednisolone for 3 days with dramatic improvement within days. Cervical lymph node biopsy lead to diagnosis of nodal marginal zone lymphoma. A CT abdomen revealed an ovarian mass concerning for teratoma vs dermoid cyst (pending resection and final pathology). She was treated with weekly boosters of IVIG and IV methylprednisolone and was started on Rituximab for her Lymphoma. When seen in clinic a month post discharge nearly all of her symptoms had resolved. Neurological exam showed subtle residual spasticity making this presentation most consistent with a reversible pure upper motor neuron syndrome.

Understanding hyper-reflexia in acute motor axonal neuropathy (AMAN)

Hyper-reflexia is occasionally seen in acute motor axonal neuropathy (AMAN), but its pathophysiology is unclear. We report a patient with AMAN following Campylobacter jejuni enteritis, who showed generalized hyper-reflexia, bilateral Hoffmann sign and right Babinski sign. MRI and transcranial magnetic stimulation of the motor cortex disclosed no corticospinal tract involvement. An extensive electrophysiological investigation documented α-motoneuron hyperexcitability and dysfunction of the interneuronal inhibitory circuits in the spinal anterior horn. We propose an immune-mediated damage of the spinal inhibitory interneuronal network as possible mechanism inducing hyper-reflexia in AMAN.

Is there a relationship between the extent of tonsillar ectopia and the severity of the clinical Chiari syndrome?

Chiari 1 malformation is diagnosed if the cerebellar tonsils extend at least 5mm below the opisthion-basion line. To examine the correlation of the extent of tonsillar ectopia with the prevalence and severity of the symptoms associated with the Chiari malformation. Patients (N = 428) were grouped according to the extent of tonsillar ectopia on the mid-sagittal MRI image (group 1, 0-< 3mm; group 2, 3-5mm; group 3, > 5mm). Groups were compared regarding demographics, symptoms, neurological signs, pain score, and response to HADS and sf-36 questionnaires. Results were analyzed using one-way ANOVA, chi-square, and two sample Z test, and Student's t test for pairwise comparison, (statistical significance p < 0.05). A logistic regression analysis was performed to determine the relationship between tonsillar ectopia and the probability of a patient reporting any particular symptom. There were 97,148 and 183 patients in groups 1, 2, and 3 respectively. Groups did not differ with regard to antecedent trauma or female preponderance. Patients in group 1 were more symptomatic than those in groups 2 and 3 with regard to some symptoms, (p = 0.04-p = 0.000). Regression analysis confirmed an inverse relationship between the extent of tonsillar ectopia and the likelihood of many symptoms. The pain score was greatest in group 1, (p = 0.006). Prevalence of objective signs of myelopathy did not differ between groups except for Hoffmann sign which was more prevalent in group 1, (p = 0.034). HADS and sf-36 scores did not differ between groups. The severity of the symptoms associated with the Chiari malformation does not correlate directly with the extent of tonsillar ectopia. The extent of tonsillar ectopia should be re-evaluated as the threshold for diagnosis of Chiari 1 malformation.

A novel ABCD1 gene mutation causes adrenomyeloneuropathy in a Chinese family

BackgroundAdrenomyeloneuropathy (AMN) is a rare genetic disease. In this study, a case of AMN was uncovered in a Chinese family.MethodsClinical manifestations were collected and observed through medical records, physical examination, laboratory tests, and magnetic resonance imaging (MRI). Generation sequencing of the ABCD1 gene was performed, and the pedigree of the family was analyzed.ResultsThe proband suffered from adrenocortical insufficiency at 8 years old and presented with a slowly progressive gait disorder at 21 years old. Physical examination, laboratory tests, and MRI showed that he had adult‐onset AMN manifestations, including spasticity and hyperactive tendon reflexes with Hoffman and Babinski signs in the limbs, difficulty in performing the heel‐to‐shin test, hyperpigmentation, increased levels of adrenocorticotropic hormone and very long‐chain fatty acids, decreased levels of corticosteroid and serum gesterol, and salient atrophy of the cervical and thoracic spinal cord. DNA analysis revealed a missense variant, c.290A>C (p.His97Pro) in exon 1 of the ABCD1 gene, in the proband. Sanger sequencing confirmed that the proband's mother was heterozygous for the same variant. The ABCD1 gene mutation transmitted in an X‐linked inheritance manner.ConclusionA novel missense mutation in the ABCD1 gene was identified in a Chinese family, which caused an unusual manifestation of adult‐onset AMN. This discovery is beneficial for the genetic counseling of patients with X‐linked adrenoleukodystrophy.

