A case report of refractory otalgia after Ramsay Hunt syndrome successfully treated by applying pulsed radiofrequency to the great auricular nerve: A CARE-compliant article

Rationale:Ramsay Hunt syndrome is a type of herpes zoster infection involving geniculate ganglion and facial nerve. Unilateral facial palsy, otalgia, and painful vesicular rash on the auricle and external auditory canal are the typical symptoms. Although postherpetic neuralgia (PHN) is a devastating complication of herpes zoster infection, PHN following Ramsay Hunt syndrome has rarely been reported.Patient concerns:A 55-year-old immunocompetent female patient visited our pain clinic, for left-sided refractory otalgia (PHN) that persisted for 3 months after she was diagnosed with Ramsay Hunt syndrome. Although facial palsy and tinnitus had recovered within 2 to 4 weeks after symptom onset, the patient had been experiencing a persistent and severe otalgia radiating to mandibular angle, temporal and upper cervical area of neuropathic nature.Diagnoses:The patient''s pain persisted despite conservative medication and administration of ultrasound-guided stellate ganglion block, facial nerve block, and great auricular nerve block several times.Interventions:The patient was treated with the application of ultrasound-guided pulsed radiofrequency (PRF) to the great auricular nerve.Outcomes:The patient experienced significant pain reduction more than 50% on a numeric rating scale after 2 weeks of PRF treatment.Lessons:Chronic otalgia might be a type of PHN after Ramsay Hunt syndrome with cervical nerve involvement. PRF treatment to the great auricular nerve can be a therapeutic option for refractory otalgia following Ramsay Hunt syndrome.     

Transient oculomotor paralysis after cerebral angiography: A case report

Rationale:A special case of transient oculomotor nerve palsy after cerebral angiography.Patient concerns:A 55-year-old man developed oculomotor nerve dysfunction after right radial artery puncture angiography.Diagnoses:Cerebral angiography-induced oculomotor nerve palsy.Interventions:According to the patient''s disease state, intravenous drip of dexamethasone 10 mg/d.Outcomes:Magnetic resonance imaging (MRI) showed no abnormalities, and the patient recovered completely after 48 hours of hormone therapy.Lessons:Transient eye palsy caused by contrast agent encephalopathy is a clinically rare neurological dysfunction caused by adverse effects of contrast agents. Early prevention and correct treatment are critical.     

Investigating the correlation between serum albumin level and the prognosis of Bell's palsy

To investigate the correlation between the serum albumin level and the prognosis of patients with Bell''s palsy.We retrospectively analyzed the clinical records of 311 inpatients with Bell''s palsy (BP) in our hospital between September 2018 and October 2019. The patients were divided into 2 groups: the recovered group (with the House-Brackmann grade ≤ 2) and the unrecovered group (with the House-Brackmann grade > 2), according to the follow-up results within 3 months after discharge. Blood test indicators (white blood cell count, neutrophil-to-lymphocyte ratio, red cell distribution width, serum albumin level, globulin level) and basic clinical data (age, sex, course of the disease, inpatient days, comorbidity of hypertension, diabetes, and hepatitis B) of the 2 groups were compared to explore whether they were correlated with the prognosis of patients with Bell''s palsy.The serum albumin level of patients with BP in the unrecovered group was significantly lower than that of the recovered group (medians [interquartile range], 40.75 [38.40, 43.85] vs 44 [42.10, 46.20], P < .001). Multivariate binary logistic regression revealed that serum albumin (odds ratio 0.772, 95% confidence interval 0.711–0.839, P < .001) was a protective factor for BP prognosis.Serum albumin is a protective factor for the prognosis of BP. Although more prospective clinical controlled trials are needed, our study provides valuable and crucial prognostic information for physicians.     

Dementia with Lewy Bodies with Pure Agraphia for Kanji (Japanese Morphograms)

A 70-year-old dextral woman was admitted to a hospital with agraphia for kanji (Japanese morphograms). She had a history of severe constipation, nightmares, and visual hallucinations. Neurological examinations revealed no obvious Parkinson''s disease symptoms. She showed poor skills in writing the kanji for looking at picture objects, [e.g., writing the Japanese word “inu” (which means dog) when she saw a drawing of a dog] or dictated words. A reduced striatal uptake of [¹²³I]-2β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (FP-CIT) on single-photon-emission computed tomography and reduced meta-iodobenzylguanidine (MIBG) cardiac uptake on myocardial scintigraphy were detected. The accumulation of amyloid beta in the bilateral cerebral cortices was observed on amyloid-positron emission tomography. We herein report a case of Lewy body dementia with pure agraphia for kanji with underlying Alzheimer''s disease pathology.     

