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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a previously healthy 4 year-old African American female who presented to the emergency department with acute onset of unilateral abducens nerve palsy and torticollis. Within 12 h of presentation, the patient's symptoms progressed to include ipsilateral facial nerve palsy and gait ataxia. On exam, the patient demonstrated right cranial nerve VI and VII palsies, ataxic gait with left lateropulsion, spasticity of bilateral lower extremities with clonus, and the presence of bilateral Babinski sign. MRI of the brain and spinal cord revealed severe Chiari I malformation with associated extensive holochord syringomyelia and syringobulbia. The patient underwent successful surgical decompression 72 h after initial presentation. We review the literature on Chiari malformations and syringomyelia, including epidemiology, presentation and neurological manifestations, and treatment recommendations. As our patient had a very acute presentation, we additionally review the previously reported cases of acute and atypical presentation of patients with Chiari I malformation and syringomyelia. The aim of this report is to make practitioners aware of the acuteness with which children with Chiari malformation type I with syringomyelia and syringobulbia can present.
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PMID:Acute VI nerve palsy in a 4 year-old girl with Chiari I malformation and pontomedullary extension of syringomyelia: case report and review of the literature. 2156 92

We describe a 62-year-old woman who presented with a history of ataxia, dizziness and urinary urgency. Neurological examination disclosed a positive Romberg sign, ataxia and postural instability. A magnetic resonance imaging (MRI) scan showed Chiari type 1 malformation (CM1). Forty-eight months later, the patient was clinically improved and underwent a second MRI examination, which showed complete resolution of the Chiari 1 malformation. Spontaneous resolution of CM1 is exceptionally rare and has to be considered in the radiological and clinical management.
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PMID:Spontaneous resolution of Chiari type 1 malformation. A case report and literature review. 2385 87

The Arnold-Chiari Type 1 malformation (CM1) is a rare congenital abnormality characterized by ectopia or caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical spine, resulting in crowding at the craniocervical junction. It seldom presents in childhood with symptoms and a normal neurological examination. More typically, CM1 presents in young adult women with neurological symptoms including a headache, cervical pain, cranial nerve palsies, neurosensory deficit, and ataxia. Ocular manifestations associated with Chiari I include third and sixth cranial nerve paresis and convergence/divergence abnormalities. Papilledema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To underscore this noteworthy complication, the authors report a case of an 8-year-old boy who presented with nyctalopia and suboccipital headaches, but was diagnosed serendipitously as a case of papilledema due to Chiari I malformation.
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PMID:Not all cases of nyctalopia are benign: Unusual and serendipitous presentation of Arnold-Chiari Type 1 malformation at a Pediatric Tertiary Care Center. 2785 98

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention.
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PMID:Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery. 2801 69

This study aimed to summarize the clinical features, diagnosis, and treatment of Chiari malformation type I- (CM-1-) associated syringobulbia. We performed a literature review of CM-1-associated syringobulbia in PubMed, Ovid MEDLINE, and Web of Science databases. Our concerns were the clinical features, radiologic presentations, treatment therapies, and prognoses of CM-1-associated syringobulbia. This review identified 23 articles with 53 cases. Symptoms included headache, neck pain, cranial nerve palsy, limb weakness/dysesthesia, Horner syndrome, ataxia, and respiratory disorders. The most frequently involved area was the medulla. Most of the patients also had syringomyelia. Surgical procedures performed included posterior fossa decompression, foramen magnum decompression, cervical laminectomy, duraplasty, and syringobulbic cavity shunt. Most patients experienced symptom alleviation or resolution postoperatively. A syringobulbic cavity shunt provided good results in refractory cases. Physicians should be aware of the possibility of syringobulbia in CM-1 patients, especially those with symptoms of sudden-onset brain-stem involvement. The diagnosis relies on the disorder's specific symptomatology and magnetic resonance imaging. Our review suggests that the initial therapy should be posterior fossa decomposition with or without duraplasty. In refractory cases, additional syringobulbic cavity shunt is the preferred option.
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PMID:Syringobulbia in Patients with Chiari Malformation Type I: A Systematic Review. 3101 90