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

From a clinical, radiological and morphological study of 9 horses with congenital malformations of the occiput, atlas and axis, and from a study of 2 reported cases, 3 diseases were defined: A. Familial occipitalisation of the atlas with atlantalisation of the axis in Arabian horses (7 cases in this report and the case reported by Leipold, et al., 1974). These horses had congenital atlantooccipital fusion, hypoplasia of the atlas and dens, malformation of the axis and modification of the atlantoaxial joint. B. Congenital asymmetrical occipitoatlantoaxial malformation (2 cases in this report). A Standardbred and a Morgan horse had atlantooccipital fusion, a wedge shaped vertebral piece attached to the caudal end of the axis and sigmoid scoliosis of the cervical vertebrae. C. Asymmetrical atlantooccipital fusion (the case reported by Schmaltz, 1915). This horse of an unknown breed had asymmetrical fusion between the atlas and occiput and cervical scoliosis. The clinical syndromes shown by horses with these malformations were variable but were broadly classified as: 1. Foal dead at birth, seen in one foal with A. 2. Tetraparesis at birth, seen in 5 foals with A. These foals were born with signs varying from tetraparesis to tetraplegia. 3. Progressive ataxia, seen in 2 foals with A. Clinical signs were due to a progressive focal cervical compressive myelopathy. 4. Congenital cervical scoliosis/deviated head, seen in the 2 horses with B and the horse with C. These horses had no signs of spinal cord or brain disease. The diagnoses were made clinically by palpation of the occipitoatlantoaxial region and were confirmed radiographically and/or by post mortem examination in all except one case. Pedigree analysis showed the familial nature of the particular occipitoatlantoaxial malformation seen in horses of only the Arabian breed.
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PMID:Congenital occipitoatlantoaxial malformations in the horse. 56 4

A 4-year-old male Great Dane with a history of progressive ataxia and associated bilateral atrophy of the muscles of the shoulder was found to have severe osseous metaplasia of the spinal dura mater. These dural ossifications were directly associated with underlying spinal cord degeneration. No other condition could be identified that would account for the pathologic changes in the spinal cord. The lower motor neuron (LMN) type weakness was in contrast to the upper motor neuron (UMN) type weakness said to occur with this disease and recently reported to be due to primary degenerative myelopathy.
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PMID:Osseous metaplasia of the spinal dura mater in a Great Dane. 80 43

Fanconi-Zinsser's disease is a serious involutive myelopathy responsible for pancytopenia, hyperpigmentation, oralplakia and onychodystrophy, together with lesser dysmorphisms in many cases. The marrow blood picture is reminiscent of the better-known Fanconi's disease, while the skin and mucosa picture is similar to that of congenital dyskeratosis - hence the combined name. A typical case, but complicated by Lewandowsky's disease for the first time in the literature, is presented. Papova-virus was noted in the typical verrucae. The modern "pathology due to genome instability" is examined. Lewandowsky's disease is regarded as a familial form and precancerous, owing to its possible Bowenoid transformation. The association is seen as particularly significant in stressing the disorder of the immunocompetent syste, this being most evident from the finding of serum anti-red-cell auto-antibodies and a deficiency of T lymphocytes. Fanconi-Zinsser's disease has only been reported 21 times in the literature, mostly in males. Incomplete forms are, however, noted in relatives, suggesting that it originates in a genetic disorder whose transmission modality is not clear, though incomplete dominance is suspected. Genome instability is probably responsible behind the onset of the disease and its neoplastic complications - these being also feature of other forms provoked by such instability, such as Bloom's syndrome and ataxia telengiectasia. Fanconi's disease also has marked neoplastic tendencies. Clinically, Fanconi-Zinsser's disease can be classified as distinct, since it has signs and an evolutive modality that distinguish it from Franconi's disease, Estren-Damesheck's syndrome and amegakaryocytic thrombocytopenia. Genetically, it can be seen that all these diseases are referable to "pathology due to genome instability".
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PMID:[Fanconi-Zinsser disease]. 86 9

We reported 9 cases of primary Sjogren syndrome (SS) who were complicated with nervous system involvement. All were women. Age between 24 to 58 years old. Their clinical symptoms of the nervous system varied widely, 7 of them manifested with the involvement of central nervous system, the main features were in case 1 bouts of seizures, case 2 multi-level damage of the brain and spinal cord which was similar to multiple sclerosis, case 3 recurrent hemiplegia caused by cerebral thrombosis then fatal vascular hemorrhage, case 4 sudden hemiplegia, case 5 persistent psychological disturbance, case 6 cranial neuropathy of V and VII, case 9 multi-focal symptoms of ataxia, myelopathy and transient blindness. 5 of the 9, case 3, 6-9 all appeared with the symptoms of peripheral neuropathy, case 7 also complicated with carpal tunnel syndrome. 4 of the 9 cases also complicated with renal tubular acidosis and/or chronic active hepatitis and/or fibrosing alveolitis and/or thrombocytopenic purpura and/or myositis. No differences of the positivity of autoantibodies was observed between those with or without nervous system involvement.
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PMID:[The manifestations of the nervous system in primary Sjogren syndrome]. 158 41

Surgical treatments for intervertebral disc disease are useful for animals with gradually progressive, severely acute, or recurrent lesions. Surgical patients require a diligent neuroradiographic evaluation to confirm location and severity of the discopathy and rule out conditions unrelated to disc disease. Although controversial, some surgeons advocate fenestration for recurrent pain or mild ataxia associated with a noncompressive lesion. Decompressive procedures are recommended for treatment and prognostication of compressive disc-associated myelopathy. Foraminotomy is reserved for patients with lateralizing extrusions confirmed with myelography or newer imaging modalities (CT, MRI). Future treatments for animals may include chemonucleolysis or various discectomy procedures currently performed in humans with symptomatic disc disease.
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PMID:Surgical treatments for intervertebral disc disease. 164 23

