Pisa Syndrome Research Articles

Risperidone-Induced Pisa Syndrome in MS

To report a case of risperidone-induced Pisa syndrome in a patient with multiple sclerosis (MS) that resolved with lurasidone, recurred with chlorpromazine, and was complicated by possible drug-drug interactions. A 31-year-old white male with MS developed Pisa syndrome after years of treatment with risperidone at varying doses for behavioral symptoms associated with pervasive developmental disorder. The patient experienced improvement in symptoms after treatment was switched to lurasidone; however, due to psychiatric decompensation, a switch to chlorpromazine was made and Pisa syndrome recurred. To maintain control of the patient's behavioral symptoms, chlorpromazine was not discontinued. Pisa syndrome is a rare adverse drug reaction induced most often by neuroleptic medications. The reaction is characterized by dystonia affecting cervical and lumbar musculature, resulting in flexion of the head and body to one side with axial rotation of the trunk. The etiology is believed to involve a dopaminergic-cholinergic imbalance. Most practitioners are not familiar with this syndrome, and it has not been reported previously in a patient with MS. Definitive diagnostic criteria and treatment have not been established. We identified 15 case reports involving risperidone, paliperidone, chlorpromazine, clomipramine, or valproic acid. The time to development of Pisa syndrome, patient demographics, dosing and titration of causative medications, approach to treatment, and resolution of Pisa syndrome varied widely in these reports. Dystonia in MS often presents differently than Pisa syndrome. The Naranjo probability scale indicated a probable relationship between either risperidone or chlorpromazine in each instance of Pisa syndrome in our patient. Pisa syndrome is a rare adverse drug reaction associated with neuroleptic medications. Our report highlights the importance of identifying this uncommon type of dystonia in order to consider modification of the medication regimen when appropriate.

Amiodarone-Induced Toxic Epidermal Necrolysis; Nilotinib-Associated Cerebrovascular Disease and Stroke; Pisa Syndrome Caused by Paliperidone; Macroglossia Associated with Methylprednisolone; Aromatase Inhibitor-Induced “Male Pattern Baldness” in Women? Acute Liver Failure Due to Etodolac

The purpose of this feature is to heighten awareness of specific adverse drug reactions (ADRs), discuss methods of prevention, and promote reporting of ADRs to the US Food and Drug Administration's (FDA's) MedWatch program (800-FDA-1088). If you have reported an interesting, preventable ADR to MedWatch, please consider sharing the account with our readers.

Komplikationsanalyse von spinalen Eingriffen bei adulten zentralen Bewegungsstörungen und Skoliosen

Adult central movement disorders, malpostures, and scolioses can have their cause in various neurological underlying diseases such as Morbus Parkinson, Pisa syndrome, or segmental dystonia. Important clinical characteristics are marked postural distortions such as camptocormia (bent spine) or laterocollis. In cases of these adult scolioses, surgical spine treatment puts high demands on the surgeon. Surgery in Parkinson's disease, for example, is associated with serious surgery-specific as well as general complications. The more rarely occurring Pisa syndrome is an entity primarily requiring medical therapy. A series of ten case reports of patients with Morbus Parkinson and Pisa syndrome who underwent spinal surgery is presented and discussed. From these reports, treatment recommendations have been derived and complemented by references from the literature. An extensive MEDLINE search was performed for this purpose. In patients suffering from Parkinson's disease, even minor surgical interventions can lead to instability of whole spine segments or even the entire spine. Implant loosening, adjacent segment instability, general perioperative complications, and progressive malposture due to disease progress can bring forth disastrous treatment courses. Spinal fixation should be performed long-segmented in combination with ventral stabilisation. Due to osteoporosis, pedicle screw cement augmentation is recommended in this collective. If the diagnosis of Pisa syndrome is established, an optimised preoperative preparation should be initiated in close cooperation with neurologists. In many cases medical therapy is sufficient and surgical interventions can be avoided.

