Cases reported "Myoclonus"

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1/13. Carotid brainstem reflex myoclonus after hypoxic brain damage.

    A patient comatose after acute anoxia developed bilaterally synchronous, periodic myoclonic jerks most prominently in the bilateral upper limbs. Although the myoclonus seemed to occur spontaneously, electrophysiological studies showed that the myoclonic jerks correlated in timing and size with arterial pulses, and was suppressed by massage over the carotid sinus. It is proposed that the present myoclonus is a variant of brainstem reflex myoclonus in which arterial pulses served as intrinsic trigger stimuli via the carotid sinus and the medullary reticular formation.
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2/13. tremor and myoclonus heralding Hashimoto's encephalopathy.

    We report the clinical laboratory, electroencephalography (EEG), magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT) findings in a 15 year-old euthyroid girl with autoimmune thyroiditis and encephalopathy. She had stupor, coma and generalized tonic clonic seizure preceded by tremor and myoclonus with a previous misdiagnosis of epilepsy and encephalitis. Response to steroid after the 3rd relapse was excellent. Another four children in the literature are also discussed.
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3/13. A case of area-specific stimulus-sensitive postanoxic myoclonus.

    The authors report a case of area-specific stimulus-sensitive postanoxic myoclonus and discuss possible pathophysiology. A 71-year-old man sustained cardiorespiratory arrest that lasted 10 minutes and remained unresponsive. On the first EEG obtained 8 hours after the arrest there was no cerebral electrical activity before stimulation of the trigeminal-innervated areas. Periorbital stimulation was associated with bursts of spike-wave activity and generalized myoclonic jerks, whereas other types of stimulation did not elicit any response. A second EEG obtained 32 hours later showed a nonreactive alpha coma pattern. The patient died 7 days after the arrest. Area-specific stimulus-sensitive postanoxic myoclonus is very rare. The regularity of generalized bursts of spike-wave activity (cortical response) in response to stimulation of trigeminal-innervated areas suggests that the resting EEG electrocerebral silence may have been a result of cortical suppression with disinhibition of stimulus-sensitive brainstem-generated myoclonus.
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4/13. subacute sclerosing panencephalitis presenting with unilateral periodic myoclonic jerks.

    BACKGROUND: subacute sclerosing panencephalitis (SSPE) is a rare complication of measles virus infection. The disease is characterized by behavioural abnormalities, intellectual deterioration, motor weakness, and generalized myoclonic jerks progressing to coma and death in one to two years in 80% of the cases. The myoclonic jerks are associated with characteristic generalized slow periodic complexes on electroencephalography (EEG). The symptoms and signs of SSPE are frequently quite variable. The clinical course is equally variable and difficult to predict. The characteristic periodic myoclonus can rarely occur unilaterally particularly in the early stages of the disease. As well, the periodic EEG complexes have been reported unilaterally in up to 3% of cases. CASE REPORT: A 12-year-old boy, who was seen at a later stage with atypical manifestation of myoclonic body jerks confined entirely unilaterally, combined with contralateral periodic EEG complexes. One could assume clinically that the more diseased hemisphere was responsible for generating the jerks. However, brain magnetic resonance imaging revealed asymmetric hemispheric changes suggesting that the less neurologically damaged hemisphere is responsible for generating the unilateral myoclonic jerks. This has led to the interpretation that the more severely damaged hemisphere has lost the neuronal connectivity required to generate these periodic myoclonic jerks. CONCLUSIONS: subacute sclerosing panencephalitis may have asymmetric hemispheric involvement, not only early, but also in the advanced stages of the disease, which can result in unilateral periodic myoclonic jerks.
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keywords = coma
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5/13. Neurophysiological and anatomical correlations in neonatal nonketotic hyperglycinemia.

