Cases reported "Cranial Nerve Diseases"

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1/63. Bilateral recurrent nerve paralysis associated with multinodular substernal goiter: a case report.

    Substernal goiter is an infrequent occurrence and is found in two to five per cent of all patients undergoing thyroid surgery. These lesions are well known to cause respiratory symptoms and alterations in phonation due to direct compression of airway structures. Infrequently, unilateral recurrent nerve palsy has been reported in patients with substernal goiter. We report a case of bilateral recurrent nerve palsy associated with multinodular substernal goiter in an 89-year-old female who presented in respiratory distress.
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2/63. Temporary multiple cranial nerve palsies in a patient with type 1 diabetes mellitus.

    Remittent isolated palsy of peripheral or of upper cranial nerves in diabetic patients is well documented, but paralysis of a lower cranial nerve or an isolated branch of any cranial nerve has rarely been reported. In the case described, besides temporary hypoglossal and facial nerve palsies previously, unilateral temporary vocal cord palsy caused by right inferior laryngeal nerve (recurrent) paralysis associated with type 1 diabetes mellitus is presented. hoarseness and vocal cord palsy of the patient, as in the case of her first admission with other complaints due to other cranial nerve palsies, totally remitted, presumably both owing to improved metabolic control.
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3/63. Mobius and Mobius-like patients: etiology, diagnosis, and treatment options.

    The surgical goal in Mobius patients is far more modest and differs from patients with unilateral developmental facial paralysis. It is impossible to restore a true smile in these mask-like, expressionless faces. Despite sophisticated microneurovascular transplantations, movement can only be restored along one vector and enhanced firmness in the cheeks, thus multiple differentiated facial animation is not achievable. A detailed neurological evaluation can identify possible motor donors or residual function, which can be used for additional dynamic restorations. Due to the multiple cranial nerve involvement a thorough clinical and electrophysiological examination is mandatory. In addition, electromyographic survey of the potential motor donors is very helpful to avoid weak wasted regeneration and prevent further downgrading of function. Because of the variety of cranial nerves involved in Mobius' syndrome, a standard procedure for dynamic restoration cannot and should not be promoted; instead, a careful preoperative objective and quantitative assessment should guide the reconstructive surgeon to the optimal reconstruction strategy. Useful movement can be restored in afflicted patients that may signal physical and psychological rehabilitation.
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4/63. Garcin syndrome resulting from a giant cell tumor of the skull base in a child.

    Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.
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5/63. Pseudoaneurysm of the cervical carotid artery with hypoglossal and glossopharyngeal nerve paralysis.

    We report a case of a pseudoaneurysm of the internal carotid artery with associated hypoglossal and glossopharyngeal nerve paralysis. Correlative findings with magnetic resonance imagery and arteriography are described.
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6/63. Gliomatosis cerebri in a young patient showing various cranial nerve manifestations: a case report.

    A case of gliomatosis cerebri in a 27-year-old man showing various cranial nerve manifestations is described. He was diagnosed as having cranial mononeuritis multiplex (bilateral oculomotor nerve paralysis, left facial nerve paralysis, bulbar palsy manifestations, and hypoglossal nerve paralysis) and was hospitalized in the neurology department on August 1, 2000. Although he continued to visit the neurology department after discharge, his manifestations showed no improvement. He was sent to our department for brain biopsy in August 2001. A biopsy performed at the Sylvian fissure from the frontal lobe/temporal lobe cortex showed high intensity on T2-weighted and Flair magnetic resonance imaging (MRI). The pathological findings were diffuse low-grade astrocytoma infiltrating between the pia mater and the cerebral cortex. We believed that the astrocytoma spreading on the subpia mater was responsible for the various cranial nerve manifestations, and we started whole-brain irradiation (46 Gy) interferon (IFN)-beta D.I.V. from September 2001. The pathological findings of the brain biopsy showed diffuse astrocytoma. The clinical presentation was dramatically improved after radiotherapy. It seemed that this tumor had spread along the subpia mater and subependyma. When he was discharged in early December, he walked by himself. The characteristic features of this case are that no lesion in the cerebellum or brain stem was found on MRI, even though the main manifestations were cerebello-brain stem manifestations, and biopsy of the cerebral cortex revealed astrocytoma. It should be noted that the clinical manifestations of astrocytoma in some cases are dissociated from the imaging observations.
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7/63. Bilateral vocal cord paralysis secondary to esophageal compression.

    Bilateral vocal cord paralysis is most commonly caused by trauma, malignancy, and neurologic disorders. Cases secondary to esophageal compression of the recurrent laryngeal nerves are rare. We report a patient admitted with an exacerbation of achalasia who developed acute respiratory distress from bilateral immobile vocal cords. Imaging studies revealed impressive dilation of the cervical esophagus causing compression of both recurrent laryngeal nerves. After securing the airway and decompression of the esophagus, mobility of the vocal cords returned within 1 week. This case shows the importance of a careful airway workup in patients with esophageal distention. Early decompression may prevent permanent recurrent laryngeal nerve injury and airway obstruction.
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8/63. Mojave rattlesnake envenomation: prolonged neurotoxicity and rhabdomyolysis.

    An 11-year-old girl presented to the emergency department with hypoventilation and shock after being bitten by a Mojave rattlesnake. intubation was required, and she improved rapidly after fluid resuscitation and antivenom administration. She was extubated four hours after envenomation and did well. The patient subsequently developed increased weakness and cranial nerve paresis and required reintubation for respiratory failure at 30 hours after envenomation despite administration of 30 vials of antivenom. She improved after administration of additional antivenom and was extubated ten hours later. Twenty-four hours after envenomation, signs of rhabdomyolysis were noted with myoglobinuria and a creatine phosphokinase level of 96,400 units/L. Myoglobinuric renal failure was treated with mannitol, hydration, and alkalinization of the urine. The patient's renal and neurological functions improved steadily during the following three to four days. Neurotoxic and myotoxic effects of Mojave venom are known to occur but are not well documented in human beings. Recognition of potential complications from envenomation such as respiratory paralysis and rhabdomyolysis with myoglobinuric renal failure is critical.
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9/63. Raised intracranial pressure and visual complications in AIDS patients with cryptococcal meningitis.

    The clinical course of cryptococcal meningitis in AIDS shows some important differences from the features of the illness in non-AIDS patients. Complications such as raised intracranial pressure and visual impairment that are recognised in non-AIDS patients may be less frequent in those with AIDS. Persistent intracranial hypertension should be managed actively to prevent visual impairment. In AIDS patients, in whom ventriculo-peritoneal shunts carry additional risks, acetazolamide can be used successfully to lower the CSF pressure and prevent visual loss.
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10/63. 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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