Distinctive Characteristics of Thoracolumbar Junction Region Stenosis.

STUDY DESIGN: Retrospective case series and systemic literature meta-analysis. BACKGROUND: Thoracolumbar junction region stenosis produces spinal cord compression just above the conus and may manifest with symptoms that are not typical of either thoracic myelopathy or neurogenic claudication from lumbar stenosis. OBJECTIVE: As few studies describe its specific pattern of presenting symptoms and neurological deficits, this investigation was designed to improve understanding of this pathology. METHODS: A retrospective review assessed surgically treated cases of T10-L1 degenerative stenosis. Clinical outcomes were evaluated with the thoracic Japanese Orthopedic Association score. In addition, a systematic review and meta-analysis was performed in accordance with guidelines provided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). RESULTS: Of 1069 patients undergoing laminectomy at 1477 levels, 31 patients (16M/15F) were treated at T10-L1 a mean age 64.4 (SD=11.8). Patients complained of lower extremity numbness in 29/31 (94%), urinary dysfunction 11/31 (35%), and back pain 11/31 (35%). All complained about gait difficulty and objective motor deficits were detected in 24 of 31 (77%). Weakness was most often seen in foot dorsiflexion 22/31 (71%). Deep tendon reflexes were increased in 10 (32%), decreased in 11 (35%), and normal 10 (32%); the Babinski sign was present 8/31 (26%). Mean thoracic Japanese Orthopedic Association scores improved from 6.4 (SD=1.8) to 8.4 (SD=1.8) ( P <0.00001). Gait subjectively improved in 27/31 (87%) numbness improved in 26/30 (87%); but urinary function improved in only 4/11 (45%). CONCLUSIONS: Thoracolumbar junction stenosis produces distinctive neurological findings characterized by lower extremity numbness, weakness particularly in foot dorsiflexion, urinary dysfunction, and inconsistent reflex changes, a neurological pattern stemming from epiconus level compression and the myelomeres for the L5 roots. Surgery results in significant clinical improvement, with numbness and gait improving more than urinary dysfunction. Many patients with thoracolumbar junction stenosis are initially misdiagnosed as being symptomatic from lumbar stenosis, thus delaying definitive surgery.

Navigation-Assisted Micro-Window Excision of Thoracic Ossification of Ligamentum Flavum (Mishima Surgery) in Professional Baseball Pitchers: A Case Report and Technical Note.

Background and Objectives: Thoracic ossification of the ligamentum flavum (OLF) often causes myelopathy and/or radiculopathy. The disease is frequently observed in East Asian populations. Although thoracic OLF in young athletes who have underwent decompression surgery has been reported, the removal of posterior spinal bony elements and ligamentous complex may often cause postoperative thoracolumbar instability. We established a novel surgical technique that preserves the posterior spinal elements, including the spinous processes, facet joints, and supraspinous and interspinous ligaments for thoracic OLF. This is the first case report to describe a navigation-assisted micro-window excision of thoracic OLF. Case: A 32-year-old male right-handed professional baseball pitcher with significant weakness and numbness in the left leg was referred to our hospital. The patient was diagnosed with thoracic OLF at T10-11 based on radiographic and magnetic resonance images in August 2022. After exposure of the left T10-11 laminae via a small unilateral incision, the location of T10-11 OLF was detected over the lamina by O-arm navigation. Then, the micro-window was made directly above the OLF using a navigated air drill, and the OLF was removed on the ipsilateral side. The contralateral side of OLF was also resected through the same micro-window, achieving complete spinal cord decompression. Results: The next day of the surgery, his leg weakness and numbness were significantly improved. Six weeks after the surgery, he started pitching. Three months after surgery, his symptoms had gone completely, and he pitched from the mound. Approximately 6 months after surgery, he successfully pitched in a professional baseball game. Conclusions: A navigation-assisted micro-window excision of thoracic OLF effectively preserved the spinal posterior bony elements and ligamentous complex. However, long-term clinical outcomes should be evaluated in future studies.

