Chronic flaccid quadriparesis from tract specific myelopathy in neurosyphilis.

INTRODUCTION: Tract-specific myelopathies with distinctive imaging features are uncommon and typically occur with metabolic or paraneoplastic syndromes. We report a unique case of tract-specific myelopathy with neurosyphilis. CASE PRESENTATION: A 53-year-old male presented with a four-month history of flaccid quadriparesis, sensory loss, and bladder dysfunction. His MRIs revealed striking symmetric T2-weighted hyperintensities in the lateral corticospinal tracts and dorsal columns of the cervical spinal cord that extended rostrally into the pyramidal decussation and medial lemnisci of the medulla oblongata. Nerve conduction and needle electromyography studies excluded axonal or demyelinating lower motor neuron disorders. The patient reported previous untreated primary syphilis and was seropositive on the T.pallidum hemagglutination assay. Penicillin therapy resulted in substantial clinical improvement. DISCUSSION: Although syphilitic meningomyelitis is well-reported, our patient was unique because of the persistent flaccidity (possibly suggesting prolonged spinal shock) and striking tract-specific MRI patterns. These features are novel in syphilitic myelitis and suggest unknown mechanisms of tract-specific tropism and neuronal injury. CONCLUSIONS: "Tract-specific" complete transverse myelopathy with persistent flaccid weakness and areflexia is a novel presentation of neurosyphilis. Early recognition and crystalline penicillin therapy can alleviate morbidity. Our report describes this patient's findings and discusses the differential diagnoses of tract-specific myelopathies.

John Goodsir (1814-1867) and his neurological illness.

John Goodsir, conservator and professor of anatomy at the University of Edinburgh, suffered an unidentified illness described by experts after his death as tabes. The features that led to this diagnosis, the understanding of tabes at that time and its relationship in some cases to syphilis, are discussed. It is concluded that the most likely diagnoses are subacute combined degeneration of the cord as a result of malnutrition or tabes dorsalis resulting from earlier syphilis. The presence of 'lightning pains' leans towards the latter diagnosis but evidence for a means of acquisition of syphilis is lacking. The disadvantages of retrospective diagnosis are discussed.

Tabes dorsalis in the 19th century. The golden age of progressive locomotor ataxia.

Tabes dorsalis, a late neurological complication of syphilis, is nowadays almost extinct. The path to understanding this disease and its pathophysiology was long and winding, spanning multiple centuries. The 19th century was a crucial period for understanding it. In the first third of the century, German and French physicians defined the semiology of tabes dorsalis, renamed in France "ataxie locomotrice progressive [progressive locomotor ataxia]." Nevertheless, the multiplicity of ancient and recent terms and the description of sometimes unclear nosological concepts (tabes nervosa, tabes spasmodic, nervo-tabes, etc.) were a hindrance to understanding it. Tabes dorsalis was a fertile ground for the description of many clinical signs that have become classics in medicine. No real treatment was available and various unusual therapies were performed. For a long time, the etiology of this disease remained unknown. The link between syphilis and tabes dorsalis was slowly established in the second part of the 19th century from epidemiologic observations. We present an overview of the concept of tabes dorsalis in the medical context of the 19th century and discuss the medical observations of some famous patients suffering from the disease such as Édouard Manet (1832-1883) and Alphonse Daudet (1840-1897).

Syphilitic meningomyelitis misdiagnosed as spinal cord tumor: Case and review.

Context: Syphilitic meningomyelitis is a rare manifestation of neurosyphilis, not well described in the literature.Methods: We reported a rare case of a 29-year-old female with syphilitic meningomyelitis. Her clinical manifestations and imaging findings were discussed with the related literatures reviewed.Results: The patient presented with progressive bilateral lower extremities numbness and weakness for months. Laboratory tests revealed positive serum Treponema pallidum Hemagglutinin Test (TPHA) and rapid plasma reagin test (RPR). The cerebral spinal fluid (CSF) was positive with TPHA but negative for RPR with lymphocytic pleocytosis and elevated protein. Spinal MRI showed swelling and high-signal intensity of thoracic spinal cord except T6-7 level with associated gadolinium enhancement ("flip-flop sign") and peripheral strip-like enhancement on T1WI ("candle guttering appearance"). She was initially diagnosed as spinal cord tumor due to the chronic clinical onset and cord swelling with central enhancement found on thoracic MRI. After dramatic clinical and radiographic improvement with dexamethosone and serological tests of syphilis, she was diagnosed as probable syphilitic meningomyelitis. Till now, there are 12 cases of syphilitic myelitis reported with spinal cord MR images. Thoracic cord is the predominant involved segment (10/12), "candle guttering appearance" is the most common enhancing characteristics of the lesion (7/12), "flip-flop sign" may be seen in the stage with significant inflammation (3/12).Conclusion: Syphilitic meningomyelitis can occur at early or late stage of syphilis, the onset may be acute, subacute or chronic. The imaging findings suggested focal inflammation of the spinal cord. Prognosis is relatively good after proper treatment.