Sarcoidosis in a patient presenting with clinical and histological features of primary Sj?gren's syndrome.

A patient presenting with bilateral enlargement of parotid and lacrimal glands, xerostomia, and keratoconjunctiva sicca, whose labial biopsy specimen showed changes consistent with Sjögren''s syndrome, is described. The patient was initially misdiagnosed as having primary Sjögren''s syndrome (SS). Subsequent investigations, however, performed to exclude an associated lymphoma or sarcoidosis, showed histological changes of the latter. The possibility that early infiltrates of the salivary glands in sarcoid may mimic those of SS is discussed.     

PRISMA—Extracapsular Dissection Versus Superficial Parotidectomy in Treatment of Benign Parotid Tumors: Evidence From 3194 Patients

Benign parotid tumor is one of the most common neoplasms in head and neck region. Its therapeutic methods have been debatable topics over the past 100 years. Recently, some surgeons suggest that extracapsular dissection (ECD) instead of superficial parotidectomy (SP) for treatment of benign parotid tumor. This study aimed to compare ECD with SP in the treatment of benign parotid tumors by a meta-analysis.We searched Cochrane Library, PubMed, Embase, Ovid, and Web of Science databases on February 14, 2015 for studies that assessed clinical outcomes of SP and ECD as surgical techniques for the management of benign parotid tumors. Outcome data were evaluated by pooled risk ratio (RR) and corresponding 95% confidence interval (CI).After serious scrutiny, a total of 14 cohort studies with 3194 patients were included in this meta-analysis. The pooled RR revealed that there were no significant difference in tumor recurrence rate between ECD and SP (fixed-effect model: RR = 0.71, 95% CI = 0.40–1.27, P = 0.249; random-effect model: RR = 0.67, 95% CI = 0.38–1.23, P = 0.197). However, there were significantly lower incidences of transient facial nerve dysfunction (FND), permanent FND, and Frey''s syndrome in patients of ECD group compared with SP group.ECD might be a good choice in treatment of the benign parotid tumor that were mobile, small, located in superficial lobe and without adhesion to facial nerve; ECD should be performed by the experienced surgeons with ability of dissection facial nerve, who should perform SP if tumor is found adhere to facial nerve during an operation; and a multicenter randomized control trial study is necessary to decide the optimal treatment of benign parotid tumor.     

Lambl's Excrescence,Migrainous Headaches,and “Tiger Stripes”: Puzzling Findings in One Patient

Lambl''s excrescences are mobile, thin, filiform structures that occur at sites of valve closure. Even though many clinicians consider them to be part of the normal aging process, evidence suggests an association between Lambl''s excrescences and ischemic stroke, migrainous headaches, and acute coronary syndromes. As a thin filamentous structure, a Lambl''s excrescence is better detected and characterized with transesophageal than with transthoracic echocardiography. Intracardiac oscillatory structures can also be seen as “tiger stripes” on spectral pulsed Doppler echocardiographic recordings. Herein, we present the case of a 68-year-old woman who had 3 concurrent enigmatic findings with unclear correlation: migrainous headaches, Lambl''s excrescences, and the uncommon finding of “tiger stripes” on spectral Doppler echocardiography. We discuss the possible correlation between these 3 findings and review the available literature on Lambl''s excrescences and tiger stripes.     

