Palatal myoclonus: A reappraisal

Herrmann, Christian; Brown, Jason W.

doi:10.1016/0022-510x(67)90086-x

Cited by 60 publications

(26 citation statements)

References 43 publications

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“…A candidate pathway in the case of CRH may be the inferior olivodentatorubral circuit. Injury to afferent (ventral tegmental or dentatoolivary [58]) inpur results, after a delay period, in olivary "hypertrophy" and palatal myoclonus in human adults [59]. The activation of such pathway may require a shorter delay in children [60].…”

Section: The Crh-excess Theory Of Massive Infantile Spasmsmentioning

confidence: 99%

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Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis

Baram

1993

Annals of Neurology

135

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Massive infantile spasms are an age-specific seizure syndrome of infancy. Uniquely, the spasms respond to hormonal manipulation using adrenocorticotropic hormone (ACTH) or glucocorticoids. A hypothesis explaining the efficacy of hormonal therapy, age-specificity, multiple causative factors, and spontaneous resolution of infantile spasms is presented. Corticotropin-releasing hormone (CRH), an excitant neuropeptide suppressed by ACTH/steroids, is implicated. Evidence for the age-specific convulsant properties of CRH is presented, and a putative scenario in which a stressinduced enhancement of endogenous CRH-mediated seizures is discussed. Clinical testing of the CRH-excess theory and its therapeutic implications are suggested.Massive infantile spasms (MIS) represent a seizure disorder with unique clinical and electrographic features [1][2][3][4]. It is relatively common (1:2,000-4,000 births [5,6]) and has been known to respond to adrenocorticotropic hormone (ACTH), since 1958 [7]. Long-term intellectual outcome of affected infants, however, remains poor, with 76 to 95% of survivors having moderate to severe mental retardation [1,3,6,8]. Therefore, MIS continues to attract research concerning both pathogenesis and therapy. Several large series [1,3,[9][10][11] and recent reviews [6,8,[12][13][14] have focused on clinical and electroencephalographic phenomenology of MIS, and on the therapy and outcome aspects of this entity. Here I introduce an age-specific endogenous-convulsant hypothesis for the pathophysiology of MIS. The hypothesis implicates an endogenous neuropeptide, which is known to cause seizures in infant rats, and is suppressed by ACTH and glucocorticoids (GCs). I shall present evidence for this hypothesis, discuss some of its predictions for treatment options, and place it in the context of current therapeutic controversies. Definition and DescriptionInfantile spasms were first described by West [15] The syndrome of MIS consists of a constellation of myoclonic seizures in an infant, whose EEG pattern is that of hypsarrhythmia or its variants [1-4, 7-11, 14, 17]. The electroencephalographic (EEG) pattern, response to therapy, and poor outcome distinguish MIS from a variety of other myoclonic epilepsies of infancy [1,18]. Furthermore, MIS is a time-locked entity; it arises in infancy after a delay from the time of insult and, in the majority of cases, disappears spontaneously. Even without treatment, 89% of patients are reported to be spasm free by 5 years of age [19]. Proposed PathophysiologyMIS develops in infants with a variety of central nervous system (CNS) pathologies [1][2][3][4]. Structural anomalies, tuberous sclerosis, and other phakomatoses are commonly associated with MIS. Prenatal as well as peri-and postnatal infections, stroke, trauma, and even chromosomal aberrations have all been implicated as causative factors. This multitude of associated factors has suggested that MIS may be a "final common pathway" or an agespecific yet cause-nonspecific response of the brain [1,20,21]. Cor...

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Section: The Crh-excess Theory Of Massive Infantile Spasmsmentioning

confidence: 99%

“…The activation of such pathway may require a shorter delay in children [60]. Only some candidate lesions result in hypertrophy and/or myoclonus [58]. CRH is a putative neurotransmitter in the inferior olive in the human and rodent [6l, 62].…”

Section: The Crh-excess Theory Of Massive Infantile Spasmsmentioning

confidence: 99%

Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis

Baram

1993

Annals of Neurology

135

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“…Attention has previously been focused on unilat eral capsulolenticular lesions as a cause of pathological laughter [8,9]. It has been suggested that in these cases, the descending pathways concerned with faciorespiratory synkinesis may be disinhibited by an interruption of the pallidotegmental connections, leading to excessive stimu lation of involuntary expression mechanisms [9,11], Our 3 patients have certain common clinical features which may be in favor of a 'motor' hypothesis. Firstly, laughter often occurred after a 'motor' stimulus, either speaking (in all 3 patients) or moving (in patient 3).…”