Acute Pisa Syndrome After Administration of a Single Dose of Mirtazapine

Mirtazapine is an antidepressant with an adequate safety profile used in major depression. Adverse effects have been reported in the field of movement disorders, but none as truncal segmental dystonia (Pisa syndrome). We describe the case of a woman who developed Pisa syndrome after a single dose of mirtazapine.

Pisa syndrome in Parkinson’s disease: an electrophysiological and imaging study

The pathophysiology of postural abnormalities in patients with Parkinson's disease is poorly understood. In the present study, 13 patients with Pisa syndrome (PS) underwent EMG study of paraspinal lumbar (L2-L4) and thoracic (T8-T10) muscles, and of non-paraspinal muscles. Patients also underwent a whole spine X-ray and an MRI assessment of paraspinal muscles (L1-S1). The EMG evaluation disclosed two main patterns: patients with pattern I (n = 6, hyperactivity of lumbar paraspinals ipsilateral to the trunk leaning side) or pattern II (n = 7: hyperactivity of lumbar paraspinals contralateral to the trunk leaning side. In pattern I, half the patients also had ipsilateral hyperactivity of the thoracic paraspinals, the other half had contralateral thoracic hyperactivity; in pattern II, thoracic paraspinal hyperactivity was contralateral in all patients (like the lumbar paraspinal hyperactivity). Non-paraspinal muscles were hyperactive ipsilaterally in four of six patients with pattern I and in all patients with pattern II. The MRI showed mild muscular atrophy with fatty degeneration in patients with pattern I, whereas in pattern II patients this was greater and prevalent on paraspinal lumbar muscles ipsilateral to the leaning side. The present data support the hypothesis that two main patterns of muscular activation are associated with PS. In both patterns, hyperactivity of contralateral paraspinal muscles is probably compensatory for the trunk leaning.

Pisa syndrome in Parkinson's disease: a mobile or fixed deformity?

BackgroundAlthough Pisa syndrome and scoliosis are sometimes used interchangeably to describe a laterally flexed postural deviation in Parkinson's disease (PD), the imaging findings of Pisa syndrome in PD have not...

Improvement of pisa syndrome with contralateral pedunculopontine stimulation

Pedunculopontine nucleus (PPN) deep brain stimulation (DBS) has been proposed to improve gait freezing and other axial motor features in advanced Parkinson’s disease (PD) patients [1–4]. However, its effects on abnormal posture such as Pisa syndrome [5] have not been specifically described. A 62-year-old right handed woman presented with a six-year history of PD, initially manifesting as right hand resting tremor and difficulty writing. Tremor, rigidity and bradykinesia responded well to dopaminergic medications. After 5-year disease duration, she developed right sided axial lean and postural instability with falls occurring 2–3 times daily. Plain films demonstrated complex sigmoid scoliosis with mild dextroscoliosis within the thoracic spine, without osteoporosis. Trials of rotigotine, donepezil, and amantadine did not reduce fall frequency. Therefore, she was referred for PPN DBS surgery. Medications at the time of pre-surgical evaluation included carbidopa/levodopa 25/100 1.5 tabs five times daily and pramipexole 3 mg daily. Pre-operative off-medication motor UPDRS score was 18.5/108, with right upper extremity resting tremor, right greater than left rigidity, mild focal bradykinesia in the hands, slow gait with significant right tilt, and mild but recoverable retropulsion on pull test. The motor score improved to 12.5/108, 30 minutes after 350 mg levodopa challenge. She was offered left lead implantation given right greater than left PD severity. Left PPN DBS surgery was performed at the Toronto Western Hospital under local anesthesia. The surgical procedure has been previously described [2]. An implantable pulse generator (Soletra, Medtronic, Inc.) was implanted the same day under general anesthesia. She tolerated surgery well, aside from mild post-operative encephalopathy and word finding difficulty which recovered within two weeks. Programming of the left PPN stimulation started 1 month after surgery. The patient was programmed weekly using monopolar stimulation at each contact at 1.0V, at 20Hz, 50Hz, 70Hz and 130Hz over 16 weeks. The patient kept a diary of falls, which significantly decreased from 2–3 falls/daily to less than 1 fall per week. The best setting was found as contacts 3+ 2−/1.5V/90μsec/130Hz. Videos taken throughout 14 months of follow-up demonstrate progressive improvement of her asymmetric bradykinetic gait and right axial lean (Video). Lateral flexion of the trunk has been described in PD, either as scoliosis related to parkinsonism with leaning usually opposite the most affected side or as Pisa syndrome, originally described as an axial dystonia in patients treated with neuroleptics but also seen in patients with PD [5]. Prior studies of PPN DBS in PD patients have not described significant alterations in axial lean, which can contribute significantly to falls and postural instability whereas several small case series and reports describe mixed results of STN DBS on various postural abnormalities including Pisa syndrome [6]. Stimulation of the PPN and surrounding pontomesencephalic tegmental field may not only affect gait but also extensor muscle tone, thus leading to changes in posture. Chronic electrical stimulation of ventral parts of this region in freely moving cats is accompanied by an increase in tone and facilitation of walking [7]. Based on this patient’s improvement in Pisa syndrome following PPN DBS, we suggest that further studies investigating the impact of PPN DBS closely examine postural abnormalities prior to and following stimulation. The video demonstrates pre- and post-operative gait and posture examinations all taken 3 hours post-levodopa intake. The improvement in the axial lean was temporally related to the reduction of falls.