Sicca syndrome in patients with sarcoidosis

Out of 134, 12 sarcoidosis patients with symptoms of mucosal dryness as the first clinical manifestation were identified and compared with 30 consecutive unselected Sjögren's syndrome (SS) patients. Sicca manifestations were similar among the two groups, while parotid gland enlargement (PGE) was more frequently found in sarcoidosis patients (P<0.05). Patients with sarcoidosis had mainly pulmonary (P<0.001) and skin involvement (P<0.05), while SS patients presented more frequently with Raynaud's phenomenon (P<0.05). Autoantibody profile was more often found in SS patients compared to sarcoidosis (P<0.0025). The histopathological findings of minor salivary gland biopsy (MSGB) revealed noncaseating granulomas (NCG) in 58% of patients with sarcoidosis, while in SS, MSGB showed focal sialadenitis in the majority of the patients. Transbronchial lung biopsy (TBLB), which was performed in 10 sarcoidosis patients, revealed the presence of NCG in all patients. In patients with sarcoidosis and sicca symptoms as the presenting syndrome, PGE is a useful clinical finding. Searching for pulmonary involvement is a determining factor to differentiate sarcoidosis from SS. The absence of autoantibodies is another useful tool for the diagnosis of sarcoidosis. Finally, MSGB is very helpful to discriminate between sarcoidosis and SS and when MSGB is not specific, then TBLB is valuable to confirm the diagnosis.     

A bibliometric analysis of research on the treatment of facial nerve palsy

BackgroundThere are various treatments for facial nerve palsy, and research into this topic is ongoing. In the present study, we carried out bibliometric and visualized analyses to identify the trends of research into facial nerve palsy treatment.MethodsTo identify articles, the SCOPUS database was searched for articles published from its inception to December 27, 2020. The search was conducted twice, with Search 1 investigating general treatment trends and Search 2 narrowing the scope to complementary and alternative treatment. The extracted keywords were analyzed using the Visualization Of Similarities (VOS) viewer. Through analysis of keywords, research hotspots in the treatment of facial nerve palsy were identified.ResultsA total of 1609 and 223 articles were identified in Searches 1 and 2, respectively. The number of articles published each year showed a tendency to increase, and most of the studies were only conducted in a few countries. In terms of subject area, “medicine” was overwhelmingly the most common(77.6%). Based on the analysis of 316 keywords in Search1, “medication treatment,” and “complementary and alternative treatment” were the hotspots of research.ConclusionThis study provides the overall trends of facial nerve palsy treatment. To date, research on medication treatment has been main focus, and antiviral use among medication treatment and complementary and alternative treatment has emerged in recent years.     

Musculoskeletal involvement in sarcoidosis,

Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It most commonly affects the pulmonary system but can also affect the musculoskeletal system, albeit less frequently. In patients with sarcoidosis, rheumatic involvement is polymorphic. It can be the presenting symptom of the disease or can appear during its progression. Articular involvement is dominated by nonspecific arthralgia, polyarthritis, and Löfgren''s syndrome, which is defined as the presence of lung adenopathy, arthralgia (or arthritis), and erythema nodosum. Skeletal manifestations, especially dactylitis, appear mainly as complications of chronic, multiorgan sarcoidosis. Muscle involvement in sarcoidosis is rare and usually asymptomatic. The diagnosis of rheumatic sarcoidosis is based on X-ray findings and magnetic resonance imaging findings, although the definitive diagnosis is made by anatomopathological study of biopsy samples. Musculoskeletal involvement in sarcoidosis is generally relieved with nonsteroidal anti-inflammatory drugs or corticosteroids. In corticosteroid-resistant or -dependent forms of the disease, immunosuppressive therapy, such as treatment with methotrexate or anti-TNF-α, is employed. The aim of this review was to present an overview of the various types of osteoarticular and muscle involvement in sarcoidosis, focusing on their diagnosis and management.     

Nodular Pulmonary Amyloidosis Associated with Sjögren's Syndrome

Amyloidosis is a rare disease characterized by the deposition of abnormal proteins in extracellular tissues. We herein report a case with instructive radiologic features of nodular pulmonary amyloidosis associated with Sjögren''s syndrome. A 67-year-old woman was referred to our department because of an abnormal chest radiograph. Chest computed tomography revealed multiple round cysts accompanied by calcified nodules. The patient was clinically diagnosed with primary Sjögren''s syndrome and pathologically diagnosed with nodular pulmonary amyloidosis (light chain, kappa). Although multiple lung cysts have many etiologies, the presence of calcified nodules associated with multiple lung cysts is useful for narrowing down the differential diagnosis.     

Abducens nerve palsy and Horner syndrome due to metastatic tumor in the cavernous sinus

A 41-year-old man was diagnosed as having primary parotid carcinoma on the right side. After radical parotidectomy, radiation therapy and systemic chemotherapy, the primary parotid carcinoma was completely remitted. Two years later, right abducens nerve palsy and Horner syndrome appeared. Neuroimaging demonstrated a gadolinium-enhanced lesion in the posterior portion of the right cavernous sinus, and metastasis of parotid carcinoma was suspected. After radiosurgery and systemic chemotherapy, the intracavernous lesion disappeared. This is the first case of combination of abducens nerve palsy and ipsilateral Horner syndrome due to metastasis from parotid carcinoma to the cavernous sinus.     