Trigeminal Schwannoma Surgery: Challenges in Preserving Facial Sensation.

Treatments of schwannoma have dramatically improved in the previous few decades, but preservation of the functions of the originating nerve, such as facial sensation in trigeminal schwannomas, still remains challenging. As the preservation of facial sensation in trigeminal schwannomas has not been analyzed in detail, we here review our surgical experience of more than 50 trigeminal schwannoma patients, particularly focusing on their facial sensation. Since the facial sensation in each trigeminal division showed a different perioperative course even in a single patient, we investigated patient-based outcomes (average of the three divisions in each patient) and division-based outcomes separately. In the evaluation of patient-based outcomes, facial sensation remained postoperatively in 96% of all the patients, and improved in 26% and worsened in 42% of patients with preoperative hypesthesia. Posterior fossa tumors tended to most rarely disrupt facial sensation preoperatively, but were the most difficult to preserve facial sensation postoperatively. Facial pain was relieved in all six patients with preoperative neuralgia. In the division-based evaluation, facial sensation remained postoperatively in 83% of all the trigeminal divisions, and improved in 41% and worsened in 24% of the divisions with preoperative hypesthesia. The V3 region was most favorable before and after surgery, with the most frequent improvement and the least frequent functional loss. To clarify current treatment outcomes of the facial sensation and to achieve more effective preservation, standardized assessment methods of perioperative facial sensation may be required. We also introduce detailed MRI investigation methods for schwannoma, including contrast-enhanced heavily T2-weighted (CISS) imaging, arterial spin labeling (ASL), and susceptibility-weighted imaging (SWI), preoperative embolization for rare vascular-rich tumors, and modified techniques of the transpetrosal approach.

Complications after minor salivary gland biopsy: a retrospective study of 630 patients from two Swedish centres.

OBJECTIVES: The aims of the study were to investigate the prevalence of impaired sensation after minor salivary gland biopsy (MSGB) in two Swedish centres [Karolinska University Hospital (KUH) and Skåne University Hospital (SUH)] and to assess its impact on quality of life (QoL) and associated risk factors. METHOD: A questionnaire including questions regarding the presence of impaired sensation, impact on QoL, and impact on everyday life was sent to patients who had undergone MSGB between 2007 and 2016, and their medical notes were scrutinized. RESULTS: The study included 630 patients (505 from KUH and 125 from SUH). In KUH the biopsies were performed by rheumatologists and in SUH by dentists or oral and maxillofacial surgeons (OMSs). Long-standing, probably permanent, impaired sensation after MSGB was reported by 21% of patients, and was associated with lower age and absence of anti-SSA antibodies. Patients with long-standing impaired sensation reported the inconvenience (1-10) of impaired sensation as 4.0 (2.0-7.0) [median (interquartile range)], and 32% reported an influence on their QoL, the reported influence (1-10) on everyday life being 3.0 (1.0-5.0). When comparing the outcomes from KUH and SUH, patients from SUH reported a significantly lower frequency of long-standing impaired sensation (14% vs 23%; p = 0.02). CONCLUSION: A high frequency of long-standing impaired sensation after MSGB was found among patients who had undergone MSGB, although it had a low impact on everyday life. The complication frequency was less pronounced when a dentist or an OMS had performed the biopsy.

Whorling Sclerosing Ependymoma of the Cervical Spinal Cord Presenting Tanycytic Histopathologic Features: A Rare Case Report.

Tanycytic ependymoma is a neuroectodermal tumor that arises from ependymoglial cells or tanycytes. It is highly uncommon. We reported a 34-year-old man who was diagnosed with intradural-intramedullary tanycytic ependymoma, located at the level of C4-5 who had a 9-months history of neck pain and left arm pain, and numbness on fingers. One month prior to presentation, his left arm numbness and paresthesia deteriorated. The lesion was removed totally by C4, C5 hemilaminoplasty. The histologic pattern of this lesion was consisted of fascicles forming nebula-like whorling structures. Because of these structures, tanycytic ependymoma should be taken into consideration in the differential diagnosis of a whorling-sclerosing variant of meningiomas.

