Navigating the complexity of skull base osteomyelitis: a case study and comprehensive review.

Skull base osteomyelitis is a rare and life-threatening infection of the skull base, commonly seen in elderly diabetic patients as a result of otogenic or paranasal infection. The diagnosis is based on a series of arguments, including a high clinical suspicion, imaging findings, negative biopsies for malignancy, and microbiological isolation. Complications, including abscess formation and vascular involvement, mandate a multidisciplinary treatment approach, primarily involving broad-spectrum antibiotics and surgical debridement, but the prognosis is usually poor. Herein, we describe the case of a 55-year-old male, who 15 years prior, underwent radiation therapy for nasopharyngeal carcinoma. He presented with an infection of the skull base with extensive bone erosion accompanied by an uncommon complication; an intra clival abscess. Despite aggressive antibiotic therapy, the patient ultimately succumbed to septic shock.

Bilateral carotid dissection due to Eagle syndrome.

Eagle syndrome is defined as a collection of symptoms affecting the cervical and cranial regions, resulting from an elongated styloid process or ossified stylohyoid ligament encroaching on surrounding structures and causing a variety of symptoms. Classically, Eagle syndrome presents as neck, throat, or ear pain. Carotid artery dissection is a rare complication of Eagle syndrome. We report the case of a 40-year-old man who presented with bilateral internal carotid artery dissection secondary to pathological elongation of the styloid processes.

Brain MRI abnormalities associated with amyotrophic lateral sclerosis: A case illustration.

Amyotrophic lateral sclerosis is a progressive neurodegenerative pathology. It involves both upper and lower motor neurons, leading to their degeneration. Lower motor neurons can be detected with an electromyogram, but the detection of upper motor neuron dysfunction may be more accurate using MRI. We present the case of a 64-year-old woman with amyotrophic lateral sclerosis, presenting the motor band sign and the bright tongue sign on MRI.

Intracochlear schwannoma: Imaging diagnosis.

Intralabyrinthine schwannomas (ILS) are rare benign tumors, often responsible for hearing loss. MRI is important in establishing the diagnosis. We present the example of a 48-year-old lady who reported a 3-years history of right-sided sensorineural deafness. MRI demonstrated a loss of the normal hypersignal of the second turn of the right cochlea compatible with intracochlear schwannoma.

Central neurocytoma-positive and differential diagnosis: An example through a case report.

Central neurocytoma is a rare intraventricular tumor, occurring typically in the lateral ventricle of young adults. It is considered as a neuronal-glial benign tumor with favorable prognosis. Imaging is a cornerstone allowing the accurate preoperative diagnosis on the basis of several characteristic features. We report the case of a 31-year-old man who has been complaining of progressive headaches and in whom brain magnetic resonance imaging revealed a central neurocytoma. We remind then, through a literature review, the main criteria to set the diagnosis of this tumor and rule out the other possible diagnoses.

A rare case of Fahr disease revealed by an epileptic seizure.

Fahr's disease is a rare neurodegenerative disorder, identified by bilateral and symmetrical intracerebral calcifications of mainly the basal ganglia. Patients often present extrapyramidal or neuropsychological symptoms. Seizure is one of the rarest manifestations that can reveal Fahr disease. We present the case of a 47-year-old male patient who had Fahr disease revealed by an inaugural tonic-clonic seizure.

Fulminant Susac syndrome-a rare cause of coma: The history of the fatal course in a young man.

Susac syndrome is a rare microangiopathy of indeterminate etiology, presumably autoimmune, characterized by a triad of encephalopathy, sensorineural hearing loss, and branch retinal artery occlusions occurring predominantly in women. The onset and progression patterns are multiple, mainly of three modes. Fulminant evolution is exceptional, rarely reported across literature. We report through this case a Susac syndrome in a young man in whom evolution was fatal. Magnetic resonance imaging is essential to raise the diagnosis and for follow-up, with almost pathognomonic findings, all the more useful as the clinical triad is usually incomplete and as the encephalopathy is the most limiting of the symptoms.

Posterior reversible encephalopathy syndrome (PRES): Should more attention be paid to the atypical forms?

Posterior reversible encephalopathy syndrome (PRES) is an uncommon, but important, pathology affecting primarily the posterior cerebral circulation. Typical imaging features include vasogenic edema involving the bilateral occipital and parietal lobes. We report 4 cases of atypical PRES: The first one is a 59-year-old woman with a medical history of diabetes and hypertension who presented a consciousness disorder. MRI of this patient revealed signal abnormalities in the thalamus, the cerebellum, the brainstem and the corpus callosum with microbleeds and vascularization disorders. The second patient is a 41-year-old woman admitted for right hemiparesis and facial paralysis. Physical examination found a hypertension and the atypical imaging features on MRI were low ADC values with a pearl necklace appearance on ARM sequences. The third patient is a 32-year-old woman who undergone a surgery for an ectopic pregnancy and went in a coma for 8 days. Physical and biological parameters were normal. Diagnosis was delayed because of atypical signal abnormalities in caudal and lentiform nuclei that worsened on follow-up MRI, resulting in a dramatic evolution to bilateral cecity and psychosis. The last case is a 34-year-old woman with terminal renal failure hospitalized for spontaneously resolving tonic-clonic seizures. Biology showed high uremia and brain MRI revealed a gyriform enhancement at gadolinium injection with unilateral putamen lesion. The patient improved after blood purification. Knowledge of these atypical findings can help the radiologist make an accurate, timely diagnosis, and improve patient care.

