Hypertrophic olivary degeneration secondary to a Guillain Mollaret triangle cavernoma: Two case report.

Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration. It is caused by a damage at the Guillain-Mollaret triangle (GMT), which is defined by three anatomical structures: the dentate nucleus, the red nucleus, and the inferior olivary nucleus (ION). Clinically, it may be revealed by palatal myoclonus. On MRI, it appears as a unilateral or bilateral enlargement of the inferior olivary nucleus which shows a high signal intensity on T2-weighted images, with sometimes a cerebellar atrophy. Here we report 2 cases of healthy patients which present hemorrhagic brainstem cavernomas, complicated later by the development of palatal myoclonus and cerebellar ataxia, with MRI features corresponding to an (HOD) secondary to a (GMT) cavernoma. The purpose is to explain the mechanism of (HOD) subsequent to lesion in (GMT), and to describe magnetic resonance imaging features.

A case of tuberous sclerosis complex revealed by epilepsy.

Tuberous sclerosis complex is a multisystem genetic disease with autosomal dominant inheritance, characterized by the development of benign tumors known as hamartomas that affect multiple organs. It is a condition with a wide phenotypic spectrum, and its clinical presentation varies over time within the same individual. Hence, the importance of early screening and rigorous monitoring of evolving clinical manifestations. Diagnosis can occur at any age. These tumors are generally benign, but their size and location can have a significant impact on the prognosis and, in some cases, even on life expectancy. Cardiac, neurological, and cutaneous manifestations are most common in childhood. The onset of early and severe epilepsy within the first year of life is associated with neurodevelopmental disorders that impact the quality of life for affected individuals and their families. We present a case of a 22-year-old female patient experiencing inaugural epileptic seizures in adulthood, with magnetic resonance imaging revealing subependymal hamartomas, cortical tubers and radial migration bands accompanied by polycystic kidney disease; the diagnosis of tuberous sclerosis complex was established based on the association of these lesions, which constitute major and minor criteria.

The "pancake-like" enhancement in cervical spondylotic myelopathy.

Cervical spondylotic myelopathy, characterized by chronic spinal cord compression resulting from degenerative spine changes, manifests with a spectrum of neurological and pain symptoms. Despite the complexity of intramedullary spinal cord abnormalities, employing a systematic approach to differential diagnosis, considering factors such as lesion location, cord length, segment involvement, and enhancement pattern, can significantly aid in narrowing down the potential diagnoses, potentially avoiding invasive diagnostic procedures and guiding treatment decisions. This article presents two cases of cervical spondylotic myelopathy characterized by progressive weakness and paraesthesia, exhibiting progressive bilateral upper extremity numbness, tingling, and impaired gait, with cervical myelopathy evident on MRI displaying transverse pancake-like gadolinium enhancement.

A burning encephalitis: Fluid-attenuated inversion recovery-hyperintense lesions in Anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures-A case report and review of the literature.

FLAMES, or fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein (anti-myelin oligodendrocyte glycoprotein)-associated encephalitis with seizures, represents a rarely documented syndrome characterized by ambiguous features. Positioned within the spectrum of inflammatory demyelinating diseases of the central nervous system, it is regarded as a distinct subset of myelin oligodendrocyte glycoprotein antibody-associated disease, the latest classification in this domain. Myelin oligodendrocyte glycoprotein antibody-associated disease exhibits a diverse clinical spectrum, spanning from solitary optic neuritis or myelitis to multifocal central nervous system demyelination, manifesting as acute disseminated encephalomyelitis, or cortical encephalitis accompanied by seizures, delineating the fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures syndrome. We present a compelling case study of a 30-year-old individual with a history of recurrent seizures initially diagnosed with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes. However, the disease's progression more closely resembled self-resolving cerebral cortical encephalitis linked with myelin oligodendrocyte glycoprotein antibodies. In addition, we undertake a systematic review of literature cases to explore the diagnostic significance of magnetic resonance angiography, fluid-attenuated inversion recovery, and specialized markers such as diffusion-weighted imaging and perfusion in discerning fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures syndrome and elucidating its distinctive characteristics.

Headache and Status Epilepticus Reveal Paget's Disease of the Bone.