Primary and reversible Pisa syndrome in juvenile normal pressure hydrocephalus

To report a case of Pisa syndrome in a patient with idiopathic normal pressure hydrocephalus, who had never been exposed to psychotropic medications. A 26 years-old, Colombian, male patient, was referred because he had cognitive abnormalities, gait disturbances and urinary incontinence. This patient also displayed pleurothotonos. Neurofunctional evaluation of sensory and motor integration at peripheral and central nervous system levels were done. Pisa syndrome disappeared after spinal tap drainage with further gait, balance and behavioral improvement. A brainstem-thalamocortical deregulation of the central sensory and motor programming, due to the chaotic enlargement of brain ventricles was thought to be the pathophysiological mechanism underlying this case. NPH must not be longer considered as an exclusive geriatric disorder. Further, uncommon movement disorders may appear with this disorder, which should be carefully approached to avoid iatrogenic and deleterious pharmacological interventions.

Cervical dystonia in an Alzheimer’s disease patient treated with donepezil

The cholinesterase inhibitor donepezil is a safe, effective, and widely used drug for the long-term symptomatic treatment of Alzheimer’s disease (AD) [1]. Dystonia is a rare complication of donepezil treatment, and some authors have reported dystonia, including Pisa syndrome, in patients treated with this drug [2]. Here, we report an AD patient treated with donepezil who developed cervical dystonia. A woman in her late 70s was referred to our department because of progressive forgetfulness. She had impaired episodic memory for recent events and her mini-mental state examination score was 12. Eye movement was normal, and she did not have akinesia or rigidity in her extremities that would suggest parkinsonism. Brain magnetic resonance imaging showed mild brain atrophy, and 99mTc-ECD single-photon emission computed tomography showed diminished blood flow in both temporal and parietal lobes, but this was more severe in the left hemisphere. She was diagnosed with AD and was started on a 5-mg dose of donepezil. Within 1 month of beginning the donepezil regimen, she developed severe torticollis and complained of difficulty in walking and eating. Physical examination revealed no abnormality other than torticollis to the left side. Physical examination and imaging studies did not provide any evidence of Parkinson’s disease or dementia with Lewy bodies, and she did not take other dopaminergic or psychotropic drugs. Therefore, we suspected that her torticollis was a side effect of donepezil. Treatment with memantine did not affect her torticollis, and we decided to discontinue donepezil treatment. Several weeks later, she experienced relief of her torticollis. She was then treated with memantine alone and had a good outcome. Dystonia is considered a rare complication of treatment with donepezil, and previous case reports and the present findings provide evidence that this complication can be due to drugs that act on the cholinergic system. Kwak et al. [2] previously reported Pisa syndrome in an AD patient treated with donepezil, and Huvent-Grelle et al. [3] reviewed Pisa syndrome in AD patients treated with 3 cholinesterase inhibitors: donepezil, rivastigmine, and galantamine. Villarejo et al. [4] suggested that cholinergic excess may induce Pisa syndrome. Although dystonia is a rare complication of cholinesterase inhibitors, clinicians must be aware of this phenomenon because of the increasing number of dementia patients and the growing use of cholinesterase inhibitors to treat them. Because cholinergic excess induced by cholinesterase inhibitors seems to be a cause of dystonia, treatment with the noncompetitive N-methyl-D-aspartate receptor antagonist memantine [5] is an alternative for AD patients who experience side effects on cholinesterase inhibitors.

