Camptocormia, a rare form of motor system disorders in Parkinson's disease

Camptocormia is characterized by pronounced forward flexion of the thoracolumbar spine, which increases while walking and disappears in recumbent position. The clinical spectrum of the described disorders with concomitant camptocormia is heterogenous. It was described for the first time in idiopathic Parkinson's disease in 1999. The pathophysiology of this phenomenon remains unclear but seems to be not related to antiparkinsonian treatment. The authors present the case of a 54 years old woman, with idiopathic Parkinson disease diagnosed 5 years ago. The rapid progression of the disease was associated with good response to Levodopa therapy, although the dose had to be increased up to 1400 mg/d (with peripheral decarboxylase and COMT inhibitor). After 5 years she developed painful spasms of paraspinal muscles which resulted in trunk flexion. The clinical picture resembled the described cases of camptocormia. There was no correlation between the appearance of camptocormia and the regime of levodopa administration (time or dosage). Therefore, one can conclude, that presumably camptocormia is not a form of dystonia of the trunk but, the result of till now unclear other factors (dysfunction in other non-dopaminergic nigrostriatal projections?).     

Kamptokormia – eine segmentale Dystonie Vorschlag einer neuen Definition für eine alte Krankheit

Camptocormia is defined as a forced posture with a forward-bent trunk which appears during standing and sitting. It was first described in 1818 by Brodie. In the last 100 years, numerous cases were observed. A psychogenic origin was presumed in most cases. We describe four patients with typical symptoms of camptocormia who present with the clinical and electromyographical criteria of a segmental dystonia. A new classification of camptocormia is proposed including (1) the primary form, a segmental dystonia of the abdominal wall muscles and (2) secondary forms. Among other conditions (psychogenic disorder, neurosis, myopathy, myositis, Parkinson's disease, multiple-system atrophy, thoracolumbar kyphosis, paraneoplastic syndrome), camptocormia is to be considered in essential tremor. A combination of dystonia of the abdominal wall muscles and essential tremor seems possible.     

Dopa-responsive camptocormia in a patient with multiple system atrophy

Camptocormia in parkinsonism is defined by marked anteroflexion of the trunk, which is relieved by lying down together with minimal or no response to levodopa. We describe here a 47-year-old patient with multiple system atrophy with predominant parkinsonism associated with camptocormia; the camptocormia and parkinsonian motor symptoms were markedly improved with levodopa treatment.     

Hemiparkinsonism-hemiatrophy syndrome – report on two cases and review of the literature

Hemiparkinsonism-hemiatrophy (HPHA) is a rare neurological syndrome. The main clinical features of HPHA consist of atrophy of one side of the body (face, trunk, limbs), ipsilateral hemiparkinsonism (bradykinesia, rigidity, tremor) and in many cases dystonia. There are no data on prevalence of HPHA as the condition is rare. The mean age of parkinsonism onset is earlier than in idiopathic Parkinson disease (43.7 years, range: 15–63). Changes in magnetic resonance imaging (MRI) (cortical, basal ganglia atrophy contralaterally to the side of clinical presentation) are described in 30% of patients. The pathogenesis of HPHA is unknown, but in many cases a history of prenatal injuries was reported.We present two male patients with HPHA – 45 and 55 years old, with left-sided parkinsonism, dystonia and hemiatrophy (to our knowledge, the first Polish cases). Both patients had no atrophic changes in MRI and levodopa treatment was ineffective. In the discussion the authors review current literature on HPHA.     

Lateral trunk flexion in Parkinson��s disease: EMG features disclose two different underlying pathophysiological mechanisms

Pisa Syndrome is clinically defined as the sustained lateral bending of the trunk worsened by a prolonged sitting position or by walking. Pisa syndrome, also termed lateral trunk flexion (LTF), has been rarely reported in patients affected by Parkinson's disease (PD) and, therefore, the pathophysiology has been poorly investigated. In some cases, the hyperactivity of paravertebral muscles contralateral to the leaning side has been interpreted as a sign of dystonia; however, it is well known that paravertebral muscles flex the trunk ipsilaterally. We systematically explored the pattern of muscular activation underlying the lateral flexion of trunk in 10 PD patients (mean disease duration: 9.2 ± 3.0 years) presenting LTF for 3.6 ± 2.1 years. EMG performed during stance and during left and right lateral trunk flexion showed a continuous ipsilateral muscular hyperactivity in three patients, while in the remaining ones there was no ipsilateral activity during standing and a tonic contraction of paravertebral muscles contralateral to the leaning side. In conclusion, this EMG study investigating the synergies of paravertebral muscles during dynamic conditions detected two different patterns with a typical dystonic activation in only a minority of cases. Possible pathophysiologic mechanisms and treatment approaches are discussed.     

