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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A unique association of Sturge-Weber syndrome and atlanto-occipital assimilation is presented. A 18-year-old male was admitted in emergency because of the sudden severe headache and vomiting. He had vascular nevus in the right half of the face at birth and several episodes of generalized convulsive seizures. On admission craniogram demonstrated calcification in the right occipital area. Neurological examination revealed tenderness in the nuchal region, moderate limitation of cervical mobility in a antero-posterior direction, Bruns-Cushing type nystagmus, bilaterally diminished gag reflex, and positive Romberg's test. Spinal tap showed crystal clear CSF with normal pressure. EEG showed paroxysmal slowing focus in the right parieto-occipital area. Polytomography of the craniovertebral junction demonstrated the unilateral atlanto-occipital assimilation on the left associated with the aplasia of the right posterior arch. Myelography was negative. A right carotid angiography disclosed the dilatation of the basal vein of Rosenthal and abnormal venous vasculature. CT-scan demonstrated the calcified region of the right occipital area more distinctly than the plain roentogenogram, but the enhancement study of the leptomeningeal angioma of the Sturge-Weber syndrome was negative. Never been found this rare association in a review of the literature, the authors discussed the clinical and radiological findings of both diseases.
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PMID:[Sturge-Weber syndrome associated with atlanto-occipital assimilation: a report of a case (author's transl)]. 68 66

A 40-year-old man was admitted after 8 months of speech disturbance and locomotive ataxia. He had no seizures, lightning pains, paresthesia, visual loss, bladder disturbance or rectal incontinence. He had never been neurologically or psychiatrically ill and had no history of syphilis. When the patient was admitted, his general physical examination including blood pressure and dermatologic examination was normal. His consciousness was alert. He was found to have a deterioration of mental status such as inability to concentrate, failing memory, amnesia and circumstantiality. His pupils were anisocoric and Achilles jerks were absent. No rigidity of the neck muscles, paralysis and sensory disturbance were recognized. Romberg's sign was absent. The right pupil was 7.0 mm and the left was 6.0 mm in room illumination. The pupils were nonreactive to bright light and both did not constricted to near stimuli. 0.125% pilocarpine eyedrops produced bilateral pupillary constriction. The results indicated bilateral tonic pupils. Laboratory data revealed white cell count of 12,600/mm3 and normal erythrocyte sedimentation rate of 8 mm/hr. Cerebrospinal fluid (CSF) examination revealed the following: opening pressure, 140 mm of water; cell counts, 76/mm2 (mononuclear cells); total protein, 116 mg/dl; glucose, 57 mg/dl. A serum venereal disease research laboratories (VDRL) test was positive in a 1:32 titer confirmed by positive treponema pallidum hemagglutination (TPHA) test in a 1:40,960 titer and positive fluorescent treponemal antibody-absorption (FTA-ABS) test. Serum TPHA-IgM was positive in a 1:320 titer but TPHA-IgG was negative. CSF examination revealed positive TPHA test (titer of 1:2,560) and positive FTA-ABS test.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of early syphilis presenting general paresis-like symptoms and bilateral tonic pupils]. 130 Feb 74

The clinical features of 19 patients with neurological manifestations unexplained by another disease and positive serology for Borrelia burgdorferi were studied. ECM was present in only 11% of the cases and 32% referred tick bite. The characteristic features for suspicion of NB according to our series was the presence of polyneuritis in 84% of the cases specially in the form of multiple mononeuritis and involvement of the facial nerve (79%) leading to even greater suspicion with the association of V pair involvement. Seizures, sleep disorders, and higher mental dysfunction may be found in association with other more characteristic neurological features. The typical triad of NB (aseptic meningitis, facial paralysis and polyradiculoneuritis) was found in 21% of the patients and in the absence of another disease to justify the same neuroborreliosis (NB) seemed evident. In all the cases components of this triad were found. Headache, arthralgia, fever and, less frequently, arthritis are other symptoms often past with the presence of anti-BB antibodies. Patients with the shortest evolution most frequently presented antecedents of facial paralysis, sensory alterations and Romberg's sign than patients of longer evolution. CSF demonstrated the presence of pleocytosis in 24% of the cases and in only one patient a slight increase in the intrathecal activity of IgG was observed which may be of use in differential diagnosis with MS. MR showed alterations in 61% of the patients and, while not specific, the lesions present subcortical predominance.
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PMID:[Positive anti-Borrelia antibodies in patients with clinical manifestations compatible with neuroborreliosis]. 161 Jun