Senile anorexia: three cases with complicated treatment and two occurrences of a Pisa syndrome

Senile anorexia: three cases with complicated treatment and two occurrences of a Pisa syndrome

Management of a Risperidone-Induced Tardive Pisa Syndrome

Kaufmann, Alexandra MD; Boesch, Sylvia MD; Fleischhacker, W. Wolfgang MD; Hofer, Alex MD Author Information

Acute Pisa Syndrome After Administration of a Single Dose of Donepezil

Back to table of contents Previous article Next article LettersFull AccessAcute Pisa Syndrome After Administration of a Single Dose of DonepezilIoannidis Panagiotis, M.D., Ph.D., Maiovis Pantelis, M.D., Balamoutsos George, M.D., and Karacostas Dimitris, M.D., Ph.D.Ioannidis PanagiotisDepartment of Neurology, AHEPA University Hospital, Thessaloniki, GreeceSearch for more papers by this author, M.D., Ph.D., Maiovis PantelisDepartment of Neurology, AHEPA University Hospital, Thessaloniki, GreeceSearch for more papers by this author, M.D., Balamoutsos GeorgeDepartment of Neurology, AHEPA University Hospital, Thessaloniki, GreeceSearch for more papers by this author, M.D., and Karacostas DimitrisDepartment of Neurology, AHEPA University Hospital, Thessaloniki, GreeceSearch for more papers by this author, M.D., Ph.D.Published Online:1 Jul 2012https://doi.org/10.1176/appi.neuropsych.11070158AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Tardive dystonia syndromes are well known complications of prolonged treatment with antipsychotic medications, especially the classic ones. Pisa syndrome, a sustained dystonia of the trunk and head to one side, is considered as an atypical form of tardive dystonia and sometimes can be acute.1 Several cases have also been reported in patients taking acetylcholinesterase inhibitors (ACEIs) for at least 1 month.2 We report a patient suffering from Alzheimer’s disease (AD) who presented with acute Pisa syndrome after administration of a single donepezil dose.Case ReportA 74-year-old, right-handed woman was referred to our clinic for evaluation of memory complaints. Her past medical history was unremarkable and negative for dementia. On examination, she had brisk tendon reflexes, Babinski sign bilaterally, and primitive reflexes. Her MMSE score was 18. Neuropsychological evaluation revealed poor episodic, visual and verbal memory, as well as learning deficits compatible with AD. A brain MRI showed generalized atrophy. She was discharged with the diagnosis of AD, and donepezil was prescribed. Two days after her dismissal, her daughter called us, saying fearfully that her mother’s head and body were turned in one side. The patient had suffered acute Pisa syndrome a few hours after donepezil treatment was initiation. The drug was discontinued. On follow-up, 8 days later, her head position had turned back to normal.DiscussionPisa syndrome had classically been understood as an exclusive complication of antipsychotic treatment. During the last decade, there have been a few reports of Pisa syndrome in patients taking ACEIs, either alone or in combination with atypical antipsychotics.3 There has also been a report of three Pisa syndrome cases in a large cohort of AD patients treated with ACEIs.4 In all of these cases, discontinuation of the drug resulted in resolution of the dystonic reaction. Interestingly, the delay from the initiation of ACEIs to the appearance of Pisa syndrome varied greatly, but was at least 1 month-long.2Our patient developed acute Pisa syndrome after the first dose of donepezil, while she had not been previously prescribed with any other ACEI, antipsychotic, antiemetic, or antidepressant medication. Withdrawal of donepezil resulted in resolution of dystonia in