Cases reported "Cranial Nerve Diseases"

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1/9. Chiari malformation, cervical spine anomalies, and neurologic deficits in velocardiofacial syndrome.

    The purpose of this investigation was to evaluate the prevalence of Chiari malformation, cervical spine anomalies, and neurologic deficits in patients with velocardio-facial syndrome. This study was a prospective evaluation of 41 consecutive patients with velocardiofacial syndrome, documented by fluorescence in situ hybridization, between March of 1994 and September of 1998. The 23 girls and 18 boys ranged in age from 0.5 to 15.2 years, with a mean age of 6.7 years. Nineteen patients were assessed with magnetic resonance imaging, 39 underwent lateral cephalometric radiography, and all patients were examined for neurologic deficits. Eight of 19 patients (42 percent) had anomalies of the craniovertebral junction, including Chiari type I malformations (n = 4), occipitalization of the atlas (n = 3), and narrowing of the foramen magnum (n = 1). One patient with Chiari malformation required suboccipital craniectomy with laminectomy and decompression. Fourteen of 41 patients (34 percent) had demonstrated neurologic deficits; 10 patients (24 percent) had velar paresis (6 unilateral and 4 bilateral). Chiari malformations, cervical spine anomalies, and neurologic deficits are common in velocardiofacial syndrome. Because these findings may influence the outcome of surgical intervention, routine assessment of patients with velocardiofacial syndrome should include careful orofacial examination, lateral cephalometric radiography, and magnetic resonance imaging of the craniovertebral junction.
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2/9. Intracanalicular aneurysm of the anterior inferior cerebellar artery revealed by multi-detector CT angiography.

    A 62-year-old woman had sudden-onset headache and posterior neck pain, and a subarachnoid hemorrhage was revealed by unenhanced CT. Both multi-detector CT angiography and digital subtraction angiography were performed and revealed a small intracanalicular aneurysm of the left anterior inferior cerebellar artery. The patient underwent successful retrosigmoid craniectomy and trapping of the aneurysm. This case shows the ability of multi-detector CT angiography to indicate bony landmarks that can alter the surgical approach.
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3/9. Enigmatic trigeminal sensory neuropathy diagnosed by facial skin biopsy.

    Facial paraesthesia due to perineural malignant infiltration is a well recognised complication of basal and squamous cell carcinomas of the head and neck. Perineural involvement was originally attributed to involvement of the perineural lymphatics; however subsequent studies have demonstrated conclusively that these lymphatics do not exist and that the invasion occurs along the line of least resistance. Previous studies on perineural spread of carcinomas of the head and neck have emphasised diagnostic biopsy of an involved nerve (e.g. the infraorbital, mental or major branches of the trigeminal nerve), or at times craniectomy with exploration of the gasserian ganglion. We suggest that in many cases the diagnosis can be obtained by biopsy of the anaesthetic skin alone, without recourse to more involved biopsy techniques. The following case report illustrates this point.
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4/9. accessory nerve neuroma presenting as recurrent jugular foramen syndrome.

    We report a patient with spontaneous recovery and recurrence of a jugular foramen syndrome secondary to an accessory nerve neuroma. He showed sudden onset of unilateral palsy of the ninth, tenth and eleventh cranial nerves in 1982. He recovered almost fully and in 1990 the palsies recurred. MRI revealed a small mass in the right jugular foramen. The tumour was resected via suboccipital craniectomy, and diagnosed as a neuroma of the eleventh cranial nerve.
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5/9. Delayed cranial neuropathy after neurosurgery caused by herpes simplex virus reactivation: report of three cases.

    BACKGROUND: Delayed cranial neuropathy is an uncommon complication of neurosurgical interventions of which the exact etiology is uncertain. Several authors have hypothesized that reactivation of herpesviruses may play a role. CASE DESCRIPTIONS: The first patient underwent microvascular decompression of the left facial nerve because of hemifacial spasm. Nine days postoperatively, he developed severe facial weakness on the ipsilateral side. The polymerase chain reaction for herpes simplex virus (HSV) was positive in the cerebrospinal fluid (CSF). Treatment with intravenous acyclovir was initiated, after which a rapid and marked improvement was observed. The second patient developed left-sided facial numbness 20 days after microvascular decompression of the left facial nerve. The polymerase chain reaction for HSV was positive in the CSF. Treatment with intravenous acyclovir resulted in full recovery. The third patient underwent a suboccipital craniectomy with excision of a meningioma located at the left petrosal apex. Three months postoperatively, she developed multiple cranial neuropathies (involving cranial nerves V, VI, VIII, and XII). This was accompanied by serologic evidence of HSV reactivation and a positive polymerase chain reaction for HSV in the CSF. The patient was successfully treated with intravenous acyclovir. CONCLUSIONS: The 3 reported cases provide evidence that delayed postoperative cranial neuropathy can be caused by HSV reactivation and can involve multiple cranial nerves. An increased awareness of this treatable postoperative complication is warranted.
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6/9. Sclerosteosis involving the temporal bone: clinical and radiologic aspects.