Brain iron accumulation on MRI revealing aceruloplasminemia: a rare cause of simultaneous brain and systemic iron overload.

Aceruloplasminemia is a rare autosomal recessive disorder of iron accumulation in the brain. It is one of the subtypes of Neurodegeneration with brain iron accumulation and is characterized by the uniform involvement of all basal ganglia, thalami, dentate nuclei, and cortex. Aceruloplasminemia is the only known iron overload disorder in which brain and systemic iron overload are combined. Here, we present a 53-year-old female who had progressive cognitive disorders with motor deficits. MRI showed extensive and abundant iron deposited in the brain and in the liver.

Spontaneous clival meningocele.

The occipital bone is an uncommon location for meningoceles protrusion. This condition occurs generally after a severe traumatism or surgical procedure. However, in some rare cases, the herniation can happen spontaneously. Nontraumatic clival meningoceles present an extremely rare entity and correspond to a herniating pachymeningeal collection containing cerebrospinal fluid through a zone of fragility in the clivus. Clinical presentation ranges from simple headache or rhinorrhea to severe complications such as recurrent bacterial meningitis or nerve compression. Computed tomography provides an analysis of the bone and magnetic resonance imaging provides a superior contrast resolution, helping to distinguish among the various types of clival lesions. We report the case of a young woman with a long history of idiopathic intracranial hypertension, who presented with a worsening headache. Magnetic resonance imaging confirmed a clival meningocele without other complications and the patient was put under medical surveillance.

Incidental optic nerve sheath arachnoid cyst: A rare case finding with literature review.

Arachnoid cysts are the most common benign cystic abnormalities formed due to congenital splitting of the arachnoid layer. They comprise 1% of intracranial masses, and the orbital location is even more rarely reported in history especially in the pediatric population. They might be discovered as an asymptomatic finding on imaging performed for a concomitant condition or, in most reported cases, as a result of ophthalmic impairment. They can be isolated or associated with gliomas, neurofibromas, empty sella syndrome, and frontotemporal porencephalic cysts. Computed tomography scan shows a non-enhancing liquid cystic lesion, and magnetic resonance imaging remains the best assessment tool confirming the similarity of the fluid to cerebrospinal fluid and evaluating the optic nerves. Herein, we report the case of an incidental discovery of an intraorbital arachnoid cyst on magnetic resonance imaging in a 53-year-old woman with a history of epilepsy. No treatment was performed as the cystic formation was asymptomatic.

Granulomatosis with polyangiitis presenting with unilateral exophthalmos: A case report.

We report a case of a patient with no medical history, admitted for right exophthalmos. For whom imaging showed orbital masses without inflammatory signs pointing to a granulomatous origin. However, the histological and immunological workup revealed the diagnosis of Granulomatosis with polyangiitis.

["Pancoast Tobias tumor"]. / Tumeur de Pancoast-Tobias.

"Dépistage des troubles visuels chez l'enfant. Le dépistage visuel chez l'enfant représente un objectif de santé publique en raison de l'impact social, scolaire et financier d'une anomalie de la vision, potentiellement irréversible en l'absence de prise en charge précoce. De plus, la plupart de ces troubles sont corrigibles, c'est le cas notamment des anomalies de la réfraction et du strabisme. Un groupe d'experts de l'Association francophone de strabologie et d'ophtalmologie pédiatrique (AFSOP) a récemment mis à jour des recommandations consensuelles pour l'optimisation de ce dépistage. Ce travail souligne l'importance d'un examen ophtalmologique rapide dans la première semaine ou première année de vie respectivement pour les enfants à risque d'amblyopie organique et fonctionnelle, ainsi que d'un dépistage systématique de tous les enfants à l'âge de 3 ans à l'aide de trois examens visant à détecter respectivement un trouble de la réfraction, un strabisme ou une amblyopie. Par ailleurs, certains signes d'appel, comme la présence d'une leucocorie ou d'une buphtalmie, conduisant à un examen ophtalmologique sans délai, doivent être reconnus par tout professionnel en charge de l'examen global de l'enfant."

Persistence of a trigeminal artery associated with a posterior meningeal artery aneurysm: case report and literature review.