Necrotizing sarcoid granulomatosis simulating pulmonary malignancy: A case report

Rationale:Necrotizing sarcoid granulomatosis (NSG) has recently been termed “sarcoidosis with NSG pattern” for the disease entity representing nodular sarcoidosis with granulomatous pulmonary angiitis. It is characterized by sarcoid-like granulomas, vasculitis, and a variable degree of necrosis. Its rarity and nonspecific clinical symptoms can easily lead to misdiagnosis or delayed diagnosis.Patient concerns:We report a 67-year-old female with a biopsy-confirmed sarcoidosis with NSG pattern mimicking pulmonary malignancy on initial chest computed tomography scan.Diagnoses:Sarcoidosis with NSG pattern.Interventions:The patient underwent video-assisted thoracoscopic surgery with a lung biopsy. No further treatment was performed after the lung biopsy.Outcomes:Follow-up imaging studies revealed spontaneous regression of the disease after 2 months.Lessons:Awareness of this rare benign disease entity and overlapping radiologic manifestations with pulmonary malignancy or other granulomatous diseases can be helpful for making a precise diagnosis with a better differential diagnosis.     

Reoperation in an adult female with "right-sided" Hirschsprung's disease complicated by refractory hypertension and cough

Hirschsprung’s disease (HD) is an intestinal malformation caused by the innate absence of ganglion cells in the neural plexus of the colorectal wall, and is most common in male infants. It is rare in adult, and is usually left-sided. Herein we reported based on the CARE guidelines a case of a 47-year-old adult female suffering from “right-sided” HD complicated by refractory hypertension and cough. The patient with a history of cesarean section and with digestive unfitness (abdominal pain, distention, and constipation) only since 20 years old had recurrence of HD after initial surgery due to the incomplete removal of the HD-affected bowel based on a diagnosis of “chronic ileus”, leading to the relapse of the digestive symptoms and the emergence of some intractable circulatory and respiratory complications which could be hardly controlled by conservative treatment. During the long interval before coming to our department for help, she had been re-hospitalized for several times with various misdiagnoses and supplied merely with symptomatic treatment which could only achieve temporary symptomatic relief. At her admission to our department, the imaging examinations strongly indicated recurrent HD which was further supported by pathological examinations, and right hemi-colectomy was performed to remove the remnant aganglionic intestinal segment. Intraoperative and postoperative pathology supported the completeness of the definitive resection. Post-operation, the patient’s bowel motility significantly improved, and interestingly, the complications disappeared. For adult patients with long-term constipation combined with cough and hypertension, rare diseases like HD which requires definite surgery and which could be “right-sided” should not be overlooked. It is vital to diagnose and cure HD patients in childhood. Through the comparison of the two surgeries, it is noteworthy that for diagnosed HD, sufficient removal of the non-functional intestine confirmed by intraoperative pathology is essential.     

Mercury poisoning complicated by acquired neuromyotonia syndrome: A case report

Rationale:Acquired neuromyotonia syndrome is a rare form of peripheral nerve hyperexcitability syndrome. It is characterized by spontaneous and continuous muscle contractions. Acquired neuromyotonia syndrome is mainly observed in patients with autoimmune diseases or tumors, but it is a rare neurological clinical manifestation in patients with mercury poisoning.Patient concerns:A 56-year-old woman presented with continuous and involuntary muscle twitching in her legs for 2 months; it was accompanied by a burning sensation in the lower limbs, insomnia, fatigue, and night sweats. These symptoms did not disappear during sleep.Diagnoses:Toxicological blood analysis via atomic fluorescence spectrometry revealed that the level of mercury was 0.07 μmol/L (normal level: <0.05 μmol/L). Her urinary mercury level measured using the cold atomic absorption method was 217.50 μmol/mol creatinine, which was considerably higher than the reference range (0–2.25 μmol/mol creatinine for people not in contact with mercury, 0–20 μmol/mol creatinine following long-term exposure). Upon further testing, a high level of mercury (10,572 mg/kg) was detected in the patient''s cream. Accordingly, this patient was diagnosed with mercury poisoning.Interventions:Treatment with 2,3-dimercapto-1-propanesulfonic acid (DMPS) was initiated. Her urinary mercury level decreased to 9.67 μmol/mol creatinine, and her neuromyotonia syndrome and hyponatremia were relieved, with urine protein completely disappearing after 3 months of treatment.Outcomes:After DMPS treatment, the clinical manifestations of the nervous system disappeared and electrolyte parameters returned to normal levels.Lessons:Acquired neuromyotonia syndrome is a rare disorder caused by the hyperexcitability of peripheral nerves, resulting in spontaneous and continuous muscle contraction. Mercury poisoning should be considered in patients with neuromyotonia syndrome. Early detection of mercury poisoning can prevent unnecessary examinations and treatments.     