A Clinical Correlation Research of the Hoffmann Sign and Neurological Imaging Findings in Cervical Spinal Cord Compression

The Hoffmann sign is usually used as an indicator of upper motor neuron lesion, but its clinical effect remains controversial in previous reports. A retrospective case control study including 107 patients with cervical complaints was carried out. According to the presence of Hoffmann sign, patients were divided into 2 groups. The radiographic results were assessed and the sensitivity, specificity, positive and negative predictive values, and false positive and false negative values of Hoffmann sign for cervical pathology, segment, cervical spine canal ratio, and S-index were calculated. There were 56 patients in the positive Hoffmann group and 51 patients in negative group. The sensitivity, specificity, positive and negative predictive values, and false positive and false negative values of Hoffmann sign for cervical pathology were found to be 61.6%, 85.7%, 94.6%, 35.3%, 14.3%, 38.4% and 60.5%, 81.0%, 92.9%, 33.3%, 19.0%, 39.5%, respectively. The ratio of cervical spine canal was lower in the positive Hoffmann group than in control group. Although the Hoffmann sign is not foolproof in the diagnosis of cervical spinal cord compression, it can be used to assess symptomatic patients. The narrower the cervical spine canal or the higher the cervical segment compression, the higher of the incidence of positive Hoffmann sign.

Snake-Eyes Appearance on MRI Occurs during the Late Stage of Hirayama Disease and Indicates Poor Prognosis.

Purpose Because Hirayama disease is stereotyped as a self-limited disease in the absence of a definite pathology, we investigated the potential relationship between snake-eyes appearance (SEA) and Hirayama disease to bring a new perspective in the pathological process of Hirayama disease based on relevant radiological and clinical evidence. Methods A total of 30 cases observed SEA were selected from 293 patients with Hirayama disease to constitute the SEA group, and an equal number of cases were randomly selected from the remaining patients to form the non-SEA group. Cervical magnetic resonance imaging (MRI) was performed and subsequently used to measure the anteroposterior diameter and anterior shifting of the spinal cord. Additionally, clinical data, such as age, sex, duration of symptoms, symptoms, and signs, were collected and analyzed. Results Of 293 patients, 10.6% appeared with the SEA, which was mainly multisegmental (86.7%), particularly at the C5-6 segment (73.3%), and intense with a well-defined border (70.0%). The SEA group was an older population (p < 0.0001) with a longer duration (p < 0.0001) and a higher incidence of Hoffmann signs and knee hyperreflexia (p < 0.0001, p = 0.0038, respectively). The degree of spinal cord atrophy demonstrated a close association with the SEA, as it was significantly worse in the SEA group and SEA segment (p = 0.0008, p < 0.0001, respectively). The degree of spinal cord atrophy was positively related to both age and duration (p = 0.0095, p = 0.0176, respectively). Conclusions Confirmed as an irreversible lesion and an indication of poor prognosis, SEA appears during the late stage of Hirayama disease and is closely related to pyramidal signs and spinal cord atrophy.

