Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018991 (hemiplegia)
3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 70 years-old man was admitted at our hospital because of unstable angina pectoris. He had essential hypertension and right hemiplegia from a ischemic stroke two years before admission. On neurologic examination, it was found mental disorientation, unstable emotionality, right spastic hemiparesis with right Babinski sign, and segmental myoclonus affecting the superior lip and the palate (palatal nystagmus) on the right side. On the CT scan, a giant aneurysm of the basilar artery was detected. We conclude that the segmental myoclonus could be explained by ischemic lesions in the Guillain-Mollaret triangle.
...
PMID:Segmental myoclonus and basilar artery. Giant aneurysm. Case report. 130 61

Several non-epileptic disorders may cause episodic and paroxysmal symptoms that resemble epilepsy and they must be considered in the differential diagnosis. Some of these disorders are discussed in the present review: vasovagal, vasomotor and cardiac syncopes, breath holding spells. Among the sleep disorders, parasomnias, nightmares and the benign neonatal sleep myoclonus are mentioned. Migraine with aura, alternating hemiplegia and benign vertigo of childhood are probably related disorders. Benign myoclonus of early infancy, paroxysmal choreoathetoses and pseudoepileptic or hysterical seizures are further non-epileptic attack disorders to be considered in the differential diagnosis.
...
PMID:[Differential diagnosis of cerebral seizures]. 150 11

We reported a case of mitochondrial encephalomyopathy with repeated stroke-like episodes. A 33-year-old single male was admitted to our hospital because of stroke-like episodes with visual field defect, hemiplegia and convulsion repeated seven times for the past seven years. There were no abnormalities on the physical examination. He was hallucinative and perseverative and had mental deficiency. Muscle weakness and atrophy were not prominent, and generalized hyporeflexia were present without pathological reflexes. Myoclonus was not observed. Serum CK and blood gas analysis were normal (pH 7.398). Although blood levels of lactate and pyruvate were almost within normal limit, lactate was elevated by 20WATT-15 minutes exercises. On the contrary, the CSF levels of lactate and pyruvate were elevated markedly. CT of the brain revealed the presence of the low density areas in the right occipital and the left frontal lobes. Cranial 4 vessels studies were unremarkable. EEG showed the diffuse slowness with spike and wave complex. CT of the muscles were normal. A specimen obtained from the left biceps brachii muscle showed ragged-red fibers without obvious myogenic or neurogenic changes, and accumulations of abnormal mitochondria with paracrystalline inclusion bodies were observed by electron microscopy. However, mitochondrial abnormalities were not seen in the vessel walls in the biopsied muscle. Activities of complex I + III, II + III, IV in mitochondria were normal. Clinical features of this case were consistent with MELAS. However, this case showed no muscle weakness, short stature and lactic acidosis which characterize MELAS, and the onset of this case was later than those cases that were reported before.
...
PMID:[A case of mitochondrial encephalomyopathy characterized by repeated stroke-like episodes]. 250 53

A 56-year-old, hypertensive man was admitted to our hospital because of confusion. He had been well unit half an hour before admission, when he had suddenly developed left hemiplegia. Although he became deep coma soon after admission, his vital signs were preserved. CT scan revealed a large right putaminal hemorrhage and a ventricular perforation. The condition was too severe for surgical approaches and conservative therapy for brain edema was performed. On the 2nd hospital day, corneal and light reflexes were disappeared and an anisocolia appeared. On the 3rd hospital day, right papilledema appeared. Doll's head-eye movements and ciliospinal reflexes were absent. CT scan demonstrated marked brain edema and collapsed ambient cisterns. Tentorial herniation were suspected. On the 4th hospital day, respiratory arrest occurred and ventilatory assistance began. On the 11th hospital day, electroencephalograms (EEG's) showed electrocerebral silence. EEG's performed next day showed still electrocerebral silence. On the 13th hospital day, brainstem auditory evoked potentials were recorded without any responses. He was thought to be in condition of brain death. On the 17th hospital day, multifocal myoclonus involving lower limbs and abdominal muscles appeared. The myoclonus lasted for about 15 hours occurred on both sides, but was asymmetrical. The myoclonus consisted of intermittent, brief, arrhythmic, stereotype, jerking contractions of the muscles. Sometimes, the contractions were sufficient enough to jump his body over the bed. These symptoms provided the characteristics of spinal myoclonus. Etiologies of spinal myoclonus are varied, but the primary abnormality exists within the spinal cord.(ABSTRACT TRUNCATED AT 250 WORDS)
...
PMID:[Spinal myoclonus in association with brain death]. 262 31