A neurological disorder in Merino sheep, characterised clinically by progressive posterior ataxia and microscopically by Wallerian degeneration in thoracic segments of the spinal cord, is described. Animals of both sexes were affected, with the earliest onset of disease being at 5 months of age. Most affected animals died before 2 years of age. The clinical, pathological and epidemiological features suggest that this degenerative thoracic myelopathy is a previously unrecognised entity differing from other reported causes of ataxia in sheep in Australia.
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PMID:Progressive ataxia associated with degenerative thoracic myelopathy in Merino sheep. 177 33

Postmortem examination of 21 patients showed a vacuolar myelopathy resembling that associated with the acquired immunodeficiency syndrome. Underlying diseases included six cases of leukemia or lymphoma, five of carcinoma, three of systemic lupus erythematosus, two of chronic lung disease, and one each of cadaveric renal transplant, cirrhosis, diabetes, hemophagocytic syndrome, and viral encephalitis. Fourteen patients were on long-term steroid therapy and 10 of these also had immunosuppressive chemotherapy. No patient had the acquired immunodeficiency syndrome, although one received blood transfusions in 1978. Signs and symptoms consistent with myelopathy included paraparesis in seven patients, ataxia in one, and bilateral extensor plantar reflexes in one. Microscopic examination showed vacuolation in spinal cord white matter primarily located in posterior and lateral columns. Lipid-laden macrophages and axonal changes were proportional to the severity of the vacuolation, which was severe in five patients, moderate in 10, and mild in six. Eight patients had coexistent viral diseases elsewhere in the central nervous system, but viral-associated antigens or genomic material was not found in regions of vacuolated spinal cord white matter. Although the etiology of these myelopathies is unknown, their association with immune suppression and coexistent viral infection of the central nervous system suggests that an opportunistic viral infection may be important.
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PMID:Idiopathic myelopathies with white matter vacuolation in non-acquired immunodeficiency syndrome patients. 186 65

A 58-year old man presented with slowly progressive spastic paraparesis, ataxia, absent ankle jerks, bladder disturbances, impairment of vibration sense and mental deterioration. Electrophysiological studies documented axonal sensory neuropathy, posterior column and optic nerve involvement. Serum tests for anti-HTLV-1 antibodies were negative but HTLV-1 proviral sequences were consistently demonstrated in white blood cell genomic DNA using the polymerase chain reaction technique. Western blot and polymerase chain reaction assays of sera and DNA from family members were negative for HTLV-1. The most likely cause of infection in this patient was a blood transfusion received 2 years before onset of symptoms. This is the second Italian case of HTLV-1 associated myelopathy and the fourth reported in white subjects living in Europe.
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PMID:HTLV-1-associated myeloneuropathy in an Italian. 195 Apr 58

A 41-year-old man was doing well until July 1989, when he noted numbness over soles, followed 4 months later by difficulty in walking. These symptoms were progressively getting worse, and he was admitted to our department on June 12, 1990. General physical examination was unremarkable. Neurologically cranial nerves were intact except old right, traumatic strabismus. Muscle tone and deep tendon reflexes were normal throughout, but bilateral Babinski and Chaddock reflexes were present. Mild weakness of lower extremities were found on muscle testing (4/5). In sensory system, superficial sensory disturbance below T10 was seen, and markedly diminished vibration and position senses of lower extremities were noted. Cerebellar test was intact, although unsteadiness was found on heel-shin test. Romberg sign was definitely positive. His gait was wide-based and ataxic. Laboratory data showed no abnormalities in CBC, chemistry, urinalysis, serological tests and endocrinological examinations. Spinal MRI (Siemens 1.5 Tesla) showed abnormal deposition of epidural fatty tissues compressing spinal cord with flattening of cord from T4 to T8. Spinal ataxia as compressive myelopathy due to epidural lipomatosis was considered and he underwent laminectomy from T4 to T8 with improvement in walking. Epidural lipomatosis is an unusual cause of spinal cord compression, presenting compressive myelopathy, radiculopathy, cauda equina syndrome, intermittent claudication, or back pain. Most of cases were associated with long-term administration of adrenocortical steroid hormone, or underlying diseases, except only 3 cases including ours. This is the first case of spinal epidural lipomatosis presenting progressive gait disturbance due to spinal ataxia.
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PMID:[A case of epidural lipomatosis presenting spinal ataxia]. 206 Feb 46

Spinal cord lesions, although far less frequent than brain lesions, are not uncommon in AIDS. Almost all diffuse or multifocal pathological processes involving the central nervous system and/or leptomeninges may also affect the spinal cord. However, some of them involve it predominantly causing specific, clinically overt, myelopathic diseases. The most characteristic of these is vacuolar myelopathy which usually manifests as progressive spastic ataxia. Its incidence varies in different pathological series and its pathogenesis is still controversial. Acute meningomyeloradiculitis in AIDS patients presents a remarkably uniform and distinct clinical and cerebrospinal fluid profile although they might be due to various causes including cytomegalovirus infection, syphilis, mycobacterial infection or leptomeningeal malignancy. Tumoral focal syndromes have exceptionally been recorded due to epidural lymphomas, intraspinal gliomas or toxoplasma abscesses. Spinal cord ischemic lesions have been documented in two cases: they were related to inflammatory lesions of the blood vessels in one case and to diffuse intravascular coagulation in the other. An ascending myelitis syndrome, secondary to spinal cord infection by both herpes simplex virus type 2 and cytomegalovirus, without significant vasculitis has also been reported.
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PMID:[Lesions of the spinal cord and spinal roots in human immunodeficiency virus infection]. 207 14


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