several days’ time.Dystonic reactions after cholinergic medications are thought to represent a disbalance between cholinergic and dopaminergic receptor activity in the basal ganglia, yet the exact pathophysiological mechanism remains unknown.5 Our present case highlights that acute laterocollis and Pisa syndrome could be induced by ACEI treatment in dementia patients, who are susceptible to polypharmacy and drug interactions, even in the first days of treatment and immediate discontinuation of the drug is needed.Department of Neurology, AHEPA University Hospital, Thessaloniki, GreeceCorrespondence: Maiovis Pantelis, M.D.; e-mail: [email protected]comReferences1 Suzuki T, Matsuzaka H: Drug-induced Pisa syndrome (pleurothotonus): epidemiology and management. CNS Drugs 2002; 16:165–174Crossref, Medline, Google Scholar2 Huvent-Grelle D, Roche J, Gaxatte C, et al.: [Relation between Pisa syndrome and choline esterase inhibitors in a cohort of Alzheimer’s disease patients]. Presse Med 2009; 38:150–153Crossref, Medline, Google Scholar3 Kwak YT, Han IW, Baik J, et al.: Relation between cholinesterase inhibitor and Pisa syndrome. Lancet 2000; 355:2222Crossref, Medline, Google Scholar4 Vanacore N, Suzzareddu G, Maggini M, et al.: Pisa syndrome in a cohort of Alzheimer’s disease patients. Acta Neurol Scand 2005; 111:199–201Crossref, Medline, Google Scholar5 Villarejo A, Camacho A, García-Ramos R, et al.: Cholinergic-dopaminergic imbalance in Pisa syndrome. Clin Neuropharmacol 2003; 26:119–121Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByPisa syndrome induced by switching of a choline-esterase inhibitor treatment from donepezil to galantamine: a case report13 May 2020 | BMC Neurology, Vol. 20, No. 1Acute dystoniaRelationship Between Pisa Syndrome and Cholinesterase Inhibitor Use for Elderly Adults with Alzheimer's Disease17 December 2014 | Journal of the American Geriatrics Society, Vol. 62, No. 12Reactions Weekly, Vol. 1431, No. 1 Volume 24Issue 3 Summer 2012Pages E26-E26 Metrics PDF download History Published online 1 July 2012 Published in print 1 July 2012

A Case of Pisa Syndrome During Sertraline and Quetiapine Treatment

A Case of Pisa Syndrome During Sertraline and Quetiapine Treatment

Pisa syndrome in Parkinson's disease: Clinical, electromyographic, and radiological characterization

Abnormal postures of the trunk are a typical feature of Parkinson's disease (PD). These include Pisa syndrome (PS), a tonic lateral flexion of the trunk associated with slight rotation along the sagittal plane. In this study we describe clinical, electromyographic (EMG), and radiological features of PS in a group of 20 PD patients. All patients with trunk deviation underwent EMG and radiological (RX and CT scan) investigation. Clinical characteristics of patients with PS were compared with a control group of PD patients without trunk deviation. PD patients with PS showed a significantly higher score of disease asymmetry compared with the control group. In the majority of patients with PS, trunk bending was contralateral to the side of symptom onset. EMG showed abnormal tonic hyperactivity on the side of the deviation in the paravertebral thoracic muscles and in the abdominal oblique muscles. CT of the lumbar paraspinal muscles showed muscular atrophy more marked on the side of the deviation, with a craniocaudal gradient. PS may represent a complication of advanced PD in a subgroup of patients who show more marked asymmetry of disease and who have detectable hyperactivity of the dorsal paravertebral muscles on the less affected side. This postural abnormality deserves attention and proper early treatment to prevent comorbidities and pain.