Inhibition of phosphodiesterase IV enzyme improves locomotor and sensory complications of spinal cord injury via altering microglial activity: Introduction of Roflumilast as an alternative therapy.

Despite the great search for an effective approach to minimize secondary injury in spinal cord injury (SCI) setting, there have been limited advances. Roflumilast is a selective inhibitor of phosphodiesterase 4 with potent anti-inflammatory properties. Here, we sought to explore Roflumilast efficacy in the improvement of locomotor and sensory deficits of SCI. In an animal setting, 50 male rats were randomly assigned to five groups: an SCI group receiving Placebo, three SCI groups receiving Roflumilast at the doses of 0.25, 0.5, and 1 mg/kg prior to T9 vertebra laminectomy, and a sham-operated group. Locomotor, mechanical, and thermal activities were evaluated for 28 days. At the end of the study, spinal cord samples were taken to assess the relative ratio of microglial subtypes, including M1 and M2, histopathological changes, levels of pro-inflammatory (TNF-α and IL-1ß) and anti-inflammatory (IL-10) biomarkers, and cAMP level. Repeated measure analysis revealed significant effect for time-treatment interaction on locomotion [F (24, 270) = 280.7, p < 0.001], thermal sensitivity [F (16, 180) = 4.35, p < 0.001], and mechanical sensitivity [F (16, 180) = 7.96, p < 0.001]. As expected, Roflumilast significantly increased the expression of spinal cAMP. H&E staining exhibited lesser histopathological disruptions in Roflumilast-treated rodents. We also observed a significant reduction in the M1/M2 ratio (p values < 0.001) as well as in pro-inflammatory biomarkers following the administration of Roflumilast to the injured rats. Furthermore, IL-10 level was increased in rodents receiving 1 mg/kg of the reagent. In conclusion, the increased spinal cAMP following Roflumilast therapy might attenuate neuroinflammation via altering microglial activity; therefore, it could be considered as an alternative therapeutic agent for SCI complications.

Medullary involvement in neurosyphilis: a report of 12 cases and a review of the literature.

Study design: Retrospective case series. Objectives: To describe the epidemiological, clinical, MRI and therapeutic features and the outcomes of patients with syphilitic myelitis in a third-level hospital in Marrakesh in southern Morocco. Setting: The Neurology Department, University Hospital Mohamed VI Marrakesh, Morocco. Methods: Twelve charts of persons with syphilitic myelitis over a period of 17 years were reviewed to determine demographics, presenting symptoms, clinical and radiological findings, biological features, treatment received and outcomes. Results: There were 120 reports of neurosyphilis. Twelve patients (10%) had syphilitic myelitis. Eleven patients (92%) were male with mean age of 44 at presentation. Tabes dorsalis was the most common clinical form. Cerebrospinal fluid analysis showed lymphocytic meningitis in nine patients (75%). Spine MRI was abnormal in four patients (33%). All patients were treated with 30 million units of aqueous penicillin G IV per day for 10 days, every 3 months. In follow-up, two patients (17%) with clinical syphilitic meningomyelitis improved significantly, eight patients (66%) with tabes dorsalis and subacute transverse myelitis showed partial improvement but clinical status was stationary for two patients (17%) with Erb paraplegia. Conclusions: All patients with myelopathy should undergo syphilitic serology because of nonspecific manifestations and curability of this disease.

Classical syphilitic lesions from the museum.

Syphilis is an increasingly diagnosed venereal disease which has four distinctive stages that may last over decades if appropriate treatment is not given. Review of the files of the Pathology Museum in the Faculty of Medicine at The University of Adelaide revealed three cases with classical cardiovascular and neurological findings. Case 1: An 80-year-old man with a large syphilitic aneurysm of the ascending aorta with a smaller aneurysm of the proximal descending aorta. Case 2: A 56-year-old man with chronic syphilitic meningoencephalitis with cerebral atrophy. Case 3: A 77-year-old man with tabes dorsalis. Given the increase in cases coming to medical attention in recent years due to high-risk sexual activity, migration, travel and reduced access to medical treatment, an awareness of the classical features of syphilis is appropriate as some cases will undoubtedly require medicolegal evaluation.

Édouard Manet's Tabes Dorsalis: From Painful Ataxia to Phantom Limb.

