RESUMO
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.
RESUMO
This article presents a unique case of ipsilateral hemiparesis in a 66-year-old individual, contrary to the conventional understanding of supratentorial strokes causing contralateral neurological deficits. The patient exhibited persistent weakness and sensory abnormalities on the left side of the body following a left occipital infarct. Neuroimaging revealed a chronic stroke in the left occipital lobe, with diffusion tensor imaging demonstrating uncrossed pyramidal tracts at the level of the medulla. The discussion encompasses the anatomical basis of corticospinal tract crossing, historical perspectives, and previous documented cases of ipsilateral strokes. The rarity of complete uncrossed corticospinal tracts without underlying congenital abnormalities or genetic disorders is highlighted. The study underscores the importance of considering such atypical presentations in stroke evaluations and the role of advanced imaging techniques in confirming diagnosis and understanding underlying mechanisms.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
En plaque meningioma is a rare type of meningioma characterized by an infiltrative nature, sheet-like growth, and at times invading the bone. We report here a case of en plaque meningioma. The patient was a 66-year-old woman presenting with headache and painful bilateral proptosis. Cerebral magnetic resonance imaging revealed a bilateral en plaque meningioma showed as a bilateral hyperostotic of greater wing sphenoid bone associated with bilateral thickening and enhancement of the dura in the anterior temporal area and the retroclival region invading bilaterally the cavernous sinus, the prechiasmatic portion of the optic nerve, and the lateral rectus muscle of the orbit through the superior orbital fissure causing bilateral exophthalmia. Due to invasion of the cavernous sinus and the orbital apex, a subtotal but extensive removal combined with bony decompression of the cranial nerves at the superior orbital fissure and optic canal followed by adjuvant radiotherapy frequently produces good functional and cosmetic results, and over a 3-year period, follow-up magnetic resonance imaging scans showed no obvious signs of recurrence.
RESUMO
Heroin-induced leukoencephalopathy (HLE) is a rare toxic encephalopathy associated primarily with heroin inhalation, commonly referred to as "chasing the dragon." This study presents a clinical case of a 27-year-old polydrug user diagnosed with HLE during hospitalization for rapidly progressive flaccid tetraplegia and aphasia. The clinical manifestations encompassed cerebellar and bulbar dysfunction, coupled with motor impairment and altered consciousness. Based on the clinical data and MRI results, HLE was identified as the most likely cause. This article aims to provide insights into the clinical and radiological aspects of HLE, emphasizing the diagnostic significance of radiological findings. The gold standard examination for diagnosis is MRI, crucial due to the difficulties in obtaining histological confirmation for this rare condition.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.
RESUMO
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.