Concomitant appearance of pisa syndrome and striatal hand in Parkinson's disease.

Pisa syndrome is (PS) usually seen in patients receiving antipsychotic drugs and characterised by lateral flexion of trunk and axial dystonia. It is believed that antipsychotic drugs lead to dopamine blockage causing PS. We describe a Parkinson’s disease patient who was doing well with levodopa/carbidopa for 3 years and developed lateral flexion of trunk. His abnormal posture used to completely improve upon lying down position. He also had striatal hand deformity suggestive of focal dystonia.

Clinical heterogeneity and underlying pathophysiological mechanisms of lateral flexion of the trunk in Parkinson’s disease

We read with interest the article by Di Matteo et al. [1] about different EMG patterns in lateral flexion of the trunk (LFT) in Parkinson’s disease (PD). A similar view would be valuable considering the clinical presentation; we would like to emphasize the clinical heterogeneity of LFT in PD, and to make some pathophysiological considerations apropos of a case. A 74-year-old woman with a 2-year history of rightpredominant PD treated with rasagiline 1 mg/day developed a slowly progressive abnormal trunk flexion unnoticed by the patient 10 months after pramipexole addition (0.18 mg tds). Neurological examination showed a mildmoderate akinetic-rigid syndrome (motor UPDRS score: 20) without any other sign suggesting atypical parkinsonism. A continuous LFT to the right with a slight forward flexion which worsened with walking and resolved with passive mobilization or when lying back was noted (Fig. 1). The patient denied any motor complication of the disease or LFT fluctuation with dopaminergic medication. Pramipexole was withdrawn and she was progressively started on levodopa/carbidopa up to 100/25 mg tds. LFT was completely resolved 4 weeks later and parkinsonian symptoms were markedly improved (motor UPDRS score: 12). Unfortunately, an EMG study of the paravertebral muscles was not performed. Although the patient was under-treated with pramipexole, the temporal relationship between LFT and dopamine agonist initiation, the lack of fluctuations of the abnormal posture and the improvement after drug withdrawal (with a minimal dose of levodopa) point to a pramipexole-induced LFT. Heterogeneity of clinical presentation and prognosis [2–6] resulted in attempts to better define LFT in PD [7, 8], distinguishing between irreversible-chronic forms characterized by rigidity and mediated by non-dopaminergic neurotransmitters, acute-subacute dystonic forms in both, off [5] and on periods [6] as a consequence of levodopa plasma level fluctuations, and drug-induced forms (DIF) associated to dopaminergic treatment changes (mainly after dopamine agonist initiation) [1–4]. The latter may resolve after prompt drug discontinuation, therefore its early diagnosis has important clinical implications. DIF are clinically similar to LFT associated to antipsychotic treatment, known as Pisa syndrome [9, 10], however the use of this term to designate them in PD patients is a matter of controversy [7, 8]. This important distinction was not made by Di Matteo et al. [1] and different types of postural abnormalities are included under the generic term LFT. The great clinical variability of LFT in PD, even in patients presenting with DIF, points to the involvement of different pathophysiological mechanisms. Close temporal relationship between postural abnormalities and dopaminergic therapy, and its reversibility after drug withdrawal [2, 4, 6], suggest a strong dopaminergic contribution. Dopaminergic asymmetrical transmission [3] or a cholinergic-dopaminergic imbalance [11] have been proposed; nevertheless, there is no clear DIF laterality and patients can deviate to the side more affected by parkinsonian symptoms [4–6] or contralaterally [1, 3, 4] suggesting the contribution of other neurotransmitter systems in certain cases, such as serotonergic and adrenergic [2, 12]. E. de Pablo-Fernandez (&) Neurology Department, King’s College Hospital, Denmark Hill, London SE5 9RS, UK e-mail: edepablofernandez@nhs.net

