Carcinoma, Merkel Cell/pathology* ; Humans ; Skin Neoplasms/pathology* ; Tongue ; Tongue Diseases

Objective: To present the case of a midline Tessier 30 cleft in a baby boy who initially underwent a glossoplasty, cheiloplasty and mentoplasty. Methods: Design: Case Report. Setting: Tertiary Government Training Hospital. Patient: One. Result: A 4-month-old boy with a complete midline cleft of the lower lip, alveolus and mandible, and bifid distal tongue that was fused with the floor of the mouth, underwent glossoplasty, cheiloplasty and mentoplasty with subsequent excellent aesthetic outcome and normal oral competency. Conclusion Tessier 30 is a rare congenital midline mandibular cleft. Prompt glossoplasty, cheiloplasty and mentoplasty can correct the gross deformity, restore oral competency, and address functional needs such as feeding, swallowing and early speech development. Future bony repair will hopefully complete the reconstruction.
Mandible ; Cleft Lip ; Tongue Diseases ; Jaw Abnormalities ; Plastic Surgery Procedures

Cheilitis granulomatosa (CG) is a subset of orofacial granulomatosis (OFG) and considered to be a monosymptomatic form of Melkersson-Rosenthal syndrome (MRS), which is characterized by the triad of chronic lip swelling, facial paralysis, and fissured tongue. The labial swelling is thought to be associated with an orofacial swelling, which affects the chin, cheeks, and oral mucosa. Histologically, it is distinguished by noncaseating granulomas consisting of lymphohistiocytes and giant cells. Although systemic steroids with or without intralesional triamcinolone injections are the mainstay of treatment, and various agents have been proposed for this rare disease, no successful treatment modality has been reported in the literature yet. Herein, we present our experience with three different CG cases, which showed a varied level of positive response to the combination therapy.
Cheek ; Cheilitis ; Chin ; Facial Paralysis ; Giant Cells ; Granuloma ; Granulomatosis, Orofacial ; Lip ; Melkersson-Rosenthal Syndrome ; Mouth Mucosa ; Rare Diseases ; Steroids ; Tongue, Fissured ; Triamcinolone

Corticobasal degeneration (CBD) is a rare neurodegenerative disease characterized by dystonia, cognitive deficits, and an asymmetric akinetic-rigid syndrome. Little information is available regarding anesthetic management for CBD patients. Our patient was a 55-year-old man with CBD complicated by central sleep apnea (CSA). Due to the risk of perioperative breathing instability associated with anesthetic use, a laryngeal mask airway was used during anesthesia with propofol. Spontaneous respiration was stable under general anesthesia. However, respiratory depression occurred following surgery, necessitating insertion of a nasopharyngeal airway. Since no respiratory depression had occurred during maintenance of the airway using the laryngeal mask, we suspected an upper airway obstruction caused by displacement of the tongue due to residual propofol. Residual anesthetics may cause postoperative respiratory depression in patients with CBD. Therefore, continuous postoperative monitoring of SpO₂ and preparations to support postoperative ventilation are necessary.
Airway Obstruction ; Anesthesia ; Anesthesia, General ; Anesthetics ; Cognition Disorders ; Dystonia ; Humans ; Laryngeal Masks ; Middle Aged ; Neurodegenerative Diseases ; Propofol ; Respiration ; Respiratory Insufficiency ; Sleep Apnea Syndromes ; Sleep Apnea, Central ; Tongue ; Ventilation