Twenty three patients are described with a Kojewnikow syndrome in order to identify possible etiological factors. According to their history, clinical and E.E.G. features, patients could be classified into two groups; 1) Eleven patients corresponded to the classical description of the Kojewnikow syndrome: variable age at onset of the disease, rare somatomotor seizures, delay of onset of myoclonic jerks often of long duration, myoclonus limited to a small region, normal neurological examination, stable hemiplegia, normal results of psychometric tests, localised E.E.G. alterations. In most of these patients the etiology of the disorder was known, and the symptoms could be related to a localised lesion of the central cortex; 2) the second group (11 patients) presented with completely different characteristics: early onset of fits (age: 2 to 10 years), presence of other seizure types, short delay of onset of myoclonic jerks, high frequency of seizures, localization of myoclonus over large parts of the body, progressive evolution of a motor syndrome, associated neurological signs and disorders of sleep and behavior, progressive mental deterioration, characteristic E.E.G. pattern with long subclinical paroxysms of slow spikes (sharp waves) with variable localization, diffuse cerebral lesions of unknown etiology were found. Only one patient could not be allocated to either group. Several circumstantial data suggest that patients of the second group may suffer from a slow virus infection. Should this hypothesis be confirmed, a reconsideration of the Kojewnikow syndrome from the etiological standpoint would be of great theoretical and practical interest.
...
PMID:[Continuous partial epilepsy: syndrome and disease]. 682 Jan 77

Two patients with epilepsy and large hemispheric lesions underwent section of the frontal fibres of the corpus callosum for the treatment of seizures refractory to medical treatment. A severely retarded girl of 18 had encephalotrigeminal angiomatosis (Sturge-Weber syndrome) with multiple daily absences, tonic-clonic, myoclonic, atonic and adversive seizures since infancy. All types of fits--with the exception of adversive seizures and rare tonic-clonic fits--disappeared after anterior callosotomy. Another moderately retarded girl of 18 had an old cystic lesion over the entire territory of the left middle cerebral artery. She had had right hemiplegia since infancy and frequent brief absences and massive myoclonus triggered by unexpected sensory stimuli since the age of six years. Following anterior callosotomy there was an almost complete disappearance of the absences and a marked reduction of her startle myoclonus. Frontal callosotomy is a useful procedure in epileptics with large hemispheric lesions and carries less risk than hemispherectomy or total commissurotomy.
...
PMID:Anterior callosotomy as a substitute for hemispherectomy. 693 7

A 27-month-old girl presented with chest pain. Further evaluation confirmed posterior mediastinal neuroblastoma with bone marrow infiltration, which was treated with a combination of chemotherapy and surgery. Four months after completing treatment, she presented with myoclonus and weakness of her right arm. The myoclonus eventually subsided but her right arm weakness progressed to a right hemiplegia. High titers of antineuronal nuclear antibodies identified as anti-Hu were found in both serum and cerebrospinal fluid. One month later she presented with a relapse of her original tumor, from which she died. Identification of anti-Hu antineuronal nuclear antibodies in this neuroblastoma-associated paraneoplastic syndrome supports the hypothesis that the syndrome is due to autoimmune disease.
...
PMID:Neuroblastoma-associated paraneoplastic syndrome with anti-Hu antineuronal antibodies presenting at the time of recurrence. 883 43

