Hyperexcitability restricted to the lower limb motor system in a patient with stiff-leg syndrome. 2011

Tomonori Iwata, and Hiroshi Shigeto, and Katsuya Ogata, and Ko-ichi Hagiwara, and Yuji Kanamori, and Taira Uehara, and Yasumasa Ohyagi, and Shozo Tobimatsu, and Jun-ichi Kira
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Maidashi 3-1-1, Fukuoka 812-8582, Japan.

We report a 29-year-old man who presented with a 2-year history of progressive stiffness and painful spasms limited to the bilateral lower limbs, exaggerated by auditory and tactile stimuli. His deep tendon reflexes were slightly increased in both lower extremities. His plantar response was flexor. His serum and cerebrospinal fluid were negative for anti-glutamic acid decarboxylase antibodies. Electromyography of antagonist muscle pairs in his distal lower limbs revealed a failure of reciprocal inhibition. We used transcranial magnetic stimulation with a paired-pulse paradigm, delivered to the cortical area of the upper and lower limbs, and revealed significantly enhanced facilitation only in the area of his lower limbs, but not that representing his upper limbs. His symptoms were improved substantially by 20mg/day of oral diazepam. To our knowledge this is the first report of a patient with hyperexcitability limited to the lower limb motor system in a patient with stiff-leg syndrome.

