Cases reported "Cranial Nerve Diseases"

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1/6. Progressive cranial nerve palsy following shunt placement in an isolated fourth ventricle: case report.

    Cranial nerve palsy is rarely seen after shunt placement in an isolated fourth ventricle. In the few reports of this complication, neuropathies are thought to be caused by catheter injury to the brainstem nuclei either during the initial cannulations or after shrinkage of the fourth ventricle. The authors treated a child who suffered from delayed, progressive palsies of the sixth, seventh, 10th, and 12th cranial nerves several weeks after undergoing ventriculoperitoneal shunt placement in the fourth ventricle. magnetic resonance imaging revealed the catheter tip to be placed well away from the ventricular floor but the brainstem had severely shifted backward, suggesting that the pathogenesis of the neuropathies was traction on the affected cranial nerves. The authors postulated that the siphoning effect of the shunt caused rapid collapse of the fourth ventricle and while the cerebellar hemispheres were tented back by adhesions to the dura, the brainstem became the only mobile component in response to the suction forces. Neurological recovery occurred after surgical opening of the closed fourth ventricle and lysis of the basal cistern adhesions, which restored moderate ventricular volume and released the brainstem to its normal position.
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2/6. A case of AIDS associated cryptococcal meningitis with multiple cranial nerve neuropathies.

    Cryptococcal meningitis is a common opportunistic infection among patients with AIDS. Cranial nerve neuropathies are well-known complications that occur due to increased intracranial pressure and inflammation of cranial nerves in such patients but have not been previously reported to involve more than four cranial nerves simultaneously. Our patient had involvement of five cranial nerves resulting in the complete loss of vision and hearing as well palsies of the third, sixth and seventh cranial nerves. He was treated with multiple antifungal medications. Repeated high volume lumbar punctures and Ommaya reservoir were used to lower intracranial pressure. At the time of discharge the patient had complete recovery of the functions of third, sixth and seventh cranial nerves bilaterally and partial recovery of hearing and vision.
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3/6. 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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4/6. Sclerosteosis: neurogenetic and pathophysiologic analysis of an American kinship.

    We studied an American kinship with sclerosteosis, an autosomal-recessive disorder of bone remodeling and bone overgrowth of the calvaria, skull base, and tubular bones. Unlike osteopetrosis, which is attributed to abnormal immune and osteoclast function as well as bone resorption, sclerosteosis appears to be primarily a disorder of osteoblast (bone formation) hyperactivity. Related to cranial vascular and neural foraminal narrowing and reduced intracranial volume, affected patients with sclerosteosis demonstrate frequent seventh nerve palsy, progressive optic and cranial neuropathies, mixed hearing loss, brainstem compression, intracranial hypertension with increased elastance, and sudden, premature death. Management should involve early childhood identification of homozygotes, monitoring and aggressive treatment of intracranial hypertension, and extensive bone removal from skull, posterior fossa, and cervical spine.
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5/6. central nervous system complications of mycoplasma pneumoniae.

    Three cases of central nervous system complications of mycoplasma pneumoniae infections are reported. There were several atypical findings: the first patient presented with an acute disseminated encephalitis with normal cerebrospinal fluid and hypogammaglobulinaemia. The second patient presented with a left sixth nerve palsy followed six weeks later by choreoathetoid movements and an acute psychosis. The third patient presented with an acute encephalitis associated with acute renal failure secondary to rhabdomyolysis and was the only patient who did not completely recover; he was discharged akinetic and mute, but with normal renal function. The diagnosis of complications of mycoplasma pneumoniae may be difficult. In two of the three cases a raised red blood cell volume secondary to a high titre of cold agglutinins rapidly led to the correct diagnosis.
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6/6. Clinical, physiologic, and pathologic evidence for vagus dysfunction in a case of traumatic brain injury.

    A trauma victim with locked-in syndrome demonstrated severely decreased bowel sounds, intact response to suppository, and elevated, but unchanging pulse. Absent cardiac response to tracheal suctioning, high gastric residual volumes, and pulmonary edema in response to a urecholine challenge demonstrated dysfunction in the automatic system. Symptoms persisted for 2 1/2 years until death. At autopsy, asymmetric bilateral involvement of the dorsal motor nucleus of the vagus and of the nerve tract in the medulla were demonstrated. In contrast, a control subject with locked-in syndrome caused by a stroke did not demonstrate these phenomena. In trauma patients with delayed gastric emptying, measurement of the heart rate response to deep suctioning may lead to the diagnosis of this vagus dysfunction syndrome.
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