Numb chin syndrome: What all oral health care professionals should know.

BACKGROUND: Numb chin syndrome (NCS) is a rare sensory neuropathy involving the mental nerve. Symptoms of NCS are often overlooked because of their apparent innocent nature; however, owing to the frequent association of NCS with malignancies, the opposite should be the rule. Oral health care professionals may be the first to encounter patients with NCS and should be aware of its clinical characteristics in an effort to decrease patient morbidity and mortality. TYPES OF STUDIES REVIEWED: A search in PubMed (MEDLINE) and the Cochrane Library was performed using the terms numb chin syndrome, numb chin, mental neuropathy, mental nerve neuropathy, and malignant mental nerve neuropathy, yielding 2,374 studies. After inclusion and exclusion criteria were applied, 102 studies remained. Descriptive statistics were performed, analyzing the etiology responsible for NCS, characteristics of NCS including associated symptoms, unilateral or bilateral nature, and information on professionals visited and examinations requested to make a diagnosis. RESULTS: NCS was associated with malignancy in 29% through 53% of the published cases. Twenty-eight percent of patients initially consulted an oral health care professional with the symptom of a numb chin. Patients more likely to have NCS were those from the ages of 61 through 70 years; 74% were unilateral; and the most common symptoms reported were numbness (100%), paresthesia (18%), and pain (17%). Forty-seven percent of the NCS cases were associated with a recurrent malignancy, and the most prevalent associated diagnoses were breast cancer (32%) and lymphoma and leukemia (24%). CONCLUSIONS: Oral health care professionals should be aware of the characteristics of NCS as they may be the first health care providers consulted for these symptoms. PRACTICAL IMPLICATIONS: A thorough medical and dental history as well as a complete cranial nerve screening should be performed on all patients, especially those with numbness, as this may prevent misdiagnosis and allow a timely referral and a substantial improvement of treatment course and prognosis.

Lateral Medullary Infarction with Contralateral Segmental Dysesthesia and Ipsilateral Headache: A Case Report.

INTRODUCTION: The medulla oblongata is the lowest segment of the brain stem, located adjacent to the spinal cord, with a complex anatomical structure. Thus, a small injury to the medulla oblongata can show complex clinical manifestations. CASE PRESENTATION: A patient experienced dysesthesia, which manifested as numbness in her right lower limb and decreased temperature sense, and dizziness 20 days before admission. The numbness worsened 1 week before admission, reaching the right Thoracic (T) 12 dermatomes. Her thermoception below the T12 dermatomes decreased, and the degree of dizziness increased, accompanied by nausea and vomiting. Magnetic Resonance Imaging (MRI) of the neck, chest, and abdomen performed at a local hospital showed no abnormalities. MRI of the brain was performed after admission. One week after admission, she experienced a severe headache in the upper left periorbital area. The numbness extended to T4, and thermoception decreased below T4. DIAGNOSIS: Lateral medullary infarction. INTERVENTIONS: Anti-platelet aggregation and mitochondrial nutritional therapies were performed along with treatments for improving circulation and establishing collateral circulation. OUTCOMES: The intensity of limb numbness decreased, and the symptoms of headache and dizziness resolved. CONCLUSION: Lesions leading to segmental sensory disorders can occur in the medulla oblongata. Ipsilateral headaches with contralateral segmental paresthesia can be a specific sign of lateral medullary infarction.

Polineuropatía sensitiva desmielinizante asociada a anticuerpos anti-Yo y cáncer papilar de tiroides / Sensitive demyelinating neuropathy associated with anti-Yo antibodies and papillary thyroid cancer

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A touching story.

A slide taped to a window at the Woods Hole Marine Biology Laboratory was my first introduction to the touch receptor neurons of the nematode Caenorhabditis elegans. Studying these cells as a postdoc with Sydney Brenner gave me a chance to work with John Sulston on a fascinating set of neurons. I would never have guessed then that 43 years later I would still be excited about learning their secrets.