Tentacled snakes turn C-starts to their advantage and predict future prey behavior

Fish are elusive prey with a short-latency escape behavior—the C-start—initiated to either the left or right by a “race” between 2 giant Mauthner neurons in the fish brainstem. Water disturbances usually excite the ipsilateral neuron, which massively excites contralateral motor neurons, resulting in a rapid turn away from striking predators. Here, it is reported that tentacled snakes (Erpeton tentaculatus) exploit this normally adaptive circuitry by feinting with their body, triggering the Mauthner cell that is furthest from their head milliseconds before a ballistic strike is initiated. As a result, fish that were oriented parallel to the long axis of the snake''s head most often turned toward the approaching jaws, sometimes swimming directly into the snake''s mouth. When strikes were instead directed at fish oriented at a right angle to the snake''s head, snakes anticipated future fish behavior by striking to where fish would later be if they escaped from the snake''s body feint, which fish usually did. The results provide an example of a rare predator taking advantage of a prey''s normally adaptive escape circuitry and suggest that the snake''s sensory-motor system is adapted to predict future behavior.     

Electrocardiogram in Friedreich's ataxia: A short‐term surrogate endpoint for treatment efficacy

Friedreich''s ataxia is a rare degenerative neuromuscular disorder, caused by a homozygous GAA triplet repeat expansion in the frataxin (FXN) gene, with a broad clinical phenotype characterized by progressive gait and limb ataxia, dysarthria, and loss of lower limb reflexes; cardiac involvement is represented by hypertrophic cardiomyopathy, ventricular arrhythmias, and sudden cardiac deaths. Currently, no definite therapy is available, while many drugs are under investigation; for this reasons, we need markers of short‐ and long‐term treatment efficacy acting on different tissue for trial evaluation. We describe the case of a 21‐year‐old patient affected by Friedreich''s ataxia on wheel‐chair, with initial cardiac involvement and electrocardiographic features characterized by thiamine treatment‐related negative T wave and QTc variations. We discuss plausible physiopathology and potential ECG role implications as an intermediate marker of treatment response in future clinical trials considering patients affected by Friedreich''s ataxia.     

Case of Heerfordt's syndrome with prolonged peripheral nerve involvement]

A 28-year-old man complaining of myiodesopsia was given a diagnosis of uveitis. Subsequently he complained facial nerve palsy and enlargement of parotid gland. Heerfordt's syndrome was diagnosed based on the results of several examinations. Facial nerve palsy, enlargement of the parotid gland and uveitis were improved by systemic corticosteroid therapy. At present he is receiving systemic corticosteroid therapy, but numbness in the mouth, thought to be the involvement of the trigeminal nerve, remains. Systemic corticosteroid therapy is usually effective for most cases with Heerford's syndrome. On the other hand, there are some cases with the prolonged peripheral nerve involvement despite systemic corticosteroid therapy, as seen in this case. If peripheral nerve involvement is prolonged, it is necessary to consider small-fibre neuropathy as one possible cause.     

Twiddler's syndrome in a patient with CRT-D device – A case report

Twiddler''s syndrome is a rare cause of pacemaker lead dislodgement. A 61-year-old female patient was implanted with a defibrillator capable of cardiac re-synchronization therapy (CRT-D); 10 months later, she presented with uneasiness and vibratory sensations in the chest. Fluoroscopy revealed rotation of the pulse generator along its longitudinal axis and dislodgement of all three leads. Diagnosis of Twiddler''s syndrome was made.