A 60-year-old hypertensive woman had a pontine hemorrhage that caused slight right hemiplegia, deep sensory disturbance on her right side and dysarthria. Three months after the stroke, she was transferred to our hospital for rehabilitation. Approximately 6 months later, she gradually began to complain of the visual oscillation. Continual, unceasing conjugate vertical/rotatory eye movements were observed. Fixation was momentary at best because of an inability to dampen the spontaneous eye movements. Electrooculography (EOG) showed bilateral vertical/rotatory sinusoidal eye movements of 2.5 Hz frequency and 10- to 35-degree amplitude. Both vertical and horizontal optokinetic nystagmus were absent. Caloric stimulation did not evoke any responses bilaterally. There were no rhythmical movements at similar frequencies in other parts of the body such as palatal myoclonus. MRI revealed not only hematoma mainly at the dorsal pontine tegmentum but also hypertrophy of the inferior olive nucleus, suggesting disruption of the central tegmental tract. Lesions of this tract may be one cause of pendular nystagmus. Several drug therapies were investigated for the nystagmus. There was no response to baclofen 15 mg. Trihexyphenidyl 4 mg was discontinued because of drug-induced hallucinations. Tiapride 600 mg and phenobarbital 90 mg were each slightly effective in reducing both frequency and amplitude of nystagmus. Treatment with clonazepam 1 mg resulted in the striking disappearance of nystagmus. She was aware of this and no longer experienced oscillopsia. Despite the visual benefit, however, the patient did not wish to continue this drug because of drowsiness and muscle relaxation. The potential long-term therapeutic application of clonazepam should be further investigated. To our knowledge, there have been no reports of successful treatment in acquired pendular nystagmus with clonazepam. Therefore, based on this favorable experience, it is suggested that clonazepam should be added to the list of potential therapies for pendular nystagmus.
...
PMID:[Acquired pendular nystagmus after pontine hemorrhage]. 1065 2

We report a 2-year-old girl who had repeated febrile or afebrile seizures since infancy. Prolonged left/right hemiconvulsions and myoclonus of the eyelids/extremities with generalization to tonic-clonic seizures, were refractory to antiepileptic agents. At age 1 year and 4 months, she contracted rotavirus infection, and developed status epilepticus with persistent right hemiclonic seizures. Left unilateral brain edema with subsequent emergence of cortical laminar necrosis and white matter lesions, and progressive atrophy of the left cerebral hemisphere were noted during this period. She showed residual right hemiparesis and mild intellectual disability, and had generalized/eyelid myoclonia and hot water epilepsy after a 5-month seizure-free period. Analysis for SCN1A, the gene encoding the neuronal voltage-gated Na+ channel alpha1 subunit revealed a nonsense mutation, R1892X. These indicate the potential risk in patients with severe myoclonic epilepsy in infancy (SMEI) to develop hemiconvulsion-hemiplegia (HH) syndrome. SCN1A mutations may need to be further explored in patients with HH syndrome without features of SMEI.
...
PMID:Hemiconvulsion-hemiplegia syndrome in a patient with severe myoclonic epilepsy in infancy. 1956 49

It is important to accurately diagnose epileptic seizures in early life to optimise management and prognosis. Conversely, however, many different movements and behaviours may manifest in the neonatal period and infancy that may not have at their root cause a change in electrical activity of the brain. It is important to distinguish them from epileptic seizures to avoid over- and inappropriate treatment. Some are physiological in the normal infant, such as neonatal tremor, benign neonatal sleep myoclonus, and shuddering attacks, whereas others may herald alternative rare neurological diagnoses with differing prognoses such as hyperekplexia, paroxysmal extreme pain disorder and alternating hemiplegia of childhood. Here are highlighted the key clinical features that distinguish some of these disorders, their management and prognosis.
...
PMID:Differential diagnosis of epileptic seizures in infancy including the neonatal period. 2364 46


1 2 Next >>