Biomaterial Database

Head tremor in dentatorubral-pallidoluysian atrophy. 2002

H Ohizumi, and Y Okuma, and J Fukae, and K Fujishima, and K Goto, and Y Mizuno

Department of Neurology, Juntendo University Izu-Nagaoka Hospital, Tagata, Shizuoka, Japan.

Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal-dominant neurodegenerative disorder characterized by variable combination of clinical manifestations including ataxia, myoclonus, seizures, dementia, and choreic movements. Head tremor has been rarely reported. We report a 66-year-old-woman with genetically determined DRPLA who presented with head tremor. A "no-no" type head tremor was the initial and the most prominent symptom, and mild cerebellar signs and choreic movements were also observed later. Neither hand tremor nor dystonia was noted. The patient did not show dementia, myoclonus, or seizures. Surface electromyogram (EMG) revealed 3.5-4 Hz rhythmic EMG bursts in both sternocleidomastoid muscles. DNA analysis disclosed expanded trinucleotide repeats (n = 54) in the DRPLA gene. We suggest that isolated head tremor can be a clinical manifestation of DRPLA.

UI	MeSH Term	Description	Entries
D004576	Electromyography	Recording of the changes in electric potential of muscle by means of surface or needle electrodes.	Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D005260	Female		Females
D006257	Head	The upper part of the human body, or the front or upper part of the body of an animal, typically separated from the rest of the body by a neck, and containing the brain, mouth, and sense organs.	Heads
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D014202	Tremor	Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of CEREBELLAR DISEASES, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of PARKINSON DISEASE.	Action Tremor,Intention Tremor,Resting Tremor,Coarse Tremor,Continuous Tremor,Darkness Tremor,Fine Tremor,Intermittent Tremor,Involuntary Quiver,Massive Tremor,Passive Tremor,Persistent Tremor,Pill Rolling Tremor,Rest Tremor,Saturnine Tremor,Senile Tremor,Static Tremor,Tremor, Limb,Tremor, Muscle,Tremor, Neonatal,Tremor, Nerve,Tremor, Perioral,Tremor, Semirhythmic,Action Tremors,Coarse Tremors,Continuous Tremors,Darkness Tremors,Fine Tremors,Intention Tremors,Intermittent Tremors,Involuntary Quivers,Limb Tremor,Limb Tremors,Massive Tremors,Muscle Tremor,Muscle Tremors,Neonatal Tremor,Neonatal Tremors,Nerve Tremor,Nerve Tremors,Passive Tremors,Perioral Tremor,Perioral Tremors,Persistent Tremors,Pill Rolling Tremors,Quiver, Involuntary,Rest Tremors,Resting Tremors,Saturnine Tremors,Semirhythmic Tremor,Semirhythmic Tremors,Senile Tremors,Static Tremors,Tremor, Action,Tremor, Coarse,Tremor, Continuous,Tremor, Darkness,Tremor, Fine,Tremor, Intention,Tremor, Intermittent,Tremor, Massive,Tremor, Passive,Tremor, Persistent,Tremor, Pill Rolling,Tremor, Rest,Tremor, Resting,Tremor, Saturnine,Tremor, Senile,Tremor, Static,Tremors
D020191	Myoclonic Epilepsies, Progressive	A heterogeneous group of primarily familial EPILEPSY disorders characterized by myoclonic seizures, tonic-clonic seizures, ataxia, progressive intellectual deterioration, and neuronal degeneration. These include LAFORA DISEASE; MERRF SYNDROME; NEURONAL CEROID-LIPOFUSCINOSIS; sialidosis (see MUCOLIPIDOSES), and UNVERRICHT-LUNDBORG SYNDROME.	Action Myoclonus-Renal Failure Syndrome,Biotin-Responsive Encephalopathy,Dentatorubral-Pallidoluysian Atrophy,May-White Syndrome,Ataxia, Chorea, Seizures, And Dementia,Atypical Inclusion-Body Disease,Familial Progressive Myoclonic Epilepsy,Haw River Syndrome,Myoclonic Epilepsy, Progressive,Myoclonus-Nephropathy Syndrome,Naito Oyanagi Disease,Naito-Oyanagi Disease,Progressive Myoclonic Epilepsy,Progressive Myoclonus Epilepsies,Action Myoclonus Renal Failure Syndrome,Atrophies, Dentatorubral-Pallidoluysian,Atrophy, Dentatorubral-Pallidoluysian,Atypical Inclusion Body Disease,Atypical Inclusion-Body Diseases,Biotin Responsive Encephalopathy,Biotin-Responsive Encephalopathies,Dentatorubral Pallidoluysian Atrophy,Dentatorubral-Pallidoluysian Atrophies,Encephalopathies, Biotin-Responsive,Encephalopathy, Biotin-Responsive,Epilepsies, Progressive Myoclonic,Epilepsies, Progressive Myoclonus,Epilepsy, Progressive Myoclonic,Epilepsy, Progressive Myoclonus,Haw River Syndromes,Inclusion-Body Disease, Atypical,Inclusion-Body Diseases, Atypical,May White Syndrome,Myoclonus Epilepsies, Progressive,Myoclonus Nephropathy Syndrome,Myoclonus-Nephropathy Syndromes,Naito-Oyanagi Diseases,Progressive Myoclonic Epilepsies,Progressive Myoclonus Epilepsy,River Syndromes, Haw,Syndromes, Myoclonus-Nephropathy