Édouard Manet (1832-1883) is considered the "father" of impressionism and even of twentieth century modern art. Manet's genius involved getting away from the classical narrative or historical topics and replacing them by the banality of daily life. Technically, he erased volumes into flat two-dimensional coloured planes, and distorted conventional perspective with often gross brushstrokes intentionally giving an "unfinished" aspect to the work. It is little known that Manet had a very painful second part of his life, due to excruciating limb and chest pains, which developed in parallel with proprioceptive ataxia and gait imbalance. Manet always remained discreet about his private life, and we mainly know that his future wife was his family piano teacher, with whom he had a liaison at the age of 17 years. Later, the great but platonic passion of his life was the painter Berthe Morisot (1841-1895), who married Manet's brother Eugène. In fact, we do not know whether he had a mistress at all, although he had several elegant "flirts" in the mundane and artistic milieu. Thus, while Manet's progressive painful ataxia from the age 40 years yields little doubt on its tabetic origin, how he contracted syphilis at least 15-20 years before will probably remain a mystery. It is fascinating that Manet's daily struggle against pain and poor coordination may have led his art to become one of the most significant of modern times, opening the way to twentieth century avant-gardes, along with another victim of syphilis, Paul Gauguin (1848-1903). Manet never showed any sign of general paresis, and like his contemporary, the writer Alphonse Daudet, his clinical picture remained dominated by paroxysmal pain and walking impairment. Difficult hand co-ordination made him quit watercolour painting, and during the last 2 years of his life he had to focus on small format oil works, the subject of which was nearly limited to modest bunches of fresh flowers, now often considered to be his maturity masterpieces. Having become bedridden, he had to be amputated of one leg, which was developing gangrene, probably associated with ergot overuse. While he died shortly thereafter, we have some witness anecdotes suggesting that he experienced a phantom limb: when Claude Monet (1840-1926) visited him and sat down on his bed, Manet violently shouted that he was sitting on his (absent) leg, which provoked terrible pains. With its facts and mysteries, the subtle interaction between Manet's illness and his work output remains one of the most intriguing stories in the neurology of art.

Comparison between self-reported and observed locomotion ability scores in patients with stroke: a cross-sectional study.

This study aimed to investigate (i) the validity of self-reported scores of locomotion ability in stroke survivors using the ABILOCO-Benin questionnaire, and (ii) the congruence between online Rasch analysis of the ABILOCO-Benin raw scores and a conversion table set along with the questionnaire development. Thirty-five adult poststroke patients [mean age: 52 (SD: 10) years, 60% men] recruited from the rehabilitation department of National University Hospital of Cotonou (Benin), self-reported their locomotion ability, filling out the ABILOCO-Benin questionnaire [self-reported scores (SRS)]. Afterwards, a physical therapist observed and rated the patients' locomotion ability, while they performed each of the 15 items of the questionnaire [performance-based scores (PBS)]. Both raw SRS and PBS were then converted to linear measures using (i) an online Rasch analysis method and (ii) an ordinal-to-interval transformation table. Analyses showed high correlation (intraclass correlation coefficient=0.74, P<0.001) and nonsignificant difference (P=0.778) between SRS and PBS. Linear measures from online Rasch and a conversion table also exhibited high correlation (intraclass correlation coefficient=0.92; P<0.001). However, the difference between online analysis and the conversion table was significant (P=0.022). In conclusion, self-report is a valid method to administer ABILOCO-Benin in stroke patients. The conversion table offers a valid opportunity for quick transfer of raw scores to linear measures. However, the accuracy of linear measures from the conversion table may be slightly affected by missing responses.

Syphilitic meningomyelitis presenting with visceral crisis: A case report.

RATIONALE: We report a rare case of syphilitic meningomyelitis presenting with visceral crisis and possessing characteristic imaging findings. PATIENT CONCERNS: The patient, a 50-year-old woman, complained of pain in the upper abdomen and back. She then developed numbness in both lower extremities and weakness in the left lower limb. DIAGNOSIS: Magnetic resonance imaging (MRI) of the spinal cord revealed the candle guttering sign and irregular enhancement at the T6 level. Rapid plasma reagin test of the cerebrospinal fluid yielded a titer of 1:8. Thus, the patient was diagnosed with syphilitic meningomyelitis. INTERVENTIONS: She was treated with ceftriaxone and dexamethasone after the failure of penicillin treatment. OUTCOMES: She could perform the activities of daily living, and her pain completely disappeared. LESSONS: A patient with syphilitic meningomyelitis can present with visceral crisis caused by the involvement of the posterior nerve roots or the posterior horn, which usually occurs in patients with tabes dorsalis. Considering the non-specific symptoms and MRI features, we should be aware that abdominal pain may be a symptom of myelopathy, and syphilitic meningomyelitis ought to be taken into account in a patient with longitudinally extensive myelitis.