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare disease that affects the sensory and autonomic nervous system. The patients do not have the ability to sense different sensations, such as pain, which tends to lead to different injuries. In addition, the patients suffer from fluctuations in body temperature due to autonomic involvement. The present case was a five-year-old girl with a neglected distal femur fracture. X-rays taken during the follow-up showed marked callus formation and pseudarthrosis of the distal femur. She had biting injuries of the tongue, auto-amputation of the fingers, some developmental delay and a history of recurrent fever with an unknown origin. The electrodiagnostic study was normal. The quantitative sudomotor axon reflex test revealed markedly reduced postganglionic sudomotor axonal responses at all sites recorded on the left. She was diagnosed with CIPA. As the initial presentation of CIPA involves the musculoskeletal system, orthopedic surgeons should have a high index of suspicion.
Autonomic Nervous System ; Axons ; Body Temperature ; Bony Callus ; Female ; Femur ; Fever ; Fingers ; Follow-Up Studies ; Hereditary Sensory and Autonomic Neuropathies ; Humans ; Musculoskeletal System ; Orthopedics ; Pain Insensitivity, Congenital ; Pseudarthrosis ; Rare Diseases ; Reflex ; Sensation ; Surgeons ; Tongue

BACKGROUND: Age-related low-grade inflammation causing endothelial disruption influences sarcopenia, hypertension, and atherosclerosis. We reported previously that maintenance of muscle strength in elderly hypertensive men with high platelet levels is positively associated with subclinical atherosclerosis but not in those with low platelet levels. Since reduced tongue pressure is related to sarcopenia, tongue pressure may be associated with subclinical carotid atherosclerosis in hypertensive elderly subjects, and platelet levels may function as an indicator of the association between tongue pressure and subclinical carotid atherosclerosis. METHODS: We conducted a cross-sectional study of 342 hypertensive elderly Japanese men aged 60-89 who participated in an annual health check-up in 2015 and 2016. Subclinical carotid atherosclerosis was defined as a common carotid intima-media thickness (CIMT) of 1.1 mm or more. RESULTS: In the overall study population, 171 subjects demonstrated low platelets (< 21.4 × 10/μL). Tongue pressure was significantly inversely associated with subclinical carotid atherosclerosis in these subjects, but not in subjects with high platelets. The known cardiovascular risk factor adjusted odds ratios (ORs) and 95% confidence intervals (CIs) of subclinical carotid atherosclerosis for a 1 standard deviation (SD) increment in tongue pressure (10.4 kPa) were 0.54 (0.35, 0.85) and 1.31 (0.87, 1.96), respectively. CONCLUSION Tongue pressure is inversely associated with subclinical carotid atherosclerosis in hypertensive elderly men with low platelet levels, but not in those with high levels. This finding may thus constitute an efficient tool for clarifying the background mechanism of age-related diseases such as sarcopenia, hypertension, and atherosclerosis.
Aged ; Aged, 80 and over ; Aging ; blood ; physiology ; Carotid Artery Diseases ; blood ; physiopathology ; Carotid Intima-Media Thickness ; Confidence Intervals ; Cross-Sectional Studies ; Humans ; Hypertension ; blood ; physiopathology ; Japan ; Male ; Middle Aged ; Odds Ratio ; Platelet Count ; Pressure ; Risk Factors ; Tongue ; physiology

Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is a peripheral neuropathy characterized by multifocal weakness and associated sensory impairment. MADSAM is associated with multifocal persistent conduction block and other signs of demyelination. The incidence of cranial nerve involvement in MADSAM was recently reported to be approximately 15%. However, reports of hypoglossal neuropathy occurring in MADSAM are rare. Unilateral hypoglossal neuropathy in MADSAM is usually misdiagnosed as motor neuron disease. We report a patient with MADSAM presenting with tongue hemiatrophy.
Cranial Nerves ; Demyelinating Diseases ; Diagnosis, Differential ; Humans ; Hypoglossal Nerve Diseases ; Incidence ; Motor Neuron Disease ; Motor Neurons ; Peripheral Nervous System Diseases ; Tongue