Marked asymmetry of putaminal pathology in an MSA-P patient with Pisa syndrome.

We report on an autopsy case of a 62-year-old Japanese woman with a 2.5-year history of axial dystonia. She presented with a form of axial dystonia reminiscent of Pisa syndrome. The pathophysiological mechanism underlying forms of axial dystonia remains to be elucidated. We report here the histopathological findings of a multiple system atrophy of parkinsonian predominance (MSA-P) patient with Pisa syndrome.     

Severe forward flexion of the trunk in Parkinson's disease: focal myopathy of the paraspinal muscles mimicking camptocormia.

Pronounced forward flexion of the trunk, often termed camptocormia, is a typical symptom of patients with Parkinson's disease. In 4 parkinsonian patients with camptocormia, paraspinal muscles were studied by electromyography (EMG) and axial computerized tomography (CT) or magnetic resonance imaging (MRI) scans and muscle biopsy. EMG of the lumbar and thoracic paravertebral muscles showed abundant fibrillations, positive sharp waves, and bizarre high-frequency discharges. Spinal CT and MRI scans revealed variable degrees of atrophy and fatty replacement of the thoracolumbar paraspinal muscles on both sides. No other signs of neuromuscular disease were found. Biopsy of the paraspinal muscles revealed end-stage myopathy with autophagic vacuoles, chronic inflammatory myopathy, unspecific myopathic changes, or mitochondrial myopathy. In parkinsonian patients with pronounced forward flexion of the trunk, myopathy confined to the erector spinae muscles must be considered.     

Postural deformities in Parkinson's disease

Postural deformities are frequent and disabling complications of Parkinson's disease (PD) and atypical parkinsonism. These deformities include camptocormia, antecollis, Pisa syndrome, and scoliosis. Recognition of specific postural syndromes might have differential diagnostic value in patients presenting with parkinsonism. The evidence to date suggests that postural deformities have a multifactorial pathophysiology. Contributing factors include muscular rigidity; axial dystonia; weakness caused by myopathy; body scheme defects due to centrally impaired proprioception; and structural changes in the spine. The relative contribution of these different factors varies between patients and across specific syndromes. Improved understanding of the mechanisms underlying postural deformities in PD might ultimately lead us to more effective management strategies for these disabling and drug-refractory complications.     

Pallidal deep brain stimulation relieves camptocormia in primary dystonia

Camptocormia, characterised by a forward flexion of the thoracolumbar spine may occur in various movement disorders, mainly in Parkinson’s disease or in primary dystonia. In severe cases, patients with camptocormia are unable to walk. While treatment options are limited, deep brain stimulation (DBS) with bilateral stimulation of the subthalamic nucleus or globus pallidus internus (GPi) has been proposed as a therapeutic option in refractory cases of Parkinson’s disease. Here we present two patients with severe camptocormia as an isolated form of dystonia and as part of generalised dystonia, respectively, which were both treated with bilateral stimulation of the GPi. Symptoms of dystonia were assessed using the Burke–Fahn–Marsden dystonia rating scale (BFM) before and during deep brain stimulation. In both patients there was a significant functional improvement following long-term bilateral GPi stimulation and both patients gained ability to walk. In the first patient with an isolated dystonic camptocormia the BFM motor subscore for the truncal flexion improved by 75 %. The total BFM motor score in the second patient with a camptocormia in generalised dystonia improved by 45 %, while the BFM score for truncal flexion improved by 87 %. In both patients the effect of the bilateral GPi stimulation on camptocormia was substantial, independent of generalisation of dystonia. Therefore, GPi DBS is a possible treatment option for this rare disease.     