Section: Discussionmentioning

confidence: 98%

“…The way in which inappro priate laughter occurred in our patients suggests the fol lowing hypothesis: (1)A unilateral lesion affecting the supranuclear control of faciorespiratory synkinesis is suf ficient to produce inappropriate laughter provided that corticobulbar control is not totally interrupted. (2) Ac cording to a hierarchical view of the integration of facio respiratory contractions, this type of unilateral lesion may lead to deregulation of the intact synkinetic expression mechanisms [11], These lesions may cause a disturbance in the control of the coordination of synkinesis by the pyramidal and extrapyramidal pathways, especially if there is activation of the damaged pyramidal pathway. (3) The temporary appearance of these attacks of laughter could be explained by the cerebral lesions being unilater al.…”

Section: Discussionmentioning

confidence: 99%

Temporary Forced Laughter after Unilateral Strokes

et al. 1994

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Forced laughter generally occurs in bilateral or diffuse lesions of the brain and is generally associated with pathologic crying. We report the cases of 3 patients in whom temporary forced laughter occurred after unilateral supratentorial infarction demonstrated by CT scan or MRI. In all cases, the lesion was exclusively subcortical and involved the striatocapsular region. For all patients the laughter occurred during the revovery phase of motor deficit and was ‘pure’ without associated weeping or other clinical features of pseudobulbar palsy. In all cases, the laughter attacks had become much less frequent or had totally disappeared 1 or 2 months after the stroke. According to a ‘motor’ hypothesis, a unilateral striatocapsular lesion may lead to temporary deregulation of the expression mechanisms.

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“…1 A literature search showed few reports of OPT from MS. An article that reviewed 171 publications on palatal myoclonus found 9 cases of MS among 287 cases. [2][3][4][5][6] However, on review many of these cases do not meet the current clinical criteria for diagnosis of MS. Neuroimaging or pathology was not available in any. There is one report of bilateral OPT with abnormal T2 signal in the pons in a case of clinically definite MS. …”

Section: Discussionmentioning

confidence: 99%

Oculopalatal tremor in multiple sclerosis with spontaneous resolution

2012

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O culopalatal tremor (myoclonus) is a rare movement disorder that develops after brainstem or cerebellar stroke, tumor, or following radiation. We describe a patient with transient OPT from relapsing-remitting multiple sclerosis (MS) and review the imaging changes over the course of his OPT. Case reportA 16-year-old boy with mild cognitive impairment presented with vision loss in his left eye of 6 weeks duration. At age 5 years he had undergone 2 surgical resections and radiation for a posterior fossa ependymoma. Postoperatively, he developed a left temporal lobe stroke and subsequently a seizure disorder requiring lamotrigine and levetiracetam. In the last 2 years he had become seizure-free. Serial MRI showed no tumor recurrence in the last 10 years.On examination, his visual acuity was 20/25 in the right eye and counting fingers in the left eye with a left afferent pupillary defect and normal optic discs in both eyes. His right eye had superotemporal quadrantanopia from his previous stroke. He had abnormal tandem gait and mild dysmetria on finger-nose-finger testing bilaterally.MRI of the brain and orbit showed abnormal enhancement of the left prechiasmatic optic nerve consistent with optic neuritis and abnormal fluid-attenuated inversion recovery (FLAIR) signal in the left corona radiata and right medial temporal lobe with enhancing lesions consistent with a clinically isolated syndrome. Encephalomalacia of the left inferior temporal lobe from his previous infarct and postoperative changes in the posterior fossa were seen. Spine MRI and CSF examination were unremarkable. He received IV methyl prednisone for 3 days followed by a tapering dose of oral prednisone.Over the next 6 months his left eye visual acuity improved to 20/30. The left optic disc became pale. A follow-up MRI 6 months after the previous MRI showed multiple new T2 and FLAIR signal abnormalities consistent with demyelination.Seven months after the optic neuritis, he presented with a 1-week history of oscillopsia lasting for 2-3 hours on waking in the morning. On examination in the afternoon he had palatal myoclonus without nystagmus.One week later, he was examined in the morning for persistent oscillopsia and new blurred vision on gazing to either side. Extraocular movements showed bilateral internuclear ophthalmoplegia.In primary gaze, he had a slow intermittent vertical pendular nystagmus that increased in frequency and amplitude on upgaze. He continued to have palatal myoclonus. Repeat brain

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Palatal myoclonus: A reappraisal

Cited by 60 publications

References 43 publications

Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis

Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis

Temporary Forced Laughter after Unilateral Strokes

Oculopalatal tremor in multiple sclerosis with spontaneous resolution

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