    Electroencephalographic (EEG) and brainstem auditory evoked response (BAER) findings have not been previously described and correlated with the pathological findings in an autopsied case of neonatal nonketotic hyperglycinemia (NKH). A 38 week gestation male infant presented within two hours of age with stimulus-evoked myoclonus and seizures in the context of progressive coma. Electrographic studies demonstrated cortical myoclonus and electrical seizures exquisitely localized to the midline region as well as a suppression-burst background disturbance. These vertex spike discharges were elicited after tactile stimulation. Prolonged intra-axial latencies for waves III and V were recorded on the BAER on the second day of life. Spongy leukodystrophy was noted on gross and microscopic examination of the brain involving all myelinated tracts especially in the reticular activating system, cerebellar peduncles and optic tracts. Neuropathological confirmation of brainstem involvement emphasizes the role of the nonspecific diffuse somatosensory projection system in the generation of myoclonus and stimulus-evoked seizures in the comatose patient with NKH.
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keywords = coma
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6/13. bismuth encephalopathy. A clinical and anatomo-pathological report of one case.

    A fatal case of toxic encephalopathy due to ingestion of bismuth salts is reported in a twenty year old female patient. The clinical features were characterized by a confusional state, ataxia, myoclonic jerks, and epileptic seizures. Despite supportive therapy and administration of chelating agents, there was an irreversible evolution towards coma. The immediate cause of death was an intercurrent septicaemia. The anatomo-pathological study showed non specific anoxic lesions including a widespread loss of purkinje cells in the cerebellum. Despite a drastic decrease of bismuth levels in blood, the concentration of bismuth in visceral organs and different parts of the central nervous system remained very high.
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keywords = coma
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7/13. Burst-suppression pattern with unusual clinical correlates.

    A 44-year-old man suffered a severe anoxic encephalopathy with rapid fatal outcome. His EEG prior to expiration showed a burst-suppression pattern (BSP) with occurrence of two previously unreported clinical features: spontaneous chewing movements during the burst and tonic posturing during the suppression phase. In coma the appearance of BSP in the EEG commonly implies a severe anoxic/metabolic insult to the brain with a grave prognosis. The pattern is considered to be interictal, except in cases presenting with myoclonic status epilepticus. Subtle intermittent movements of the eye, mouth and cardiovascular rhythm are uncommon and it is unclear whether these are epileptic events or not. This case indicates that the suppression phase can also have associated clinical signs.
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keywords = coma
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8/13. Severe myoclonus in a patient recovering from falciparum malaria.

    Isolated myoclonus has rarely been reported as a complication of plasmodium falciparum malaria. We describe the development of chaotic myoclonic jerks in an afebrile and conscious patient, the fourth day of treatment with quinine for P. falciparum infection. The myoclonus finally resulted in a generalized tonic-clonic seizure and coma, which resolved without further antimalarial treatment.
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keywords = coma
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9/13. Selective stimulus-sensitive myoclonus in acute cerebral anoxia. A case report.

    We report a 69-year-old patient, a chronic diabetic, who had a cardiac arrest after myocardial infarction. In the state of acute anoxic coma, massive myoclonic jerks occurred, and it was found that myoclonus could be elicited by light touch of the right trigeminal area (but of no other body region). The myoclonus was associated with complex EEG discharges against an almost flat background of activity. Findings of the autopsy showed cerebral edema with bilateral uncal herniation and prominence of the right cerebellar tonsil. Stimulus-sensitive myoclonus based on proprioceptive stimuli has been reported in chronic postanoxic states. This type of somatosensory elicitation seems to be extremely rare. We discuss the neurophysiological substratum; it is presumed that the cortex was still reached by the somatosensory stimuli.
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keywords = coma
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10/13. Opsoclonus and palatal myoclonus during prolonged post-traumatic coma. A clinico-pathologic study.

    A case of opsoclonus and palatal myoclonus following blunt head injury is described. The syndrome appeared 1 month after the injury and lasted unchanged until death. Postmortem examination showed the presence of widespread lesions of the brain stem affecting the dentato-rubro-olivary system. The main clinical features of the syndrome and their anatomical correlates are discussed.
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ranking = 4
keywords = coma
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