Retropulsion: Presentation, Diagnosis and Applications of Functional Neurology

Event Abstract Back to Event Retropulsion: Presentation, Diagnosis and Applications of Functional Neurology Gilbert S. Jaudy1* 1 Jaudy Treatment Center, United States A 45-year-old male presented to our office, complaining of frequent and constant seizures since 2000, that happen when he assumes any sitting or standing position, and sometimes spontaneous. He also reported a history of falls, injuries, muscle spasms, balance problems, stomachaches, osteoarthritis, eye pain, blurred vision, vertigo, belching, gas, gallbladder removal, depression, light-headedness, headaches, dizziness, itching, back/neck/shoulder/arm/knee/ankle pain, allergies, painful joints, swollen joints, migraines, and extreme fatigue. He’s on different types of medications. He has seen about 15 physicians for his condition, and was told that he had to get used to it and manage it. On 03/15/2017 during consultation with Dr. Jaudy at Jaudy Treatment Center, the patient had a 10 minute spastic seizure which was described by her husband as “that’s what happens almost all day long”. Methods The patient had undergone a neurological examination in stages, information was obtained when the patient was still for moments. Standard neurological tests and diagnostics including VNG and posturography were obtained. She had general sensory and motor deficiency. Posterior center of pressure with left deviation. Severe spastic myoclonus on lower extremities. During seizure patient developed decerebrate posturing on the right side, and decorticate posturing on the left, in addition to bilateral positive Babinski and Hoffman signs. The patient had slow and irregular pursuits with loss of gaze. A slow systemic Functional Neurology approach was implemented using various sensory and motor, orthostatic postural positions and applications, organ remapping procedures, visual and auditory stimulation, cerebellar activation, complex therapeutic activities, home instructions and dietary changes. Results The patient responded favorably during the first week. First visit post testing revealed immediate improvement and resolution of pathological reflexes (Babinski’s and Hoffman’s). Post first treatment testing showed immediate improvement in motor and sensory function on the left side vs. the right, and reduction of frequency and duration of seizures. The patient was treated for 3 months. Today he lives symptom free, migraine free, and enjoys life with family and friends. References All references and clinical date are available at the Jaudy Treatment Center Keywords: Retropulsion, Seizures, Migraines, balance problems, Eye Pain, joint pain, Vertigo, Muscle spasms, Blurred vision, Gas, Itching, Osteoarthritis Conference: International Symposium on Clinical Neuroscience, Orlando, United States, 24 May - 26 May, 2019. Presentation Type: Poster Presentation Topic: Clinical Neuroscience Citation: Jaudy GS (2019). Retropulsion: Presentation, Diagnosis and Applications of Functional Neurology. Front. Neurol. Conference Abstract: International Symposium on Clinical Neuroscience. doi: 10.3389/conf.fneur.2019.62.00067 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 28 Mar 2019; Published Online: 27 Sep 2019. * Correspondence: Dr. Gilbert S Jaudy, Jaudy Treatment Center, Palm Desert, California, 92211, United States, drjaudy@hotmail.com Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Gilbert S Jaudy Google Gilbert S Jaudy Google Scholar Gilbert S Jaudy PubMed Gilbert S Jaudy Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

ESCLEROSE LATERAL PRIMÁRIA

Introdução: Esclerose Lateral Prima?ria (ELP) é uma doenc?a neurodegenerativa rara de origem desconhecida caracterizada por envolvimento do trato corticoespinhal, que comumente se manifesta por espasticidade progressiva, porém sem lesão do segundo neurônio motor.&#x0D; Objetivos: Relatar um caso de ELP, realizar revisão da literatura e dos critérios diagnósticos.&#x0D; Metodologia: relato de caso e revisão da literatura.&#x0D; Relato do Caso: Mulher, branca, hígida, apresentou aos 35 anos quadro progressivo de alteração da marcha, do equilíbrio e perda na orientação dos movimentos. Redução progressiva da força em membros superiores, posterior ao quadro de paraparesia instalado. Inicia-se também nesse período importante perda ponderal em razão de disfagia associada. Progrediu para tetraparesia, e disfonia severas sem qualquer verbalização aos 37 anos. Na evolução observou-se comprometimento de vários domínios da cognição, além de grande labilidade emocional. Ao exame observa-se tetraparesia espástica, hiperreflexia nos quatro membros, clonus simétrico, sinais de Babinski e Hoffman bilaterais, e afasia motora. Exames laboratoriais, TC de tórax e abdômen, EEG sem alterações. RM de crânio demonstrando atrofia cortical difusa, dilatação ventricular compensatória; RM de coluna sem alterações. ENMG sem sinais de acometimento do segundo neurônio motor.&#x0D; Conclusões: Trata-se de um caso de doença neurodegenerativa do neurônio motor superior, com acometimento de funções cognitivas. A paciente preenche critérios clínicos propostos por Pringle et al., 1992 para o diagnóstico da ELP. Outras causas de lesão do neurônio motor foram excluídas para a confirmação diagnóstica.