Trunk muscle activation pattern in parkinsonian camptocormia as revealed with surface electromyography

IntroductionCamptocormia is frequently seen in Parkinson's disease (PD) and multiple system atrophy. It is characterized by a pathological forward bending of the trunk during standing, often combined with a lateral trunk deviation. The etiology of camptocormia in PD is still unknown. Muscle MRI studies show abnormalities mainly of the erector spinae confirmed by muscle biopsies. Quantitative electromyographic examination of trunk muscle activity is missing.MethodsVentral (rectus and obliquus externus abdominis) and dorsal (iliocostalis lumborum, longissimus, multifidus) trunk muscles and the rectus femoris were recorded bilaterally with surface electromyography in standing PD patients with camptocormia (n = 10) and matched healthy controls (n = 10) who mimicked the patients' posture. EMG amplitudes were compared quantitatively. In controls, the relation between varying degrees of trunk flexion and EMG was established systematically.ResultsIncreasing forward trunk flexion was associated with increasing back muscle activity in controls, while abdominal muscle activity was negligible. During anterolateral trunk flexion, back muscle activity increased particularly on the contralateral side. The patients showed a similar pattern. However, normalized EMG activity of their trunk extensors was significantly higher than in controls, often reaching half-maximal amplitudes. Their rectus femoris and oblique abdominal muscles were overactive, but to a lesser extent.ConclusionsPD patients with camptocormia must use the functional reserve of their lumbar trunk muscles to counteract gravity. We interpret this as a weakness of the paravertebral muscles. Compared to the other examined muscles the paravertebral muscles are most affected. The increased EMG activity of the rectus femoris warrants further research.     

Camptocormia in a patient with multiple system atrophy.

The term "camptocormia" describes a severe forward-flexed posture. Although initially used to describe a conversion disorder, early authors also recognized organic camptocormia occurring in old age, or "camptocormie senile," as well as traumatic and arthritic camptocormia. More recently, camptocormia has been described in patients with Parkinson's disease and in an individual with parkinsonism. We describe a case of progressive camptocormia as part of the initial presentation of a patient with multiple system atrophy.     

Role of dopamine transporter imaging in routine clinical practice.

Functional imaging of the dopamine transporter (DAT) defines integrity of the dopaminergic system and has its main clinical application in patients with mild, incomplete, or uncertain parkinsonism. Imaging with specific single positron emission computerised tomography ligands for DAT (FP-CIT, beta-CIT, IPT, TRODAT) provides a marker for presynaptic neuronal degeneration. Striatal uptake correlates with disease severity, in particular bradykinesia and rigidity, and monitoring of progression assists in clinical trials of potential neuroprotective drugs. DAT imaging is abnormal in idiopathic Parkinson's disease, multiple system atrophy and progressive supranuclear palsy and does not distinguish between these disorders. Dopamine loss is seen even in the earliest clinical presentations of true parkinsonism; a normal scan suggests an alternative diagnosis such as essential tremor, vascular parkinsonism (unless there is focal basal ganglia infarction), drug-induced parkinsonism, or psychogenic parkinsonism. Congruence between working clinical diagnosis and DAT imaging increases over time in favour of baseline DAT imaging results. Additional applications are characterising dementia with parkinsonian features (abnormal results in dementia with Lewy bodies, normal in Alzheimer's disease); and differentiating juvenile-onset Parkinson's disease (abnormal DAT) from dopa-responsive dystonia (normal DAT).     

Parkinsonism, dystonia, and hemiatrophy.

Hemiatrophy has been reported in association with a variety of neurologic conditions, including parkinsonism. Patients with the hemiparkinson-hemiatrophy syndrome (HP-HA) have asymmetric parkinsonism with limb atrophy on the more affected side. Several authors have suggested that asymmetric brain damage early in life results in both atrophy and parkinsonism. Dopa-responsive dystonia (DRD) is a disease in which a deficiency of tetrahydrobiopterin, or, less commonly, of tyrosine hydroxylase, results in levodopa-responsive dystonia with parkinson features in children. We have recently identified four patients with DRD who had asymmetric dystonia and limb atrophy on the more affected side. Based on these patients, we suggest that a deficiency of the nigrostriatal dopamine system may, by itself, be sufficient to cause body atrophy and may underlie the limb atrophy in both DRD and HP-HA.     

A patient with multiple system atrophy presenting prolonged levodopa-responsive parkinsonism and off-dystonia of the trunk]

A 46-year-old man had a 7-year history of dopa-responsive parkinsonism. Four years after starting levodopa, he had typical motor complications such as wearing-off and peak dose as well as off-period dystonia of his trunk. Brain MRI showed marked atrophy of the brainstem and cerebellum, and the cross sign was present in the pontine base. There was neither abnormal signal intensity nor atrophy in the basal ganglia. Then, he was suspected as having multiple system atrophy (MSA). It is not easy to differentiate MSA from Parkinson diseases, particularly when the patient shows good response to levodopa and motor complications like those seen in Parkinson's disease. If the striatal pathology was not severe and nigral degeneration was prominent, presynaptic parkinsonism might occur in MSA, and putaminal preservation might account for good response to levodopa therapy. In patients with MSA, disproportionate antecollis is common before levodopa treatment, and levodopa induced off-dystonia of his trunk is very rare.     