A 59-year-old female presented with severe headache and trouble of consciousness. The CT showed a subarachnoid hemorrhage FISHER IV with a hematoma of the right cerebellum. The angiography discovered a persistent left trigeminal artery associated with an aneurysm of the right posterior meningeal artery. The attempt of embolization failed and the patient suffered cerebral vasospasm and died. The persistence of a trigeminal artery is found in 0.1-0.6% of the population. It is the most frequent embryological carotid-vertebral anastomosis. It appears at the fifth week of development and disappears when the embryo reaches 5-7 mm. This embryological persistence is associated with numerous vascular abnormalities. The literature review showed the presence of those aneurysms in the trigeminal artery itself or the communicating artery. The association that we described is the first to our knowledge. Therefore, this association is particular and interesting to expose.

[Tumor involving the sciatic nerve: neurinoma or neurofibroma? Can MRI make the difference?] / Tumeur du nerf sciatique: neurinome ou neurofibrome? L'IRM peut-elle faire la différence?

The sciatic nerve is the terminal branch of the sacral plexus. Sciatalgia is a nerve root pain. In most cases, sciatica originates from degenerative disc disease. Tumor involving the sciatic nerve is extremely rare. We here report the case of a 33-year old patient with nerve tumor detected on MRI performed for drug-resistant sciatica. Tumor involving the sciatic nerve is rare and diagnosis is difficult. MRI data are crucial for establishing an effective surgical approach.

De novo mutations in SMCHD1 cause Bosma arhinia microphthalmia syndrome and abrogate nasal development.

Bosma arhinia microphthalmia syndrome (BAMS) is an extremely rare and striking condition characterized by complete absence of the nose with or without ocular defects. We report here that missense mutations in the epigenetic regulator SMCHD1 mapping to the extended ATPase domain of the encoded protein cause BAMS in all 14 cases studied. All mutations were de novo where parental DNA was available. Biochemical tests and in vivo assays in Xenopus laevis embryos suggest that these mutations may behave as gain-of-function alleles. This finding is in contrast to the loss-of-function mutations in SMCHD1 that have been associated with facioscapulohumeral muscular dystrophy (FSHD) type 2. Our results establish SMCHD1 as a key player in nasal development and provide biochemical insight into its enzymatic function that may be exploited for development of therapeutics for FSHD.

[Moya moya disease: a rare cause of ischemic stroke in children: about a case]. / Moya moya: étiologie rare d'accident vasculaire cérébral ischémique chez l'enfant: à propos d'un cas.

Moya moya disease is an angiogenic disease characterized by the narrowing of the distal internal carotid artery extending to the proximal segments of the middle and anterior cerebral arteries, inducing collateral vessels formation. These vessels come from the collateral parenchymal vessels, the perforating vessels, leptomeningeal vessels and other transdural anastomoses. These collateral vessels have a characteristic appearance on angiography, forming a cloud of smoke: net-like moyamoya. Its etiology is still poorly understood. Moyamoya disease accounts for 10-15% of the causes of stroke, with 2 age peaks at which its occurrence is more frequent: children around 5 years old and adults around 40 years old. Its evolution can be slow with intermittent symptoms or fulminant with fast neurological deterioration. The current data show the role of surgery as the gold standard for the treatment of moyamoya syndrome, in particular in patients with progressive and recurrent symptoms.

Relationship between dimensions of muscles of mastication (masseter and lateral pterygoid) and skeletal dimensions: study of 40 cases.

INTRODUCTION: The masticatory muscles play an important part in determining the morphology of the facial skeleton. Skeletal typology and the characteristics of the masticatory muscles are closely linked. Several authors have studied muscle characteristics as related to facial typology. The aim of this work is to study the relationship between vertical and transverse skeletal dimensions and the dimensions (length, width and thickness) of two muscles of mastication, the masseter and the lateral pterygoid. MATERIALS AND METHOD: Our study was based on CT-scan examinations of a sample composed of patients consulting the X-ray department of the Rabat-Salé Teaching Hospital, and for whom a CT-scan had been requested. Forty CT examinations of the skull, performed in the context of sinus explorations or pre-surgical work-ups in the radiology department of the Rabat-Salé Teaching Hospital, were selected for this study. The sample comprised 19 women and 21 men aged between 20 and 45, with a mean of 40.9 ± 12.8. A Siemens 32-row 64-slice spiral CT-scan device was used for spiral acquisition of data around the facial bones, with the mouth closed. The study was carried out in the parenchymal window for the muscle measurements, in the axial and coronal planes. Bone measurements were performed after 3D reconstruction in VRT mode. RESULTS: Our study showed that, for the masseter muscle, thickness is the dimension that correlates significantly with skeletal dimensions in the vertical, transverse and sagittal directions. For the lateral pterygoid muscle, length and width both present significant correlations with transverse skeletal dimensions. Analysis of these results shows that the dimensional characteristics of the masticatory muscles vary according to the vertical and transverse skeletal typology of the subjects concerned.