DEVELOPMENT OF GANGLIONOPATHY AND TABETIC VISCERAL CRISES ON THE BACKGROUND OF POLYRADICULONEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMOPATHY (CASE REPORT).

The article presents an analysis of the clinical occurrence of development of chronic polyradiculoneuropathy associated with monoclonal IgG/k (kappa) gammopathy of the undetermined significance. The peculiarity of this occurrence is the uniqueness of the development of the symptoms which are characteristic of tabes dorsalis in this pathology with episodic severe visceral crises and also with ganglionopathy. The example describes the clinical polymorphism of the course of visceral crises, the problems of their diagnosis and as a consequence of inadequate treatment with the development of severe social maladaptation. The importance of timely diagnosis and treatment of such conditions is discussed.

Meningovascular neurosyphilis with basilar artery thrombosis in HIV patient.

Currently, neurosyphilis is an uncommon disease and may occur at any stage of syphilis. Co-infection of syphilis with HIV can affect the clinical course. We report a case of meningovascular neurosyphilis in a patient with HIV in whom basilar artery thrombosis and reactive VDRL in CSF were detected. Treatment with penicillin was instituted with adequate response showing clinical improvement. Neurosyphilis is a differential diagnosis in young patients with cerebral infarction, especially in HIV patients.

Longitudinally Extensive Transverse Myelitis and Optic Neuropathy Associated with Syphilitic Meningomyelitis and Human Immunodeficiency Virus Infection: A Case Report and Review of the Literature.

The incidence of co-infection with Treponema pallidum and human immunodeficiency virus (HIV) is increasing in developing and developed countries. The neurological complications of both infections occasionally occur simultaneously during a clinical course. We herein report the case of an HIV carrier with syphilitic meningomyelitis and subclinical optic neuropathy. The patient presumably had latent syphilis and slowly developed longitudinally extensive transverse myelitis (LETM). A cerebrospinal fluid examination confirmed the diagnosis of active neurosyphilis based on an elevated T. pallidum hemagglutination assay index. A change in the patient's immune status, possibly due to HIV, might have converted the syphilis from latent to active, leading to LETM of the spinal cord.

Jules Tinel (1879-1952): Beyond the eponym, the man and his forgotten neurological contributions.

The trauma of World War I had a lasting impact on clinician and physiologist Jules Tinel (1879-1952). His treatment of peripheral nervous system injuries led him, in 1917, to describe the eponymous sign that he linked to activity of the sympathetic nervous system. Among the sequelae of nerve injuries, he was confronted with causalgia that he attributed, here again, to the autonomic nervous system, the main focus of his laboratory research throughout his career. Tinel's sign became so well known that it eclipsed the originality of his seminal descriptions of exertional headache and of hypertensive emergency caused by pheochromocytoma, which could also have been associated with his name. He was always able to marry his clinical practice of neurology and psychiatric consultations with his anatomicopathological, physiological and pathophysiological research, which was based on his daily practice as a physician. At the same time, he directed the work of numerous assistants in his research laboratory, which has since been unjustly forgotten. Several hundreds of scientific publications, including three seminal works, bear witness to his intense activity, which he combined with a genuine talent for teaching and making his findings accessible to a wider public. Those publications alone would fully justify the historical value of extending his renown beyond the existing eponym.

Multifocal neuropathic arthropathy in patient with undiagnosed neurosyphilis: A case report.

Although the prevalence of syphilis has decreased significantly, syphilis is still a common cause of neuropathic arthropathy. Tabetic arthropathy means progressive painless joint destruction that is related to neurosensory deficits caused by syphilis. In general, lower limb joints are involved and gradual swelling and instability of the involved joints are observed. Diagnosis of tabetic arthropathy is difficult as its clinical presentation is not specific and differential diagnosis is wide ranging. Hence, diagnosis of tabetic arthropathy requires clinical suspicion and an appropriate serological test. Laboratory tests for the diagnosis of syphilis include nontreponemal tests and treponemal tests. Conventionally, a nontreponemal test such as the Venereal Disease Research Laboratory test or the Rapid Plasma Reagin test is performed first as a screening test for syphilis, followed by a treponemal test to confirm the positive response found in the screening test. However, the sensitivity and specificity of the serum nontreponemal and treponemal tests for the diagnosis of syphilis are different based on the test type and the syphilis stages. We herein report a case of multifocal neuropathic arthropathy found in a patient whose syphilis was not diagnosed due to a nonreactive screening test result.