Reversible Pisa syndrome in patients with Parkinson's disease on rasagiline therapy

Reversible Pisa syndrome in patients with Parkinson's disease on rasagiline therapy

P6.18 Pisa Syndrome in Parkinson's disease: EMG features disclose two different underlying pathophysiological mechanisms

P6.18 Pisa Syndrome in Parkinson's disease: EMG features disclose two different underlying pathophysiological mechanisms

Pisa syndrome as presenting symptom of amyotrophic lateral sclerosis

Pisa syndrome (PS) is a truncal dystonia causing lateral flexion of the body. It was first described by Ekbom [1] as a side effect of neuroleptic drugs. It has been reported as a drug-induced syndrome secondary to neuroleptics, antidepressants, antiemetics, cholinesterase inhibitors and dopaminagonists. It was associated with some neurodegenerative diseases including Alzheimer’s dementia, multiple system atrophy, Parkinson’s disease (PD), and it was also reported as an idiopathic form [2–6]. We describe the clinical features of a patient affected by PD, who developed PS as presenting symptom of amyotrophic lateral sclerosis (ALS). A 63 year-old man had suffered from PD, starting with a 6 Hz left-hand rest tremor, since his mid-50s. PD diagnosis was made according to the UK PD Society Brain Bank criteria [7] and supported by a ‘‘typical’’ I-FP-CIT SPECT (Fig. 1). The parkinsonian symptoms had progressed slowly and the patient had received levodopa therapy with sustained benefits for almost 10 years. Because of motor fluctuations, his physician decided to switch levodopa/benserazide (LB) 600/150 mg daily to levodopa/carbidopa/entacapone (LCE) 600/150/1,200 mg daily. Two weeks later, he complained of leaning forward to the left side, which was prominent when walking or sitting, and was relieved when lying back on his left side. Based on the temporal relation with the therapeutic change, LCE-induced PS was suspected. The treatment was switched to LB (600/150 mg daily) with PS transient and incomplete improvement. Six months later he was referred to our department for a second opinion about his abnormal posture which became fixed and unrelated to his motor state. Over the past several months, he had also displayed progressive weakness and had become wheelchair dependent without any history of pain, sensory loss or trauma. On examination, wasting and fasciculations were prominent in all muscles below his knees. Strength testing showed a bilateral footdrop. Deep tendon reflexes were brisk in both lower and upper limbs with bilateral extensor plantar response. Mental status, cranial nerves function and sensory examination were normal. The marked left truncal flexion, diagnosed as PS, was persistent in both on and off state (Fig. 2) and unresponsive to any therapeutic changes. On EMG evaluation, there was evidence of widespread spontaneous activity in proximal and distal muscles of all four limbs, with positive sharp waves and fibrillation potentials more prominent in the distal lower extremities. Bulbar muscles were normal; bilateral thoracic paraspinal muscles, at T6–T8 levels, showed profuse fibrillation potentials and positive sharp waves, mainly in the left side. All the muscles sampled in the upper, lower extremities and thoracic paraspinal, showed large-amplitude, longduration, polyphasic motor unit action potentials with decreased recruitment. Fasciculations were evident in the lower limbs but not in paraspinal muscles. Brain and cervicodorsolumbar spine MRI, serological and liquoral exams were unrevealing. Clinical and EMG findings were consistent with diagnosis of ALS according to El Escorial Criteria [8]. The patient died almost 1 year after ALS diagnosis because of respiratory insufficiency. M. Deriu and D. Murgia equally contributed to the paper.

Pisa syndrome caused by an acetylcholinesterase inhibitor in a patient with dementia with Lewy bodies

Pisa syndrome caused by an acetylcholinesterase inhibitor in a patient with dementia with Lewy bodies