Les manifestations neuro-encéphaliques de la sclérose tubéreuse de Bourneville

Tuberous sclerosis is a phacomatosis resulting from an autosomal dominant inheritance. It is characterized by the presence of multiple hamartomas in various organs, especially the brain, the skin, the kidneys and the heart. The diagnosis of tuberous sclerosis is based on imaging and clinical examination, where magnetic resonance imaging constitutes the key investigation showing characteristic brain lesions. Neuro-encephalic manifestations may be particularly severe, and may even be life threatening. The authors report personal cases series and review the literature highlighting epidemiology, clinical features and imaging of neuro-encephalic tuberous sclerosis.     

Cerebellar abscesses secondary to occipital dermoid cyst with dermal sinus: case report

BACKGROUND: Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION: A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.     

Cavernous hemangioma of the parietal bone. A case report

Intraosseous cavernous hemangiomas are a rare finding in the calvarium. It is a benign tumors arising from the intrinsic vasculature of the bone. We report one case observed in a 31 year-old female, presented with a progressive left parietal mass. A plain X-ray of the skull and cranial CT-scan showed a osteolytic lesion in the left parietal bone. Surgery consisted of total resection of the lesion and cranioplasty. The postoperative course was uneventful. Histological study revealed a cavernous hemangioma of the diploe. In view of this observation and the literature review, clinical; radiological and therapeutic aspect of this rare entity are discussed.     

Benign fronto-orbital osteoblastoma arising from the orbital roof: case report and literature review

BACKGROUND: Osteoblastoma is an uncommon benign osteogenic neoplasm that rarely involves the orbit. Intracranial and intraorbital extension causing neurologic and ophthalmologic symptoms and signs is very unusual. We report the case of an osteoblastoma of the orbital cavity with ethmoidal and anterior cranial fossa extension presenting as unilateral proptosis. Manifestations and management of this rare fronto-orbital lesion are discussed, and the relevant literature is reviewed. CASE DESCRIPTION: This 22-year-old man experienced a 3-month history of progressive left proptosis without neurologic symptoms. Computed tomography and magnetic resonance imaging scans demonstrated a bony mass involving the roof of the left orbit and extending laterally to the adjacent ethmoid cells and upward to the lower part of the homolateral frontal convexity without parenchymal abnormality. A presumptive diagnosis of osteoma was considered. A left fronto-orbital craniotomy was performed. At surgery, the tumor was well circumscribed by a sclerotic margin. It was granular with bony spicules, destroying the orbital roof and involving the orbital cavity, ethmoidal cells, and anterior cranial fossa. The lesion was totally removed, and the anterior cranial base reconstructed. The histologic features were typical of benign osteoblastoma. After a follow-up period of 12 months, the patient has remained well without evidence of recurrence. CONCLUSION: Osteoblastoma should be considered in the differential diagnosis with other fronto-orbital bone-forming lesions. Although generally regarded as benign, a complete resection is recommended to prevent the possibility of postoperative recurrence and malignant transformation. Twelve previously reported cases were also reviewed.     

Influence of cranial morphology on the location of chronic subdural haematoma

Background  

The purpose of the present study was to evaluate the relationship between cranial morphology and location of a chronic subdural haematoma (CSDH) in patients with and without intracranial vault asymmetry.     

Idiopathic spinal epidural lipomatosis without obesity: a case with relapsing and remitting course

STUDY DESIGN: Case report of a rare form of idiopathic spinal epidural lipomatosis (SEL) manifesting with relapsing and remitting course. OBJECTIVE: To describe this very rare clinical and pathological condition and the results of surgical intervention. SETTING: A department of neurosurgery in Morocco. METHODS: A 24-year-old man presented with a 2-year history of mid-thoracic back pain and progressive neurogenic claudication with two episodes of remitting and relapsing course. Spinal magnetic resonance imaging revealed a fatty epidural mass extending from T4 to T9. Posterior decompression was performed, and he was followed for 3 years after the operation. RESULTS: Pathological examination of the surgical specimen revealed nodules of mature fat cells without neoplasm. The patient's symptoms completely resolved after surgical decompression. CONCLUSION: Fluctuating clinical course has not been reported previously in SEL. Such pathology should be considered in the differential diagnosis of demyelinating diseases.     

Arachnoiditis ossificans of the cauda equina

A case of post-traumatic arachnoiditis ossificans of the cauda equina is reported. The lesion is a rare pathological entity usually confined to the thoracic and high lumbar regions that can cause progressive spinal cord and cauda equine compression. The pathophysiology and therapeutic strategy of this rare entity are still controversial.