An endothelial growth factor receptor compound mutation of T790M substitution with exon 19 deletion in a previously untreated patient: a case report.

BACKGROUND: Endothelial growth factor receptor (EGFR) mutations are an essential driver of personalized therapy for patients with lung cancer and are detected in approximately 15% of Caucasian and 50% of Asian patients. EGFR tyrosine kinase inhibitors have been developed and used for this set of patients. T790M mutation in exon 20 is usually associated with secondary resistance to EGFR tyrosine kinase inhibitors therapy but is also present in treatment-naïve patients. The frequency for baseline T790M mutation varies from 4 to 35% according to the detection method used. Newer techniques have yielded higher rates, but concerns about false-positive results have been raised. Compound mutations account for 4-14% of all EGFR-mutated tumors, with no studies yet to provide a frequency rate for T790M + 19 deletion association due to the small number of cases. However, there are reports that pretreatment T790M + L858R association is significantly more frequent compared to T790M + exon 19 deletion mutations. Diagnostic challenges, current knowledge on the subject, and therapeutic decisions are discussed. CASE PRESENTATION: We present the case of a 43-year-old Hispanic woman, a treatment-naïve patient, with metastasized lung cancer adenocarcinoma harboring a T790M deletion along with the classic 19 mutation. The initial symptoms were monoparesis of her left leg, associated with hyperreflexia, and hypoesthesia. In the absence of third-generation tyrosine kinase inhibitors, a platinum-based therapy was initiated with no response and she died 4 months after diagnosis. CONCLUSIONS: Osimertinib seems to be a suitable therapy for treatment-naïve patients with sensitizing and resistant compound EGFR mutations. More studies regarding the clinical characteristics of these patients and the appropriate management of this condition are needed to provide the highest standard of care.

Impaired mechanical, heat, and cold nociception in a murine model of genetic TACE/ADAM17 knockdown.

TNF-α-converting enzyme, a member of the ADAM (A disintegrin and metalloproteinase) protease family and also known as ADAM17, regulates inflammation and regeneration in health and disease. ADAM17 targets are involved in pain development and hypersensitivity in animal models of inflammatory and neuropathic pain. However, the role of ADAM17 in the pain pathway is largely unknown. Therefore, we used the hypomorphic ADAM17 (ADAM17ex/ex) mouse model to investigate the importance of ADAM17 in nociceptive behavior, morphology, and function of primary afferent nociceptors. ADAM17ex/ex mice were hyposensitive to noxious stimulation, showing elevated mechanical thresholds as well as impaired heat and cold sensitivity. Despite these differences, skin thickness and innervation were comparable to controls. Although dorsal root ganglia of ADAM17ex/ex mice exhibited normal morphology of peptidergic and nonpeptidergic neurons, a small but significant reduction in the number of isolectin ß-4-positive neurons was observed. Functional electrical properties of unmyelinated nociceptors showed differences in resting membrane potential, afterhyperpolarization, and firing patterns in specific subpopulations of sensory neurons in ADAM17ex/ex mice. However, spinal cord morphology and microglia activity in ADAM17ex/ex mice were not altered. Our data suggest that ADAM17 contributes to the processing of painful stimuli, with a complex mode of action orchestrating the function of neurons along the pain pathway.-Quarta, S., Mitric, M., Kalpachidou, T., Mair, N., Schiefermeier-Mach, N., Andratsch, M., Qi, Y., Langeslag, M., Malsch, P., Rose-John, S., Kress, M. Impaired mechanical, heat, and cold nociception in a murine model of genetic TACE/ADAM17 knockdown.

Deletion of Tsc2 in Nociceptors Reduces Target Innervation, Ion Channel Expression, and Sensitivity to Heat.