Paget's disease of the bone (PDB) is a benign osteodystrophy of the elderly characterized by excessive remodeling of bone tissue, mainly in the pelvis, femur, and skull. Its neurological manifestations are numerous and affect both the central and peripheral nervous systems. As headaches are often reported, epileptic seizures remain exceptional. We report the case of a 75-year-old female patient with a history of chronic worsening headache who was admitted to the emergency department for the first episode of a seizure. Brain imaging revealed heterogeneous bone thickening and circumscribed skull osteoporosis. Bone scintigraphy showed pagetoid lesions restricted to the skull and face. Alkaline phosphatases increased. The rest of the biological work-up and the cerebrospinal fluid study ruled out other metabolic causes or central nervous system infections. The patient was treated with bisphosphonates and anti-convulsive treatment. The evolution was satisfactory, with progressive improvement in headache and seizure control, even several months after discontinuation of anti-seizure medication. Our case report highlights the importance of exploring chronic headaches in the elderly, not only in search of lesions of the cerebral parenchyma but also of the structures containing them, in this case, the skull.

Brachial Plexus Rupture: A Neglected Trauma, Is There Any Hope?

Teaching point: Magnetic resonance imaging (MRI) has significantly improved the evaluation of brachial plexus injuries, offering new possibilities for microsurgical repair and contributing to the functional prognosis.

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.

Acute headache and seizures in psychiatric patient revealing atypical location of a ruptured dermoid cyst.

Intracranial dermoid cysts are rare slow-growing cystic lesions. They are frequently extra-axial, intra-axial localization is very rare. These benign congenital ectodermal inclusions cysts have a rare risk of rupture. Ruptured dermoid cysts can manifest with headache, epilepsy seizure, cerebral infarction, meningitis, and hydrocephaly. Neuroimaging features are quite characteristic. We report a case of a 30-year-old male who presented to the emergency room with subacute-onset headaches. CT scan and MRI show a ruptured intracranial dermoid cyst.

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.

Advocacy for Epilepsy Surgery in Africa: Moroccan Experience of 132 Cases.

BACKGROUND: In Africa, epilepsy is a real burden. Temporal lobe epilepsy is the most common drug-resistant focal epilepsy disorder, and temporal lobectomy is the most common effective treatment for patients with drug-resistant epilepsy. OBJECTIVE: We aim to highlight the Moroccan experience in epilepsy surgery and to ascertain its long-term outcome. Through the results of surgical treatment in our series, we hope to raise awareness of the need for epilepsy surgery in Africa and contribute to its development. METHODS: We present a retrospective study of 132 patients who underwent surgery for epilepsy from January 2005 to December 2021 at our institution. The presurgical evaluation was based on clinical screening, interictal electroencephalography, video-electroencephalography, neuropsychological tests, magnetic resonance imaging, and positron emission tomography in some cases. Data are presented as the median and ranges. For all analyses, P values <0.05 were considered statistically significant. RESULTS: Our series includes 132 patients (69 males; 52.27%); the median age at surgery was 24 years (range, 1-64). One hundred and fifteen patients (87%) were operated on for temporal lobe epilepsy, of whom 98 (85%) had anterior temporal lobectomy and 17 (15%) had lesionectomy. Seventeen patients (13%) were operated on for extratemporal epilepsy, of whom 4 had lesionectomy, 7 functional hemispherotomy, and 5 Gamma Knife stereotactic radiosurgery. Our postoperative outcomes 3 months after surgery found 113 patients (85.6%) seizure-free (Engel class I), 16 with Engel class II (12.1%), and 3 with Engel class III (2.3%) in temporal lobe epilepsy. In extratemporal lobe epilepsy, 12 patients (70.5%) showed Engel class I, seizure-free, 4 Engel class II (23.5%), and 1 Engel class III (6%). CONCLUSIONS: These results confirm that most patients with drug-resistant epilepsy may benefit from surgical treatment without submitting to preoperative invasive explorations. This finding should help develop epilepsy surgery widely in Africa.

Lipid-poor vertebral hemangioma mimicking a vertebral metastasis of cervical carcinoma.

Vertebral hemangiomas are classified into typical and atypical forms. On imaging, atypical forms may look like malignant lesions. The confrontation of data from different imaging modalities (MRI; CT; scintigraphy) facilitates the diagnosis.

Schizencephaly: A rare cause of late-onset epilepsy in an adult.

Schizencephaly, a rare congenital cerebral deformity that affects the cerebral mantle and is caused by both hereditary and acquired factors, is defined by a cleft that spans the entire cerebral hemisphere from the lateral ventricle to the cerebral cortex. Adult symptoms have only been described in a few cases in the literature. Hemiparesis, developmental delays, or seizures are manifestations of the clinical presentation, which is dependent on the size and location of the lesion. Neuroimaging investigations, particularly MRI, enable the diagnosis and rule out other causes. Usually discovered at birth, this entity begins to show up in the first several years. To prevent receiving the wrong treatment, the ailment must be correctly diagnosed. We report the observation of a 43-year-old patient with partial epileptic seizures but with an asymptomatic childhood, in relation to unilateral closed cleft schizencephaly associated with gray matter heterotopia. In this case, we highlight the importance of neuroimaging, in particular MRI on the diagnosis of this entity and to identify the associated anomalies.