Reversible Pisa syndrome induced by clozapine: a case report

Pisa syndrome, manifested with persistent lateral flexion of the trunk, is most commonly associated with prolonged treatment with typical antipsychotics. However, it was also reported as occurring with atypical antipsychotics. To our knowledge, there have been very few reports of clozapine-associated Pisa syndrome. Here we report 1 case of Pisa syndrome in a 39-year-old woman with schizoaffective disorder who developed tonic flexion of trunk and head toward the left side after clozapine treatment (400 mg/d) for 5 months. Clozapine was reduced to 25 mg/d within 15 days; the dystonic reaction then completely resolved within the next 3 to 4 weeks. Caution should be taken while prolonged use of clozapine in patients with risk factors of Pisa syndrome.     

Camptocormia: pathogenesis, classification, and response to therapy

Originally considered a psychogenic disorder, camptocormia, an abnormal posture with marked flexion of thoracolumbar spine that abates in the recumbent position, is becoming an increasingly recognized feature of parkinsonian and dystonic disorders. Prior reports were limited by sample size, short follow-up, and paucity of data on response to therapy. The authors reviewed 16 patients evaluated in their PD Center and Movement Disorders Clinic diagnosed with camptocormia. In addition to detailed neurologic assessment all patients were videotaped. The mean age was 64.9 +/- 17.4 years, mean age at onset of neurologic symptoms was 51.5 +/- 19.9 years, duration from onset of neurologic symptoms to development of camptocormia was 6.7 +/- 7.6 years, and the mean duration of camptocormia was 4.5 +/- 3.9 years. Of the 16 patients, 11 (68.8%) had Parkinson disease (PD); others had dystonia (n = 4) and Tourette syndrome (n = 1). Twelve patients received levodopa, with minimal or no improvement in the camptocormia. Nine patients received botulinum toxin type A injections into the rectus abdominus, with notable improvement in their camptocormia in four. One patient underwent bilateral subthalamic nucleus deep brain stimulation for PD, but there was no improvement in camptocormia. Based on this series and a thorough review of the literature of camptocormia, head drop, and bent spine syndrome, the authors propose etiologic classification of camptocormia and conclude that this heterogeneous disorder has multiple etiologies and variable response to systemic and local therapies.     

Clinical characteristics of the Pisa syndrome

The Pisa syndrome is not yet well characterized, although there have been increasing reports on its prevalence and the clinical features of drug-induced dystonia. In this report, we present 20 cases of the Pisa syndrome and discuss the clinical symptoms compared with those of classical types of drug-induced dystonia. The Pisa syndrome may occur not only as a subtype of acute dystonia but also as a subtype of tardive dystonia. Abnormal findings on brain CT were noted in both acute and tardive types of the Pisa syndrome, indicating that, like tardive dystonia, the Pisa syndrome may be associated with cerebral lesions. Young female patients were susceptible to both acute and tardive types of the Pisa syndrome, but the prevalence of both types of dystonia was inconsistent in these patients. Thus, it seems likely that very complicated pathophysiological changes in the brain are involved in the development of the Pisa syndrome.     

Clinical spectrum of Hallervorden-Spatz syndrome in India

Hallervorden-Spatz syndrome is a rare autosomal recessive disorder that involves progressive extrapyramidal manifestations. Classical and atypical clinical presentations are known. Clinical details of patients admitted to the neurology ward or attending the movement disorder clinic of the All India Institute of Medical Sciences between January 2001 and July 2007 were reviewed. Sixteen patients (9 males and 7 females) were included in the study (median age 14 years; range 6–25). The most common clinical presentation was limb or cranial onset progressive dystonia. The patients with early onset had more frequent truncal and axial dystonia, including retrocollis, oromandibular-facial dystonia and chorea, dysarthria, pyramidal signs, gait disturbance, cognitive impairment, delay in milestones, retinitis pigmentosa, optic atrophy, oculomotor abnormalities, positive family history and acanthocytosis. Although rare, cerebellar ataxia, behavioural abnormalities, parkinsonism and apraxia of eyelid opening were exclusively seen in late onset patients. The present study highlights the heterogeneity of this disease entity and also describes certain unusual clinical features.     