The mechanistic target of rapamycin complex 1 (mTORC1) is known to regulate cellular growth pathways, and its genetic activation is sufficient to enhance regenerative axon growth following injury to the central or peripheral nervous systems. However, excess mTORC1 activation may promote innervation defects, and mTORC1 activity mediates injury-induced hypersensitivity, reducing enthusiasm for the pathway as a therapeutic target. While mTORC1 activity is required for full expression of some pain modalities, the effects of pathway activation on nociceptor phenotypes and sensory behaviors are currently unknown. To address this, we genetically activated mTORC1 in mouse peripheral sensory neurons by conditional deletion of its negative regulator Tuberous Sclerosis Complex 2 (Tsc2). Consistent with the well-known role of mTORC1 in regulating cell size, soma size and axon diameter of C-nociceptors were increased in Tsc2-deleted mice. Glabrous skin and spinal cord innervation by C-fiber neurons were also disrupted. Transcriptional profiling of nociceptors enriched by fluorescence-associated cell sorting (FACS) revealed downregulation of multiple classes of ion channels as well as reduced expression of markers for peptidergic nociceptors in Tsc2-deleted mice. In addition to these changes in innervation and gene expression, Tsc2-deleted mice exhibited reduced noxious heat sensitivity and decreased injury-induced cold hypersensitivity, but normal baseline sensitivity to cold and mechanical stimuli. Together, these data show that excess mTORC1 activity in sensory neurons produces changes in gene expression, neuron morphology and sensory behavior.

Numb chin syndrome: A harbinger of tumor progression or relapse.

Numb chin syndrome (NCS) is a rare yet potentially ominous sensory neuropathy in the distribution of the mental or inferior alveolar nerve characterized by unilateral hypoesthesia over the lower lip, chin and occasionally gingival mucosa. Recognizing NCS is clinically important as this may be a subtle sign of occult malignancy progression or heralding the relapses. It may also occur in benign disease, both systemic and dental in origin. Current expert opinion is that patients with NCS without apparent cause should be assumed to have a malignant etiology until proven otherwise Lossos and Siegal (1992) [1]. Here we report a relapse of Non-Hodgkin lymphoma with NCS with no evidence of metastasis.

68Ga PSMA PET/CT in a Rare Case of Metastatic Adenocarcinoma Prostate Presenting as Numb Chin Syndrome.

A 68-year-old man presented with right-side facial numbness. MRI showed an extra-axial mass infiltrating the right temporal bone. It was debulked surgically, and histopathology revealed metastatic adenocarcinoma. Ga PSMA PET/CT done in view of increased PSA levels and clinically suspicious hard lesion in prostate showed primary lesion in left side of prostate with metastases to the right temporal bone. Primary carcinoma of the prostate and metastases to the right temporal bone were proven histopathologically. Our case highlights the usefulness of Ga PSMA PET/CT in identifying the primary site in suspected prostate cancer and mapping the metastatic sites.

Squamous cell carcinoma presenting with trigeminal anesthesia: An uncommon presentation of head & neck cancer with unknown primary.

BACKGROUND: The differential diagnosis of facial anesthesia is vast. This may be secondary to trauma, neoplasm, both intracranial and extracranial, infection, and neurologic disease. When evaluating a patient with isolated facial anesthesia, the head and neck surgeon often thinks of adenoid cystic carcinoma, which has a propensity for perineural invasion and spread. When one thinks of head and neck squamous cell carcinoma with or without unknown primary, the typical presentation involves dysphagia, odynophagia, weight loss, hoarseness, or more commonly, a neck mass. Squamous cell carcinoma presenting as facial anesthesia and perineural spread, with no primary site is quite rare. METHODS: Case presentations and review of the literature. CONCLUSIONS: Trigeminal anesthesia is an uncommon presentation of head and neck squamous cell carcinoma with unknown primary. We present two interesting cases of invasive squamous cell carcinoma of the trigeminal nerve, with no primary site identified. We will also review the literature of head and neck malignancies with perineural spread and the management techniques for the two different cases presented.