A Systematic Review of the Utility of the Hoffmann Sign for the Diagnosis of Degenerative Cervical Myelopathy.

Systematic review. To determine the validity of the Hoffmann sign for the detection of degenerative cervical myelopathy (DCM) for patients presenting with cervical complaints. While physical examination maneuvers are often used to diagnose DCM, no previous review has synthesized diagnostic accuracy data. Medline, Embase, and HealthStar were searched for articles from January 1, 1947 to March 1, 2017 using the following terms: Spinal Cord Diseases, Spinal Cord Compression, Cervical Vertebrae, Signs and Symptoms, Physical Examination, Epidemiologic studies, Epidemiologic Research Design, Predictive Value of Tests, and Myelopathy. The Quality Assessment of Diagnostic Accuracy Studies (QUADAS) checklist was applied to determine the level of evidence. Articles included were published in English or French language, rated as QUADAS level 3 or higher with a minimum 10 patients presenting with cervical complaints having undergone the Hoffman sign. Excluded studies recruited patients with a nondegenerative type of cervical myelopathy, and/or no evaluation with magnetic resonance imaging. A total of 589 articles were selected for review. Following the application of inclusion and exclusion criteria, 45 articles were analyzed using the QUADAS checklist. Only of three articles were of QUADAS quality 3 or higher. Analysis of combined data from 2/3 studies indicated that the Hoffman sign has a positive likelihood ratio of 2.2 (95% CI 1.5-3.3) and a negative likelihood ratio of 0.63 (95% CI 0.5-0.8). A positive Hoffman alone is unlikely to lead to more than a small change in estimated probability of DCM as compared with the gold standard test (magnetic resonance imaging). Variability in results across individual studies may result from differences in study design. There are insufficient data to support use of the Hoffman sign alone to confirm or refute a diagnosis of DCM. 1.

Wide-Open Eyes to the “Snake-Eye Sign”

Patient with progressive four-limb weakness, unsteadiness and falls. Examination revealed paretic-spastic gait, spastic tetraparesis, brisk tendon reflexes, reduced pain sensation at the right side and bilateral Hoffman and Babinski signs. Cervical magnetic resonance (MRI) revealed the “snake-eye sign” and spinal cord compression by protruded discs during cervical extension.

Improvement of severe myalgic encephalomyelitis/chronic fatigue syndrome symptoms following surgical treatment of cervical spinal stenosis

BackgroundMyalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a potentially disabling disorder. Little is known about the contributors to severe forms of the illness. We describe three consecutive patients with severe ME/CFS whose symptoms improved after recognition and surgical management of their cervical spinal stenosis.MethodsAll patients satisfied clinical criteria for ME/CFS and orthostatic intolerance, and were later found to have cervical spinal stenosis. Overall function was assessed before and after surgery using the Karnofsky score and the SF-36 physical function subscale score.ResultsNeurological findings included > 3+ deep tendon reflexes in 2 of 3, a positive Hoffman sign in 2 of 3, tremor in 2 of 3, and absent gag reflex in 1 of 3. The cervical spine canal diameter in the three patients ranged from 6 to 8.5 mm. One had congenital cervical stenosis with superimposed spondylosis, and two had single- or two-level spondylosis. Anterior cervical disc replacement surgery in two patients and a hybrid anterior cervical disc fusion and disc replacement in the third was associated with a marked improvement in myelopathic symptoms, resolution of lightheadedness and hemodynamic dysfunction, improvement in activity levels, and improvement in global ME/CFS symptoms.ConclusionsThe prompt post-surgical restoration of more normal function suggests that cervical spine stenosis contributed to the pathogenesis of refractory ME/CFS and orthostatic symptoms. The improvements following surgery emphasize the importance of a careful search for myelopathic examination findings in those with ME/CFS, especially when individuals with severe impairment are not responding to treatment.

