Prominent Enlargement of the Facial Nerve Canal in a Case of a Persistent Stapedial Artery.

In this case report, we present a 23-year-old patient with complaints of a recurrent plop sound in the left ear. Non-contrast CT and MR imaging revealed an enlargement of the tympanic segment of the facial canal due to venous congestion. A persistent stapedial artery (PSA) and absent foramen spinosum were identified as concomitant factors. This unique combination of anomalies has not been previously reported and knowledge about this condition is essential to avoid unnecessary surgery. Teaching Point: A persistent stapedial artery may be associated with enlargement of the tympanic facial nerve canal due to venous congestion.

A metformin add-on clinical study in multiple sclerosis to evaluate brain remyelination and neurodegeneration (MACSiMiSE-BRAIN): study protocol for a multi-center randomized placebo controlled clinical trial.

Introduction: Despite advances in immunomodulatory treatments of multiple sclerosis (MS), patients with non-active progressive multiple sclerosis (PMS) continue to face a significant unmet need. Demyelination, smoldering inflammation and neurodegeneration are important drivers of disability progression that are insufficiently targeted by current treatment approaches. Promising preclinical data support repurposing of metformin for treatment of PMS. The objective of this clinical trial is to evaluate whether metformin, as add-on treatment, is superior to placebo in delaying disease progression in patients with non-active PMS. Methods and analysis: MACSiMiSE-BRAIN is a multi-center two-arm, 1:1 randomized, triple-blind, placebo-controlled clinical trial, conducted at five sites in Belgium. Enrollment of 120 patients with non-active PMS is planned. Each participant will undergo a screening visit with assessment of baseline magnetic resonance imaging (MRI), clinical tests, questionnaires, and a safety laboratory assessment. Following randomization, participants will be assigned to either the treatment (metformin) or placebo group. Subsequently, they will undergo a 96-week follow-up period. The primary outcome is change in walking speed, as measured by the Timed 25-Foot Walk Test, from baseline to 96 weeks. Secondary outcome measures include change in neurological disability (Expanded Disability Status Score), information processing speed (Symbol Digit Modalities Test) and hand function (9-Hole Peg test). Annual brain MRI will be performed to assess evolution in brain volumetry and diffusion metrics. As patients may not progress in all domains, a composite outcome, the Overall Disability Response Score will be additionally evaluated as an exploratory outcome. Other exploratory outcomes will consist of paramagnetic rim lesions, the 2-minute walking test and health economic analyses as well as both patient- and caregiver-reported outcomes like the EQ-5D-5L, the Multiple Sclerosis Impact Scale and the Caregiver Strain Index. Ethics and dissemination: Clinical trial authorization from regulatory agencies [Ethical Committee and Federal Agency for Medicines and Health Products (FAMHP)] was obtained after submission to the centralized European Clinical Trial Information System. The results of this clinical trial will be disseminated at scientific conferences, in peer-reviewed publications, to patient associations and the general public. Trial registration: ClinicalTrials.gov Identifier: NCT05893225, EUCT number: 2023-503190-38-00.

Renal Angiomylipoma with Epithelial Cyst (AMLEC): A Rare Cystic Variant of Angiomyolipoma.

Teaching Point: AMLEC is a rare subtype of an angiomyolipoma (AML) and not a primary cystic lesion.

Baló Concentric Sclerosis.

Teaching Point: Baló concentric sclerosis is a rare subtype of multiple sclerosis, characterized by a concentric layered mass on magnetic resonance imaging.

Pneumococcal Meningitis.

Teaching Point: Spontaneous, atraumatic pneumocephalus is a rare presentation of pneumococcal meningitis.

Behçet Disease.

Teaching Point: Although Behçet disease is a multisystemic and chronic vasculitis, it can be superimposed with a variety of acute vasculitis.

Nasal and Nasopharyngeal Rosai-Dorfman Disease.

Teaching Point: Nasal and nasopharyngeal Rosai-Dorfman disease is a rare cause of nasal obstruction.

Teenage Neurologic Manifestation of a Complex Brain Malformation Consisting of Gray Matter Heterotopia, Schizencephaly and Absent Septum Pellucidum.

Teaching point: Since some minor congenital brain defects manifest long after birth, even in adults they should be kept in the differential of new epileptic seizures or focal neurologic deficits.

MRI of Wallerian Degeneration in the Brainstem: A Pictorial Essay.

Wallerian degeneration of the cerebral peduncle is a common MRI finding after cerebral injury. The degree of peduncular atrophy reflects the extent of damage in the corticospinal tract. The acute phase of Wallerian degeneration is visible with Diffusion-Weighted-Imaging. New investigation with Diffusion-Tensor-Imaging quantifies Wallerian degeneration in the subacute ischemic phase and is a good predictor for later functional recovery after stroke.

Epineurial Pseudocyst of the Intratemporal Facial Nerve: A Case Series Study.