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.

A spinal cord compression syndrome revealing neurofibromatosis type 1: A case report.

Neurofibromatosis type 1 (NF1), formerly known as von Recklinghausen disease is an autosomal dominant disease with multisystem involvement. In the peripheral nervous system, it leads to the development of benign tumors from the tissue of the spinal or cranial nerve sheaths, known as "neurofibromas." We report the case of a 40-year-old patient with spinal cord compression syndrome in whom spinal MRI revealed cervical, dorsal and lumbosacral neurofibromas revealing neurofibromatosis type 1.

Septo-optic dysplasia PLUS syndrome in a 23 years old patient: A case report.

Septo-optic dysplasia (SOD) is a rare malformation defined by septum pellucidum abnormalities and hypoplasia of the optic nerves and chiasm. It can be associated with cortical development malformations such as schizencephaly, which is then called septo-optic dysplasia plus. It usually manifests at birth, although it may not be diagnosed until childhood, or rarely, adolescence. We report the case of a 23 years old patient, with a history of epilepsy since early childhood never labeled, which was diagnosed with SOD-PLUS with brain MRI in our department.

CLIPPERS manifesting with dizziness: Recognizable and treatable.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a very rare entity which is considered as a treatable condition. In some cases, clinical and radiological findings, associated to favorable evolution on steroids therapy can be sufficiently distinguishable to diagnose chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. We report the case of a 50-year-old man, suffering from acute dizziness with right facial paralysis and limited ocular abduction with his magnetic resonance imaging showing large confluent T2 and fluid-attenuated inversion recovery brainstem hyperintensities extending into the upper cervical spinal cord, infiltrating the basal ganglia and the thalami, with some punctate hyperintensities "peppering" the medial aspects of cerebellar hemispheres. This case illustrates atypical imaging features of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids on magnetic resonance imaging and our work also reviews different studies in the literature and highlights the differential diagnosis.

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.

Bifocal Intracanial Rosai-Dorfman Disease Mimicking Lymphoplasmacyte-Rich Meningioma: Diagnostic Pitfalls About a Case Report.

Rosai-Dorfman disease (RDD) is a clonal histiocytic proliferation characterized by large S100 positive histiocytes with variable emperipolesis. Extranodal locations were confirmed with the central nervous system or the meninges involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas in radiological and intra-operative pathological examination. Histopathology and immunohistochemistry are the keys to definitive diagnosis. We present a case of bifocal Rosai-Dorfman disease in a 26-year-old man, mimicking Lymphoplasmacyte-rich Meningioma. This case allows us to demonstrate the diagnosis pitfalls encountered in this localization.

Outcome of Brain Metastases Treated with Gamma Knife Stereotactic Radiosurgery: A Retrospective Cohort Study of 205 Cases.

BACKGROUND: Brain metastases (BMs) are the most common expansive intracranial lesions in adults. Approximately 50% of patients diagnosed with new BMs will have >1 BM at the diagnosis. We report our experience with BMs treated with Leksell Gamma Knife stereotactic radiosurgery (GKSR) and evaluate the outcomes. METHODS: Patients treated by GKSR in our institution between 2008 and 2021 for BMs were evaluated retrospectively. RESULTS: A total of 205 patients (56.6% women) were included, with a median age of 59 years (range, 25-83 years). The breast (n = 85; 42.5%) and lung (n = 76; 38%) were the most common original locations for the primary tumors. Of the 205 patients, 103 (50.3%) had a single BM and 102 (49.7%) had ≥2 BMs. The median number of multiple BMs treated was 4 (range, 2-43). The mean overall survival (OS) time was 6.00 months (95% confidence interval [CI], 5.07-6.93 months) for all BMs. The median rate of tumor control after radiosurgery was 65% (range, 20%-99%) during a median follow-up of 6.00 months (95% CI, 3-84 months). In the overall population, the 1-, 2-, and 5-year OS rate was 37.55%, 25.12%, and 18.51%, respectively. The mean OS time was longer for those with multiple BMs than for those with a single BM (mean, 10 months [95% CI, 6.67-13.33 months] vs. mean, 5 months [95% CI, 4.21-5.70 months]; P = 0.03). Retreatment, tumor stability (control), and progression influenced the OS of patients with BMs, whether single or multiple (P < 0.001). CONCLUSIONS: GKSR provides good results in terms of OS and better quality of life for patients with BMs, whether single or multiple. Tumor stability and retreatment influenced the OS of patients with BMs.

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.