Collet-Sicard syndrome is a rare syndrome that involves paralysis of 9th to 12th cranial nerves. We report an uncommon case of schwannoma of the hypoglossal nerve in a 39-year-old woman presented with slurred speech, hoarse voice, and swallowing difficulty. Physical examination revealed decreased gag reflex on the right side, decreased laryngeal elevation, tongue deviation to the right side, and weakness of right trapezius muscle. MRI revealed a mass lesion in the right parapharyngeal space below the jugular foramen. The tumor was surgically removed. It was confirmed as hypoglossal nerve schwannoma via pathologic examination. Videofluoroscopic swallowing study revealed aspiration of liquid food and severe bolus retention in the vallecula and piriform sinus. Laryngoscopy revealed right vocal cord palsy. Electrodiagnostic study revealed paralysis of the right 11th cranial nerve. In summary, we report an uncommon case of schwannoma of the hypoglossal nerve with 9th to 12th cranial nerve palsy presenting as Collet-Sicard syndrome.
Adult ; Cranial Nerve Diseases ; Cranial Nerves ; Deglutition ; Female ; Humans ; Hypoglossal Nerve* ; Laryngoscopy ; Magnetic Resonance Imaging ; Neurilemmoma* ; Paralysis ; Physical Examination ; Pyriform Sinus ; Reflex ; Superficial Back Muscles ; Tongue ; Vocal Cord Paralysis ; Voice

BACKGROUND/AIMS: Functional gastrointestinal disorders (FGIDs), diagnosed by symptom-based criteria due to lack of biomarkers, need translated-validated questionnaires in different languages. As Bengali, the mother tongue of Bangladesh and eastern India, is the seventh most spoken language in the world, we translated and validated the Enhanced Asian Rome III questionnaire (EAR3Q) in this language. METHODS: The EAR3Q was translated in Bengali as per guideline from the Rome Foundation. The translated questionnaire was validated prospectively on Bengali-speaking healthy subjects (HS, n = 30), and patients with functional dyspepsia (FD, n = 35), irritable bowel syndrome (IBS, n = 40) and functional constipation (FC, n = 12) diagnosed by clinicians using the Rome III criteria. The subjects were asked to fill-in the questionnaire again after 2 weeks, to check for its reproducibility. RESULTS: During translation, the original and the backward translated English versions of the questionnaire demonstrated high concordance. Sensitivity of the Bengali questionnaire to diagnose patients with FD, IBS, FC, and HS was 100%, 100%, 75%, and 100%, respectively, considering diagnosis by the clinicians as the gold standard. On test-retest reliability analysis, Kappa values for FD, IBS, FC, and HS were 1.0, 1.0, 0.83, and 1.0, respectively. The Bengali questionnaire detected considerable overlap of FD symptoms among patients with IBS, IBS among patients with FD, and FD among patients with FC, which were not detected by the clinicians. CONCLUSIONS: We successfully translated and validated the EAR3Q in Bengali. We believe that this translated questionnaire will be useful for clinical evaluation and research on FGIDs in the Bengali-speaking population.
Asian Continental Ancestry Group* ; Bangladesh ; Biomarkers ; Constipation ; Diagnosis* ; Dyspepsia ; Gastrointestinal Diseases* ; Humans ; India ; Irritable Bowel Syndrome ; Mothers ; Prospective Studies ; Tongue

We report a 57-year-old man with bilateral cranial nerve IX and X palsies who presented with severe dysphagia. After a mild head injury, the patient complained of difficult swallowing. Physical examination revealed normal tongue motion and no uvular deviation. Cervical X-ray findings were negative, but a brain computed tomography revealed a skull fracture involving bilateral jugular foramen. Laryngoscopy indicated bilateral vocal cord palsy. In a videofluoroscopic swallowing study, food residue remained in the vallecula and pyriform sinus, and there was reduced motion of the pharynx and larynx. Electromyography confirmed bilateral superior and recurrent laryngeal neuropathy.
Brain ; Brain Injuries* ; Cranial Nerve Diseases ; Craniocerebral Trauma ; Deglutition ; Deglutition Disorders ; Electromyography ; Glossopharyngeal Nerve ; Humans ; Laryngoscopy ; Larynx ; Middle Aged ; Paralysis* ; Pharynx ; Physical Examination ; Pyriform Sinus ; Skull Fracture, Basilar ; Skull Fractures ; Tongue ; Vocal Cord Paralysis