UI MeSH Term Description Entries
D008297 Male Males
D009044 Motor Cortex Area of the FRONTAL LOBE concerned with primary motor control located in the dorsal PRECENTRAL GYRUS immediately anterior to the central sulcus. It is comprised of three areas: the primary motor cortex located on the anterior paracentral lobule on the medial surface of the brain; the premotor cortex located anterior to the primary motor cortex; and the supplementary motor area located on the midline surface of the hemisphere anterior to the primary motor cortex. Brodmann Area 4,Brodmann Area 6,Brodmann's Area 4,Brodmann's Area 6,Premotor Cortex and Supplementary Motor Cortex,Premotor and Supplementary Motor Cortices,Anterior Central Gyrus,Gyrus Precentralis,Motor Area,Motor Strip,Precentral Gyrus,Precentral Motor Area,Precentral Motor Cortex,Premotor Area,Premotor Cortex,Primary Motor Area,Primary Motor Cortex,Secondary Motor Areas,Secondary Motor Cortex,Somatic Motor Areas,Somatomotor Areas,Supplementary Motor Area,Area 4, Brodmann,Area 4, Brodmann's,Area 6, Brodmann,Area 6, Brodmann's,Area, Motor,Area, Precentral Motor,Area, Premotor,Area, Primary Motor,Area, Secondary Motor,Area, Somatic Motor,Area, Somatomotor,Area, Supplementary Motor,Brodmann's Area 6s,Brodmanns Area 4,Brodmanns Area 6,Central Gyrus, Anterior,Cortex, Motor,Cortex, Precentral Motor,Cortex, Premotor,Cortex, Primary Motor,Cortex, Secondary Motor,Cortices, Secondary Motor,Gyrus, Anterior Central,Gyrus, Precentral,Motor Area, Precentral,Motor Area, Primary,Motor Area, Secondary,Motor Area, Somatic,Motor Areas,Motor Cortex, Precentral,Motor Cortex, Primary,Motor Cortex, Secondary,Motor Strips,Precentral Motor Areas,Precentral Motor Cortices,Premotor Areas,Primary Motor Areas,Primary Motor Cortices,Secondary Motor Area,Secondary Motor Cortices,Somatic Motor Area,Somatomotor Area,Supplementary Motor Areas
D009046 Motor Neurons Neurons which activate MUSCLE CELLS. Neurons, Motor,Alpha Motorneurons,Motoneurons,Motor Neurons, Alpha,Neurons, Alpha Motor,Alpha Motor Neuron,Alpha Motor Neurons,Alpha Motorneuron,Motoneuron,Motor Neuron,Motor Neuron, Alpha,Motorneuron, Alpha,Motorneurons, Alpha,Neuron, Alpha Motor,Neuron, Motor
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
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000200 Action Potentials Abrupt changes in the membrane potential that sweep along the CELL MEMBRANE of excitable cells in response to excitation stimuli. Spike Potentials,Nerve Impulses,Action Potential,Impulse, Nerve,Impulses, Nerve,Nerve Impulse,Potential, Action,Potential, Spike,Potentials, Action,Potentials, Spike,Spike Potential
D016750 Stiff-Person Syndrome A condition characterized by persistent spasms (SPASM) involving multiple muscles, primarily in the lower limbs and trunk. The illness tends to occur in the fourth to sixth decade of life, presenting with intermittent spasms that become continuous. Minor sensory stimuli, such as noise and light touch, precipitate severe spasms. Spasms do not occur during sleep and only rarely involve cranial muscles. Respiration may become impaired in advanced cases. (Adams et al., Principles of Neurology, 6th ed, p1492; Neurology 1998 Jul;51(1):85-93) Moersch-Woltmann Syndrome,Stiff-Man Syndrome,Congenital Stiff-Man Syndrome,Congenital Stiff-Person Syndrome,Familial Hyperekplexia,Hereditary Hyperekplexia,Startle Syndrome,Stiff-Baby Syndrome,Stiff-Trunk Syndrome,Stiffman Syndrome,Congenital Stiff Man Syndrome,Congenital Stiff-Man Syndromes,Congenital Stiff-Person Syndromes,Familial Hyperekplexias,Hereditary Hyperekplexias,Hyperekplexia, Familial,Hyperekplexia, Hereditary,Hyperekplexias, Familial,Hyperekplexias, Hereditary,Moersch Woltmann Syndrome,Startle Syndromes,Stiff Man Syndrome,Stiff Person Syndrome,Stiff Trunk Syndrome,Stiff-Baby Syndromes,Stiff-Man Syndrome, Congenital,Stiff-Man Syndromes, Congenital,Stiff-Person Syndrome, Congenital,Stiff-Person Syndromes, Congenital,Stiff-Trunk Syndromes,Syndrome, Congenital Stiff-Man,Syndrome, Congenital Stiff-Person,Syndrome, Moersch-Woltmann,Syndrome, Startle,Syndrome, Stiff-Baby,Syndrome, Stiff-Man,Syndrome, Stiff-Person,Syndrome, Stiff-Trunk,Syndrome, Stiffman,Syndromes, Congenital Stiff-Man,Syndromes, Congenital Stiff-Person,Syndromes, Startle,Syndromes, Stiff-Baby,Syndromes, Stiff-Trunk
D050781 Transcranial Magnetic Stimulation A technique that involves the use of electrical coils on the head to generate a brief magnetic field which reaches the CEREBRAL CORTEX. It is coupled with ELECTROMYOGRAPHY response detection to assess cortical excitability by the threshold required to induce MOTOR EVOKED POTENTIALS. This method is also used for BRAIN MAPPING, to study NEUROPHYSIOLOGY, and as a substitute for ELECTROCONVULSIVE THERAPY for treating DEPRESSION. Induction of SEIZURES limits its clinical usage. Transcranial Magnetic Stimulation, Paired Pulse,Transcranial Magnetic Stimulation, Repetitive,Transcranial Magnetic Stimulation, Single Pulse,Magnetic Stimulation, Transcranial,Magnetic Stimulations, Transcranial,Stimulation, Transcranial Magnetic,Stimulations, Transcranial Magnetic,Transcranial Magnetic Stimulations
D018450 Disease Progression The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis. Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease
D018482 Muscle, Skeletal A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles. Anterior Tibial Muscle,Gastrocnemius Muscle,Muscle, Voluntary,Plantaris Muscle,Skeletal Muscle,Soleus Muscle,Muscle, Anterior Tibial,Muscle, Gastrocnemius,Muscle, Plantaris,Muscle, Soleus,Muscles, Skeletal,Muscles, Voluntary,Skeletal Muscles,Tibial Muscle, Anterior,Voluntary Muscle,Voluntary Muscles

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