Cases reported "Cranial Nerve Diseases"

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1/9. Familial congenital corneal anaesthesia.

    Congenital corneal anaesthesia is a cause of severe corneal ulceration and scarring in childhood. Although uncommon, it may be underdiagnosed when present as an isolated entity. Measures such as the use of elbow splints and tarsorrhaphy may be necessary to prevent visual loss. In rare instances, the condition may be inherited. A family is presented with autosomal dominant isolated congenital corneal anaesthesia, and the systemic associations and treatment of the condition are reviewed.
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2/9. Unilateral hypoplasia of the trigeminal ganglion.

    CASE REPORT: We report a case of unilateral anaesthesia of the V1 (ophthalmic) division of the trigeminal cranial nerve presenting with persistent corneal erosions and ulceration secondary to trigeminal ganglion hypoplasia. The patient had a lifelong history of unexplained left-sided ophthalmic symptoms for which numerous diagnoses were provided. Cranial nerve testing demonstrated partial trigeminal dysfunction on the left side. Further investigation eliminated viral etiologies, and subsequent magnetic resonance imaging determined that the patient had a hypo-plastic left trigeminal ganglion. COMMENTS: We present the case to alert clinicians to the possibility of this rare condition.
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3/9. Neurological complications of systemic sclerosis--a report of three cases and review of the literature.

    We report three cases of systemic sclerosis demonstrating four different neurological complications: trigeminal neuropathy, peripheral neuropathy, carpal-tunnel syndrome and prolonged response to local anaesthesia. A review of the literature reveals a wide range of neurological abnormalities associated with systemic sclerosis. When they occur, these are often presenting features.
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4/9. Hemiageusia, hemianaesthesia and hemiatrophy of the tongue.

    A patient with a submandibular gland carcinoma was found clinically to have a unilateral chorda tympani, lingual and hypoglossal nerve deficit. This unique neurological entity of loss of taste sensation of one-half of the tongue (hemiageusia), hemianaesthesia and hemiatrophy of the tongue, has not previously been reported.
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keywords = anaesthesia
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5/9. Trigemino-abducens synkinesis: an unusual case of aberrant regeneration.

    An unusual case of major head trauma is described involving injury to the right third, fifth, sixth and seventh cranial nerves in a basal skull fracture in a young woman. Two years later there persisted a total voluntary abducens nerve palsy, right facial hemianaesthesia and right temporalis and masseter palsy. However, involuntary abduction of the involved eye occurred on eating or chewing. electromyography of the lateral rectus muscle documented aberrant reinnervation to support the clinical findings. Extraocular muscle surgery improved the compensatory head posture and minimized the chewing-induced abduction. The mechanisms for acquired synkinesis and the anatomy of the involved nerves are reviewed. It is postulated that regenerating motor fibres of the trigeminal nerve were misdirected along proprioceptive channels to the lateral rectus in the case reported here.
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keywords = anaesthesia
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6/9. Congenital trigeminal anaesthesia. A review and classification.

    Forty-three patients with the syndrome of congenital trigeminal anaesthesia (CTA) have been reported to date. The cases have been divided into three groups based on the presence and nature of any associated clinical problems. Three additional cases are presented as characteristic of each of the groups. A different aetiology is proposed for each group. Group I consists of patients with CTA as an isolated finding. It is almost always bilateral, and typically involves the distribution of only the first division of the fifth nerve. There is no evidence of other neurological abnormalities or associated mesoectodermal congenital anomalies. The aetiology is suspected to be a primary neural hypoplasia. patients with associated congenital mesenchymal anomalies were placed in the second group. CTA was often a minor part of another well defined, more extensive clinical syndrome, such as Mobius or oculoauriculovertebral dysplasia (OAVD). The sensory abnormality was either unilateral or bilateral. The skin of the face was almost always involved, as well as the cornea and conjunctiva. This may be a heterogenous group with multiple aetiologies having in common an injury early in embryogenesis. The third group is defined as patients with CTA without evidence of mesenchymal dysplasia. These cases differ from the first group primarily because of the presence of associated focal brainstem signs. The aetiology is thought to be due to focal neural dysgenesis secondary to a prenatal injury, possibly vascular in nature.
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7/9. Craniosynostosis, ataxia, trigeminal anaesthesia and parietal alopecia with pons-vermis fusion anomaly (atresia of the fourth ventricle). Report of two cases.

    A probably new syndrome of craniosynostosis, ataxia, trigeminal anaesthesia, and parietal alopecia area associated with pons-vermis fusion anomaly (atresia of the fourth ventricle), in two unrelated Mexican girls, is described. The cerebellar anomaly was proven by CT scan only and it correlated with ataxia. Other abnormalities seen in both patients were midfacial hypoplasia, bilateral corneal opacities, low-set ears, mental retardation and short stature. This disorder could be a new neurocutaneous syndrome.
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keywords = anaesthesia
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8/9. Mental nerve anaesthesia as a result of mandibular metastases of prostatic adenocarcinoma.

    Three cases of mental nerve anaesthesia as a result of metastatic deposits from prostatic carcinoma are presented. They were referred to Consultant Oral and Maxillofacial Surgeons by alert GDPs. This highlights the fact that dental surgeons must be aware that systemic illness may manifest in the mouth and appropriate referral is essential.
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keywords = anaesthesia
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9/9. Multiple cranial nerve palsies complicating retrobulbar eye block.

    This report details a case of accidental brainstem anaesthesia complicating retrobulbar block for cataract surgery in an 83-year-old man. Prompt diagnosis and treatment resulted in a successful outcome. The many possible presentations of brainstem anaesthesia are described and causative mechanisms are discussed.
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