    Sclerosteosis is one of the rare, potentially lethal, autosomal recessive, progressive, craniotubular sclerosing bone dysplasias. syndactyly of the second and third or other fingers is evident at birth. hyperostosis and sclerosis are most prominent in the skull and tubular bones, and are frequently associated with excessive height and weight. The typical facial deformity is apparent by the age of 5 years. The changes involving the temporal bone include a marked increase in overall dimensions, extreme sclerosis, and narrowing and constriction of the external ear canal, middle ear cleft, internal acoustic meatus, and falloppian canal. Impairment of hearing, as a rule bilateral, is a frequent presenting symptom which may manifest in early childhood. Initially it is an expression of interference with sound conduction; later it may become associated with a loss of sound perception. Impairment of facial nerve function is another salient feature which occasionally is present at birth. As a rule, it manifests initially as a unilateral, recurrent paresis, eventually progressing to a bilateral permanent partial loss of facial nerve function. Since impairments of hearing and facial nerve function are two of the salient features, present at birth or in early childhood, the responsibility for recognizing the disease often falls upon the otolaryngologist. The clinical and radiologic features permit not only early recognition of the disorder but also differentiation from similar bony dysplasias. Hyperosteosis and sclerosis of the skull lead to thickening and distortion of the calvaria, cranial base, and foramen magnum resulting in reduction of the intracranial volume, interference with the cerebral blood flow, resorption of cerebrospinal fluid, and gradual increase of intracranial pressure. Severe headaches resulting from this mechanism often develop in early adulthood, and several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum. decompression of the transverse sigmoid sinus and jugular bulb may be lifesaving, combined with a posterior, and if necessary, an anterior, craniectomy for decompression. Early decompression of the internal acoustic meatus and falloppian canal may help in the preservation of cochlear and facial nerve function.
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7/9. Angiocentric T-cell lymphoma presenting with multiple cranial nerve palsies and retrobulbar optic neuropathy.

    Angiocentric T-cell lymphoma (lymphomatoid granulomatosis) may present with prominent central nervous system (CNS) findings with variable radiographic features. We describe a patient who presented with multiple cranial nerve palsies involving the left optic nerve, left facial nerve, left ocular motor nerves, and bilateral acoustic nerves. Enhancement of the right temporal meninges and a cavernous sinus mass were noted on magnetic resonance (MR) scan. A right temporal craniectomy and meningeal biopsy were performed. Meningeal biopsy revealed atypical angiocentric granulomatous lymphoid infiltrates without associated necrosis, giant cells, or granuloma formation. Morphologic and T-cell, receptor gene rearrangement findings were diagnostic of an angiocentric T-cell lymphoma. Retrobulbar optic neuropathy and multiple cranial nerve palsies may be the presenting features of angiocentric T-cell lymphoma. The neurologic and unique radiographic changes in our case expand the previously reported findings in CNS angiocentric T-cell lymphoma.
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8/9. Intracranial and extracranial reduction osteoplasty for craniodiaphyseal dysplasia.

    Craniodiaphyseal dysplasia is a rare, sporadic form of craniotubular bone dysplasia, characterized by massive generalized hyperostosis and sclerosis, particularly of the skull and facial bones, leading to severe deformity. The clinical course is typically characterized by progressive encroachment of the craniofacial foramina and brain by the relentless deposition of bone. Compression of cranial nerves, the foramen magnum, and intracranial contents commonly leads to blindness, loss of hearing, and death. This report describes a unique case of craniodiaphyseal dysplasia manifesting with asymmetric craniofacial and axial hyperostosis. The tubular bones demonstrated the characteristic diaphyseal endostosis, undertubulation, and relative overgrowth on the involved side. Significant brain compression with signs and symptoms of increased intracranial pressure was managed successfully with decompressing craniectomy at age 12 years, enlarging the anterior and middle fossae. Calvarial thickness measured nearly 4 cm. Further calvarial, midfacial, and mandibular recontouring were performed 6 and 22 months later. Follow-up in our case indicates that close observation is mandatory to manage further progression of the disease.
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9/9. Trigeminal trophic syndrome.

    A case report of a 51-year-old woman who developed trigeminal trophic syndrome following craniectomy is presented. This syndrome represents trophic ulceration by self-induced trauma to skin in the trigeminal area. Treatment of the present case was complicated by the patient's underlying psychological problems.
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