OBJECTIVES: The goal of this case series was to describe the clinical and radiological characteristics of epineurial pseudocysts of the intratemporal facial nerve (EPIFs) and to discuss the relevance in clinical practice. MATERIALS AND METHODS: A retrospective case series of 10 consecutive patients with EPIFs identified through computed tomography (CT), between 2009 and 2018. Morphological characteristics, coexisting pathology, facial nerve function, and evolution over time were analyzed. RESULTS: A unilateral EPIF was found in 5 patients (50%) and a bilateral EPIF was found in the other 5 (50%). The largest dimensions were observed in the coronal plane, with an average craniocaudal length of 6.0 mm (range, 3-9 mm). None of the patients presented with facial nerve dysfunction. Growth could not be observed in any of the patients. In 5 cases (33.3%), CT imaging showed a reduced transmastoid access to the facial recess caused by the EPIF. CONCLUSION: All EPIFs in this study were incidental findings. Facial nerve function was normal in all patients. Knowledge of EPIFs is important to perform safe cholesteatoma and cochlear implant surgery and to prevent unnecessary follow-up imaging.

Craniofacial and Intracranial Langerhans Cell Histiocytosis.

Main Teaching Point: Multiple osteolytic calvarial lesions in a child raise suspicion of Langerhans cell histiocytosis.

A Typical but Underdiagnosed Nasal Cavity Mass.

Respiratory epithelial adenomatoid hamartoma is a relatively new diagnosis, only added to the World Health Organization classification of tumours in 2005. The lesion results from non-neoplastic overgrowth of glandular tissue in the nasal cavity and rarely in de sinus cavities and is often associated with nasal polyposis. The classical appearance of a bilateral mass in the olfactory cleft causing widening of the olfactory cleft allows the radiologist to suggest the diagnosis on computed tomography or magnetic resonance imaging.

Painless Swelling of the Left Jaw in a Two-Year-Old Boy.

We present a case of a 2-year-old boy with chronic suppurative osteomyelitis of the left jaw. A computed tomography (CT) scan demonstrated a periosteal reaction on the left side of the mandible with cortical destruction of the left mandibular head. The diagnosis could be confirmed histologically. During biopsy of the mandibular lesion, a purulent exudate was withdrawn.

Familial Multiple Cavernous Malformation Syndrome: MR Features in This Uncommon but Silent Threat.

Cerebral cavernous malformations (CCM) are vascular malformations in the brain and spinal cord. The familial form of cerebral cavernous malformation (FCCM) is uncommon. This autosomal dominant pathology mostly presents with seizures and focal neurological symptoms. Many persons affected by FCCM remain asymptomatic. However, acute hemorrhages may appear over time. MRI demonstrates multiple focal regions of susceptibility induced signal loss, well seen on gradient-echo sequences (GRE) or even better on susceptibility-weighted imaging (SWI). The presence of a single CCM - especially in young persons - without history of FCCM does not exclude this diagnosis. Some clinicians also advise an MRI of the spinal cord at the time of diagnosis to serve as a baseline and a control MRI of the brain every one to two years. MRI is certainly indicated in individuals with obvious new neurologic symptoms. Symptomatic siblings should also undergo an MRI of the brain to determine presence, size, and location of the lesions. Even in asymptomatic siblings, a screening MRI may be considered, as there may be an increased risk of hemorrhage, spontaneous or due to the use of certain medications; the knowledge of the presence and the type of these lesions are important. Surgical removal of a CCM may be justified to prevent a life-threatening hemorrhage. Control MRI may reveal the postoperative outcome.

Friday: Fish Day.

A 78-year-old man presented with diffuse abdominal pain, localized peritonitis and raised inflammatory markers. CT revealed an mesentery abcess with a linear high density structure in continuity with the adjacent small bowel lumen. Laparoscopy showed a perforation by a fish bone. Gastrointestinal tract perforation by foreign body ingestion is rare complication (1%). There are some risk factors and prefered locations of perforation. Time interval between ingestion and complication can vary extremely.

Hemilingual spasm: a new neurosurgical entity? Case report.

Hemilingual spasm is a little-known movement disorder, presenting as intermittent paroxysmal involuntary contractions of half of the tongue muscles. The authors report a case of hemilingual spasm caused by an arachnoid cyst. After marsupialization of the cyst, the patient's symptoms immediately resolved. There has been no recurrence of hemilingual spasm during the follow-up period of more than 40 months.

Benign metastasizing leiomyoma to skull base and spine: a report of two cases.

OBJECTIVE AND IMPORTANCE: We report two patients with benign uterine leiomyoma metastasizing to the nervous system, respectively, to the skull base and to the spine. Although primary cranial and metastatic spinal leiomyomas have been rarely described, to our knowledge no case of benign leiomyoma metastasizing to the skull base has been reported before. CLINICAL PRESENTATION: Two female patients with history of hysterectomy for benign leiomyoma, subsequently metastasizing to the lungs and smooth muscles of the skin, presented with a focal neurologic deficit. Magnetic resonance imaging revealed a sacral mass in one case and a skull base tumor in the other. INTERVENTION: Both patients underwent surgery for resection of the lesions. Good postoperative results were obtained. Histologic examination of the surgical specimen revealed a benign metastasizing leiomyoma. CONCLUSION: Benign metastasizing leiomyoma should be considered in the differential diagnosis of mass lesions in the sacral spine and skull base in patients who have a history of uterine leiomyoma or benign metastases of the same disease in organs outside the nervous system.

Paediatric lipoblastoma in the head and neck: three cases and review of literature.

Lipoblastoma is a rare benign tumour arising from embryonic white fat. The tumours occur primarily in infancy and early childhood and usually arise from the limbs and the trunk, but neck involvement is rare. We report three cases of head and neck lipoblastoma. In all cases, imaging showed a well-delineated, fat-containing tumour. After surgical resection, the outcome of these patients was uneventful.