painful legs and moving toes ( plmt ) is a rare syndrome characterized by spontaneous movements of a single or all digits and pain in one or both lower extremities123 ) .
the largest case series identified 76 patients during an 18-year period2 ) , and only one pediatric case has been reported4 ) .
we report the first adolescent case of plmt in korea and review current literature on the plmt syndrome .
a 16-year - old girl complained of tingling pain in her left leg and continuous involuntary movements of her ipsilateral great toe one month after a second untethering surgery .
extension and flexion of her great toe , which occurred when awake and in the early stages of sleep , could not be suppressed voluntarily . walking , sitting , or straining
did not exacerbate the pain . at birth , she had a sacral dimple at the s23 vertebral level .
four years previously , she had a voiding difficulty and underwent untethering surgery to correct the lipomeningomyelocele at the s2 level of the conus medullaris . a nerve conduction velocity study ( ncvs ) showed polyradiculopathy at the left l5 level and below with axonal involvement . a needle electromyogram ( emg ) showed abnormal spontaneous activities of the left gastrocnemius medial head , abductor hallucis , and tibialis anterior / posterior .
five months later , the voiding difficulty recurred , and a second untethering surgery was performed .
cerebrospinal fluid ( csf ) analysis revealed a red blood cell count of 60,000/mm , a white blood cell count of 150/mm ( polymorphonucleocytes 69% , lymphocytes 18% ) , a protein level of 274 mg / dl , and a glucose level of 39 mg / dl .
since the complete blood count showed neutropenia ( absolute neutrophil count=600/mm ) , teicoplanin was discontinued .
one month postoperatively , the patient presented with left leg pain and involuntary movements of her left great toe . increased deep tendon reflex of both knees was observed with no other pathologic signs .
magnetic resonance imaging of the lumbar spine showed reactive inflammatory changes in the operative scar and a decreased extent and degree of reactive meningeal enhancement compared to the previous study ( fig .
needle emg showed abnormal spontaneous activities of the left gastrocnemius medial head , abductor hallucis , and tibialis anterior / posterior with no significant interval change since the previous study .
we prescribed gabapentin ( 100 mg three times a day ) ; left leg pain and involuntary movements diminished within a day .
complete relief from involuntary toe movements was achieved within four months of gabapentin treatment in the outpatient department .
some variants of plmt syndrome include painless legs and moving toes , painful arms and moving fingers , painless arms and moving fingers , and painful mouth and moving tongue235 ) . in the largest case series ,
the mean age of onset was 58 years ( range , 2488 years)2 ) . one pediatric case ( an 11-year - old girl ) was reported in 20134 ) . in the republic of korea , there have only been a few adult cases of the plmt syndrome567 ) .
most patients ' primary complaint is pain , which is more distressing than involuntary movement23 ) .
although patients ' descriptions of their pain are diverse ( e.g. , tingling , numbness , aching , burning , etc . ) , all of them report that it is constant .
some patients ' pain was exacerbated by certain situations , including physical positions ( e.g. , sitting , walking , bearing weight , or bending ) , diurnal variation ( night or day ) , cold temperature , external pressure , and the valsalva maneuver .
conversely , in other patients , the same situations have alleviated the pain2 ) . voluntary and temporal suppression of symptoms by applying pressure to the sole of the foot has been reported3 ) . in our patient , none of the aforementioned situations diminished or intensified the symptoms of involuntary movement .
the involuntary movements tended to be constant , sometimes fluctuating in severity , and stereotypical to a particular individual2 ) .
reportedly , involuntary movements include flexion / extension , abduction / adduction , wriggling / dystonia / writhing , or irregular , random , rhythmic twitching , fanning , clawing , waving , etc.3 ) . underlying disorders of the plmt syndrome have been reported at the level of the peripheral or central nervous system ( e.g. , peripheral neuropathy , radiculopathy , spinal cord lesions , trauma , myelitis , compressive myelopathy , wilson disease , parkinson disease , and hashimoto disease)123 ) .
recently , peripheral neuropathy was considered to be the most commonly identifiable cause of pain in 21 of 76 patients ( 28% ) ; conversely , 32 ( 42% ) had no identifiable cause of pain2 ) .
clinically , the plmt syndrome 's potential origin is within the peripheral nervous system ; however , the disorder 's evolution suggests that the central nervous system is involved in the persistence of pain , which mediates movement of the digits .
this makes the condition very difficult to treat ; presumably , neuromodulation drives the movement of the digits via a generator at the spinal cord or brainstem2 ) .
recently , the findings of an ncvs were normal in 33 of 63 cases , and the remainder showed mild abnormalities , including chronic radiculopathy ( 14 cases ) , peripheral neuropathy ( 9 cases ) , cramps and fasciculations ( 4 cases ) , isolated fasciculations / fibrillations ( 5 cases ) , mononeuropathy ( 3 cases ) , and evidence of prior poliomyelitis ( 1 case ) .
an emg showed irregular ( not truly rhythmic ) phasic bursts with various durations ( range , 10 msec to 2 seconds ) , which were associated with cocontraction and digit or distal limb movements2 ) .
the bursts had broad frequencies ( range , 1.5200 hz)23 ) . in the pediatric case , ncvs and emg were unremarkable4 ) .
restless leg syndrome also has both discomfort and movements of lower limbs , but the pain of plmt is not relieved by movements .
plmt does not involve skin change or altered temperature unlike complex regional pain syndrome2 ) .
epilepsia partialis continua is usually painless and accompanied by structural brain lesions and epileptic discharges on an eeg3 ) .
other movement disorders that need to be differentiated from plmt are chorea , pseudoathetosis , spinal segmental myoclonus , psychogenic movement disorder23 ) . in a recent large case series ,
only one - third of patients responded to pharmacologic treatment for involuntary movements or pain2 ) . in the pediatric case and our adolescent case ,
the difference may originate from the onset age or the duration between onset and treatment .
however , we need more pediatric and adolescent cases to verify that . there were also some case reports that gabapentin was effective for pain and movements symptoms in adult plmt patients and the therapeutic effect started in 1 - 4 days68 ) .
there was no consensus for the duration of gabapentin therapy . in our case , we ceased gabapentin after 4 months .
the plmt syndrome is rare but it should be considered during differential diagnosis of children and adolescents with pain in the limbs and involuntary movements in the toes .
 painful legs and moving toes ( plmt ) syndrome is characterized by spontaneous movements of the digits and pain in one or both lower extremities . of the reported cases , 
 a majority of the patients was female , and the mean age of onset was 58 years . 
 only one pediatric case has been reported so far . herein , we report the first adolescent case of plmt in korea . a 16-year - old girl complained of tingling pain in the left leg and involuntary movement of the ipsilateral great toe one month after a second untethering surgery . 
 three years ago , she had undergone untethering surgery to correct lipomeningomyelocele at the s2 level of the conus medullaris . at that time 
 , she was diagnosed with polyradiculopathy at the left l5 level with axonal involvement . 
 we diagnosed her with plmt syndrome and prescribed gabapentin . 
 her symptoms diminished within a day . 
 complete relief from involuntary movement of the toe was achieved within four months . 
 plmt is a rare syndrome but it should be considered in the differential diagnosis of children and adolescents with limb pain and spontaneous movement in their toes . 
Introduction
Case report
Discussion