Mycolactone-mediated neurite degeneration and functional effects in cultured human and rat DRG neurons: Mechanisms underlying hypoalgesia in Buruli ulcer.

BACKGROUND: Mycolactone is a polyketide toxin secreted by the mycobacterium Mycobacterium ulcerans, responsible for the extensive hypoalgesic skin lesions characteristic of patients with Buruli ulcer. A recent pre-clinical study proposed that mycolactone may produce analgesia via activation of the angiotensin II type 2 receptor (AT2R). In contrast, AT2R antagonist EMA401 has shown analgesic efficacy in animal models and clinical trials for neuropathic pain. We therefore investigated the morphological and functional effects of mycolactone in cultured human and rat dorsal root ganglia (DRG) neurons and the role of AT2R using EMA401. Primary sensory neurons were prepared from avulsed cervical human DRG and rat DRG; 24 h after plating, neurons were incubated for 24 to 96 h with synthetic mycolactone A/B, followed by immunostaining with antibodies to PGP9.5, Gap43, ß tubulin, or Mitotracker dye staining. Acute functional effects were examined by measuring capsaicin responses with calcium imaging in DRG neuronal cultures treated with mycolactone. RESULTS: Morphological effects: Mycolactone-treated cultures showed dramatically reduced numbers of surviving neurons and non-neuronal cells, reduced Gap43 and ß tubulin expression, degenerating neurites and reduced cell body diameter, compared with controls. Dose-related reduction of neurite length was observed in mycolactone-treated cultures. Mitochondria were distributed throughout the length of neurites and soma of control neurons, but clustered in the neurites and soma of mycolactone-treated neurons. Functional effects: Mycolactone-treated human and rat DRG neurons showed dose-related inhibition of capsaicin responses, which were reversed by calcineurin inhibitor cyclosporine and phosphodiesterase inhibitor 3-isobutyl-1-Methylxanthine, indicating involvement of cAMP/ATP reduction. The morphological and functional effects of mycolactone were not altered by Angiotensin II or AT2R antagonist EMA401. CONCLUSION: Mycolactone induces toxic effects in DRG neurons, leading to impaired nociceptor function, neurite degeneration, and cell death, resembling the cutaneous hypoalgesia and nerve damage in individuals with M. Ulcerans infection.

Increased fronto-hippocampal connectivity in the Prrxl1 knockout mouse model of congenital hypoalgesia.

Despite the large number of studies addressing how prolonged painful stimulation affects brain functioning, there are only a handful of studies aimed at uncovering if persistent conditions of reduced pain perception would also result in brain plasticity. Permanent hypoalgesia induced by neonatal injection of capsaicin or carrageenan has already been shown to affect learning and memory and to induce alterations in brain gene expression. In this study, we used the Prrxl1 model of congenital mild hypoalgesia to conduct a detailed study of the neurophysiological and behavioral consequences of reduced pain experience. Prrxl1 knockout animals are characterized by selective depletion of small diameter primary afferents and abnormal development of the superficial dorsal laminae of the spinal cord, resulting in diminished pain perception but normal tactile and motor behaviour. Behavioral testing of Prrxl1 mice revealed that these animals have reduced anxiety levels, enhanced memory performance, and improved fear extinction. Neurophysiological recordings from awake behaving Prrxl1 mice show enhanced altered fronto-hippocampal connectivity in the theta- and gamma-bands. Importantly, although inflammatory pain by Complete Freund Adjuvant injection caused a decrease in fronto-hippocampal connectivity in the wild-type animals, Prrxl1 mice maintained the baseline levels. The onset of inflammatory pain also reverted the differences in forebrain expression of stress- and monoamine-related genes in Prrxl1 mice. Altogether our results suggest that congenital hypoalgesia may have an effect on brain plasticity that is the inverse of what is usually observed in animal models of chronic pain.

Ictus talámico secundario a disección vertebral tras la práctica de yoga / Thalamic stroke secondary to vertebral dissection after doing yoga

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