The Significance of the Trömner Sign in Cervical Spondylotic Myelopathy Patient.

This study is a diagnostic analysis. To investigate the diagnostic accuracy of Trömner sign in cervical spondylotic myelopathy (CSM), and how its presence correlates with the severity of myelopathy. A clinical presentation of myelopathy corresponding with image findings is a current standard to diagnose CSM. Trömner sign is an alternative of well-known Hoffmann sign to detect CSM. Little is known about its diagnostic accuracy and how its presence correlates with the severity of CSM. Consecutive patients with clinical diagnosis of CSM and other cervical spondylosis-related problems were enrolled in either CSM group, cervical spondylotic radiculopathy group, or axial pain group. Normal volunteers and patients without spine-related issues were used as a control. All participants were examined for the presence of myelopathic signs. Magnetic resonance imaging studies of all participants were reviewed by a radiologist. There were 85 participants included in the study. Diagnostic sensitivity was 76%, 94%, 76%, and 36% for Hoffmann sign, Trömner sign, inverted radial reflex, and Babinski sign, respectively. Trömner sign had relatively high sensitivity (95%) despite of mild degree of myelopathy. Negative predictive value was 60%, 85%, 59%, and 38% for Hoffmann sign, Trömner sign, inverted radial reflex, and Babinski sign, respectively. There were 63%-71% of patients in either axial pain group or cervical spondylotic radiculopathy group had positive Trömner sign. Most of CSM patients with cord signal changed had positive myelopathic sign. Regarding CSM patient without cord signal change, most of tests were negative except Trömner sign. High sensitivity (94%) and relatively high negative predictive value (85%) for Trömner sign indicate the usefulness of Trömner sign in ruling out CSM. High incidence of positive Trömner sign in presymptomatic cervical cord compression patients suggests Trömner sign could have a useful role in early detection of presymptomatic patients.

Reversible and unilateral corticospinal tract disease secondary to autoimmune free-T3-thyrotoxicosis

68-year-old female patient with no significant medical history presents with a 3-month history of progressive neurological symptoms, which began with left eye ptosis, blurred vision and non-painful jaw discomfort, followed...

Association Between Paraspinal Muscle Morphology, Clinical Symptoms, and Functional Status in Patients With Degenerative Cervical Myelopathy.

A cross-sectional study. The aim of this study was to assess fatty infiltration and asymmetry of the multifidus (MF), semispinalis cervicis (SCer), semispinalis capitis (SCap), and splenius capitis (SPL) muscles in patients with degenerative cervical myelopathy (DCM), and evaluate their correlations with clinical symptoms and functional scores. Cervical muscle alterations have been reported in patients with chronic neck pain, but the assessment of cervical muscle morphology has been overlooked in patients with DCM. Thirty-eight patients diagnosed with DCM and spinal cord compression at C4-C5 or C5-C6 (first level of compression) were included. Cervical muscle measurements of cross-sectional area (CSA) and ratio of functional CSA (fat-free area, FCSA) to total CSA were obtained from T2-weighted axial images at the level above, same, and level below the most cranial level of spinal cord compression. Muscle fatty infiltration and asymmetry was assessed at every level and their associations with respect to clinical signs and symptoms and functional scores were investigated. There was a significant increase in fatty infiltration (decrease in FCSA/CSA ratio) of the MF (P = 0.001) and SPL (P < 0.001) muscles at the level below the spinal cord compression. A significant increase in MF CSA asymmetry was also observed at the level below the compression. Lower MF FCSA/CSA ratio was associated with longer 30-m walking test time. Lower SCer FCSA/CSA was associated with corticospinal distribution motor deficits and atrophy of the hands. Greater asymmetry in SCap CSA was associated with higher Neck Disability Index (NDI) scores, whereas lower asymmetry in MF CSA was associated with a positive Hoffman sign and weakness. A significant increase in muscle fatty infiltration and CSA asymmetry at the level below the compression was observed in patients with DCM. Our results also suggest an association between cervical muscle morphology and DCM clinical symptoms and functional status. 2.