臨床症例報告ジャーナル

Background: Duane Syndrome is a congenital cranial dysinnervation disorder that primarily restricts eye movements. The majority of patients have isolated Duane syndrome with no other congenital abnormalities, however in certain cases, there are non-ocular symptoms which do not fit aspecific syndrome. In this case we describe bilateral Duane syndrome type 1(limited eye abduction) with findings of developmental arrest in the inner ear and cochlear aplasia which to our knowledge has not been reported yet.

Case presentation: This case reports a 3 year old boy who presented with watery eyes during feeding and speech delay. He had a right sided sensorineural hearing loss, with ABR being inconclusive and ASSR showed severe hearing loss. CT mastoids and MRI were performed and a diagnosis of cochlear aplasia with inner ear developmental arrest was made. The patient showed good response and improvement in speech with a soft band bone anchored hearing aid.

Conclusion: The early recognition and diagnosis of other organ anomalies associated with Duane’s syndrome is of utmost importance for the prognosis of the patient. Radiological imaging, most importantly MRI is the gold-standard for identification of inner ear abnormalities.

COVID-19 vaccination has been very efficient in preventing severe illness and death from SARS CoV-2. These vaccines are usually reported to have mild and transient side effects. We report a case of a 59-year-old woman with an acquired hemophilia A following the administration of her second dose of mRNA 1273 vaccine. The patient presented with worsening swelling and bruising of her left arm three days after vaccination. She had a history of COVID-19 infection 3 months ago. Laboratory workup showed elevated partial thromboplastin time (PTT) and mixing study showed possible inhibitor. Factor VIII level (%) was <5% and the inhibitor panel confirmed presence of inhibitor. The patient was treated with high dose steroids and cyclophosphamide. Currently the patient is on slow taper steroids and hematoma has improved.

Macrophage activation syndrome is a potentially life-threatening complication of rheumatic diseases. Here, we report the case of a 4-yearold boy who presented with fever for 5 months, initially moderate-high grade, abdominal pain for 3 months, and pain in BL knee and ankle joints for 2 months. On examination, the child had multiple cervical lymphadenopathies and no organomegaly. The differential of disseminated tuberculosis, lymphoma, and juvenile idiopathic arthritis. The initial blood count was normal. The Chest X-Ray was also normal. Bone marrow examination showed normal hematopoiesis. The excisional biopsy of the cervical lymph node showed reactive lymphadenitis. During the course of hospitalization, the patient developed high-grade fever with hepatosplenomegaly and swelling of BL ankle and knee joints. The blood count at 2 weeks suggested pancytopenia, and liver function test indicated increased aspartate aminotransferase. Chest X-Ray showed BL pleural effusion, a clear transudative fluid was aspirated. C-Reactive protein, serum ferritin, and triglycerides were raised, and his bone marrow exhibited hemophagocytosis, while the Erythrocyte sedimentation rate and fibrinogen levels were decreased. Based on ILAR (International league against Rheumatism’s) and PRINTO (Paediatric Rheumatology International Trials Organisations) criteria, a diagnosis of Systemic onset juvenile rheumatic arthritis with macrophage activation syndrome was made. The patient was managed with intravenous methylprednisolone, intravenous immunoglobulin, and tacrolimus. The patient showed gradual clinical improvement and was discharged on clinical improvement and was doing well on follow-up at 4 weeks and 6 months.

Objective: Report a case with probable autoimmune sensorimotor neuropathy positive for serum anti-Yo antibodies.

Background: Paraneoplastic neurological syndrome occurs because of immune-mediated neuronal dysfunction secondary to systemic malignancy, and the symptoms usually occur before the malignancy is discovered. Anti-Yo antibody is known to be an onconeural antibody that causes paraneoplastic cerebellar degeneration associated with ataxia and cerebellar dysfunction and denote an underlying malignancy, usually gynecological tumors. Here, we have reported a rare case of autoimmune sensorimotor neuropathy positive for serum anti-Yo antibodies presenting with tremors, slurred speech and unstable gait, in a patient with pelvic tumor.

Case Report: A 56-year-old female patient presented with an 8 months history of upper limbs tremors, slurred speech and an unstable gait for past 6 months. Initially, the patient underwent a brain MRI, which showed mild cerebellar atrophy, paranasal sinusitis and possible right frontal endogenic small osteoma. While investigating further, the anti-Yo antibodies in serum and cerebrospinal fluid were found to be weakly positive. On ultrasound, an abnormal echogenic structure was found in the left side of the pelvic cavity, with the absence of the uterus. As such, a pelvic MRI was carried out, which showed abnormal signals in bilateral accessory areas.

Conclusion: This is a rare case of autoimmune sensorimotor neuropathy positive for serum anti-Yo antibodies in a patient with a pelvic tumor. The appearance of neurological symptoms should raise suspicions for paraneoplastic autoimmune disorders.

Aim and Objectives: We conducted a clinical study to report the incidence of a second mesiobuccal (MB2) canal that was identified and treated in the maxillary first molar during eight months in our restorative department at Al Noor specialist hospital.

Material and Methods: The clinical study is retrospectively conducted to determine the number of root canals in maxillary first molars that were negotiated and treated-the data collected from the examination sheet of the patient's files. Four endodontic specialists (E1-E4) treated a total of 129 1st molar teeth. All cases were treated under routine clinical protocol, no special instruments or standard protocol for searching for MB2 canal either. Searching for an MB2 canal relies on the low-speed round burs only. One of the endodontists used a dental loupe without the light of 2.5X magnification. All other specialists did not use any magnification aid. The presence of MB2 was confirmed when it was negotiated, instrumented and obturated.

Results and Conclusion: The specialists treated 129 maxillary first permanent molars. Only 38 (29%) cases had a fourth root canal-all fourth canals were found in the mesiobuccal (MB) roots. Overall, 60% of MB2 had commonly apical exist with MB1, type II Vertucci canal configuration. 40% of MB2 had type IV Vertucci canal configuration. In the present clinical study, no microscope or ultrasonic devices have been used. One of the specialists used loupes of 2.5X magnifications. Simple magnification utilizing dental loupe with proper anatomical knowledge could increase the detection of MB2.