We initiated a prospective study of the dizzy patient to identify key factors on which a directed evaluation could be based. This study used a standardized history, physical examination, and basic laboratory evaluation totalling 66 items to assist collection of relevant clinical information on 125 patients. Diagnosis was based on the emergency physicians' diagnosis. This was modified when necessary based on one month of follow-up using diagnostic criteria adapted from previous studies. The most common disorder was some form of peripheral vestibular disorder, found in 54 patients (43%). These patients were typically vertiginous and were managed successfully as outpatients. Despite correlations with multiple factors, this diagnosis was best predicted by positive Nylen-Barany test with either vertigo, vomiting, or both with 94% specificity and 43% sensitivity. Potentially serious causes were identified, including medication-related, seizure, stroke, transient ischemic attacks, vertebral-basilar insufficiency, hypertension, pericarditis, arrhythmias, and all those requiring hospitalization. The best predictors, either older age, lack of vertigo, or neurologic deficit, could identify 86% of "serious" dizziness with 42% specificity. The following tests were of low yield and may be done in a directed manner based on a brief history: Valsalva, carotid stimulation, Romberg and Quix tests, mental status examination, complete blood count, serum electrolytes, and BUN. Our results do support routine testing of glucose in all patients and monitoring rhythm in patients age 45 and older. Such a directed approach could rapidly classify a significant number of dizzy patients and forego many time- and cost-intensive elements of provocative examination and laboratory testing.
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PMID:A directed approach to the dizzy patient. 272 92

We report two cases of gradual facial hemiatrophy (Parry-Romberg syndrome). The first patient, an adolescent girl under treatment for hypothyroidism, presented with a very severe form combining advanced facial hemiatrophy, epilepsy with hemi-generalized seizures and hemiatrophy of the brain. The second patient was a girl who presented with localized scleroderma resembling a saber injury, homolateral cerebral atrophy and contralateral hemiparesis. Our two cases are evidence in support of a close relationship between saber injury-like scleroderma and the Parry-Romberg syndrome.
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PMID:["Saber-cut" scleroderma and Parry-Romberg facial hemiatrophy. Nosologic problems. Neurologic complications]. 293 Jan 26

The relatively high incidence of persistent post-traumatic headache and vertigo in children and adolescents presents a diagnostic and therapeutic challenge. It is often difficult to differentiate between functional complaints generated by psychological trauma or compensation-seeking and symptoms reflecting an organic etiology. The clinical and laboratory findings of 22 patients with post-traumatic headaches and vertigo were delineated into five major diagnostic categories: labyrinthine concussion, whiplash syndrome, basilar artery migraine, vertiginous seizures, and a non-specific post-traumatic dizziness. Patients with post-traumatic hearing loss were excluded from this study because they represent a group with different diagnostic problems and more recognizable organic pathology. Each patient had a complete neurologic evaluation including specific clinical vestibular tests (i.e., stepping test, reinforced Romberg, past-pointing evaluation, and positional tests using the Nylen-Hallpike maneuver. Laboratory studies included skull x-ray, computed tomography, electroencephalography, electronystagmography, and audiologic assessment. Symptoms, signs, and tests were evaluated in each category of post-traumatic vertigo to help establish the diagnosis and initiate treatment.
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PMID:Post-traumatic vertigo in children: a diagnostic approach. 350 80

The authors report a case of Parry-Romberg disease with predominantly facial left atrophy, seizures, amnesic aphasia, mental retardation, right hemiplegia and hemianesthesia, and cerebellar syndrome. The rarity of cerebellar atrophy (only one case thus far reported in the literature surveyed) is emphasized, as well as the correlation between the clinical abnormalities and the computerized axial tomography of the skull.
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PMID:[Progressive facial hemiatrophy: report of a case]. 646 49

We describe a woman with right hemifacial atrophy, a high palate, partial left motor seizures, and mild atrophy of the left arm. CT scan showed asymmetrical lateral ventricles and MRI (magnetic resonance imaging) showed atrophy of the right cerebral hemisphere and agenesis of the head of the right caudate nucleus. To our knowledge, this is the first report of Parry-Romberg syndrome associated with structural abnormalities of the basal nuclei documented by MRI. We suggest that a neurovascular aetiology can explain the spectrum of segmental defects associated with hemifacial atrophy.
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PMID:Progressive hemifacial atrophy with agenesis of the head of the caudate nucleus. 789 83

We report the unusual association of Progressive Facial Hemiatrophy (Parry-Romberg syndrome) with multiple benign tumors (orbital neurinoma, mandibular odontogenous fibroma) and hamartomas. The neurological clinical features were infantile hemiplegia, mild mental retardation and focal seizures. Brain CT-scan and MRI showed porencephaly and cerebral calcifications ipsilateral to hemifacial atrophy. Immunological investigations proved negative. The etiology of the disease and the bridging of this case to phakomatoses are discussed.
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PMID:Progressive facial hemiatrophy with multiple benign tumors and hamartomas. 871 45

The Parry-Romberg syndrome is a rare and poorly understood disease characterized by slowly progressive, localized atrophy of the skin, subcutaneous tissue, muscles, and bones. The atrophy is typically localized in the face and begins in youth. In some patients, imaging can show the lesions and atrophy of the ipsilateral hemisphere of the brain. We report on a patient in whom the disease has lasted 36 years and discuss the possibility that the Parry-Romberg syndrome is related to known autoimmune disorders of the soft tissue (e.g., linear scleroderma) and Rasmussen's syndrome. There are some remarkable clinical similarities between these two syndromes, including age of onset, unilateral manifestation, and occurrence of focal seizures. It is most probable that both diseases have an autoimmunological background.
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PMID:[Parry-Romberg syndrome and Rasmussen syndrome: only an incidental similarity?]. 1151 7


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