Camptocormia as a presentation of generalized inflammatory myopathy

Camptocormia is an abnormal truncal flexion posture that occurs while walking or standing. It is usually caused by various hypokinetic movement disorders such as Parkinson disease and multiple system atrophy. Myopathy or motor neuron disease can also be infrequent causes of camptocormia. Paraspinous muscle biopsy usually reveals focal myositis, regardless of the etiology of camptocormia. We describe the first case of generalized inflammatory myopathy with prominent camptocormia and proximal muscle weakness. Muscle biopsy of the quadriceps confirmed the diagnosis of polymyositis, and the posture showed modest improvement in response to steroid treatment. Muscle Nerve, 2009     

Parkinson's disease with camptocormia

Background

Camptocormia is defined as an abnormal flexion of the trunk that appears when standing or walking and disappears in the supine position. The origin of the disorder is unknown, but it is usually attributed either to a primary or a secondary paravertebral muscle myopathy or a motor neurone disorder. Camptocormia is also observed in a minority of patients with parkinsonism.

Objective

To characterise the clinical and electrophysiological features of camptocormia and parkinsonian symptoms in patients with Parkinson''s disease and camptocormia compared with patients with Parkinson''s disease without camptocormia.

Methods

Patients with parkinsonism and camptocormia (excluding patients with multiple system atrophy) prospectively underwent a multidisciplinary clinical (neurological, neuropsychological, psychological, rheumatological) and neurophysiological (electromyogram, ocular movement recording) examination and were compared with age‐matched patients with Parkinson''s disease without camptocormia.

Results

The camptocormia developed after 8.5 (SD 5.3) years of parkinsonism, responded poorly to levodopa treatment (20%) and displayed features consistent with axial dystonia. Patients with camptocormia were characterised by prominent levodopa‐unresponsive axial symptoms (ie, axial rigidity, gait disorder and postural instability), along with a tendency for greater error in the antisaccade paradigm.

Conclusion

We suggest that (1) the salient features of parkinsonism observed in patients with camptocormia are likely to represent a specific form of Parkinson''s disease and camptocormia is an axial dystonia and (2) both camptocormia and parkinsonism in these patients might result from additional, non‐dopaminergic neuronal dysfunction in the basal ganglia.Camptocormia is defined as an abnormal flexion of the trunk that appears when standing or walking and disappears in the supine position (fig 1​1).). This rare symptom, usually found in patients >65 years of age, is often attributed to normal ageing and is not diagnosed. The term “camptocormia” is derived from the Greek words “kamptos” (to bend) and “kormos” (trunk), and was coined by the French neurologist Souques.¹ The authors reported a soldier, who had a forced posture with a bent‐forward trunk after a gunshot wound. The patient was reportedly cured after a psychotherapeutic interview and subsequent application, in narcosis, of a plaster jacket that was removed after 2 days. Souques even suggested that the disorder could be cured completely and permanently by means of a persuasive galvanisation or faradisation of the dorsolumbar region, and termed the condition “cyphose hystérique” (“hysterical kyphosis”). Although a few authors pointed out that patients with an organic condition such as spondylitis should be differentiated from those with hysterical kyphosis, a psychogenic explanation for the disorder prevailed, owing to the many cases similar to those of Souques that were reported during the First and Second World Wars.^2,3,4,5,6Open in a separate windowFigure 1 Drawing of a patient with Parkinson''s disease and camptocormia (Bibliothèque Charcot, Hôpital de la Salpêtrière; left) and photographs of one of the patients studied (camptocormia appears when the patient is standing or walking and disappears in the supine position; right). Patient consent was obtained for publication of this figure.In 1995, Laroche et al⁷ reported 37 patients with camptocormia who were compared with 15 age‐matched patients without camptocormia but with posterior interapophysial osteoarthritis and elderly patients surgically treated for narrowing of the lumbar canal. In patients with camptocormia, magnetic resonance imaging (MRI) showed features consistent with circumscribed myopathy in the paravertebral muscles, and it was stated that camptocormia might be ascribed to a primary paravertebral myopathy.⁸ The spectrum of these neuromuscular disorders was subsequently extended to myasthenia gravis, nemaline myopathy,^9,10 amyotrophic lateral sclerosis,¹¹ inclusion body myositis, polymyositis^9,12 and miscellaneous causes such as paraneoplastic disorder¹³ and valproate toxicity¹⁴ (see also review by Azher and Jankovic¹⁵). None of the above studies, however, mentioned the presence of parkinsonism. Indeed, in the few studies that have dealt with the issue of camptocormia and parkinsonism, the coexistence of these disorders was thought to result from the incidental occurrence of Parkinson''s disease and neuromuscular disorders,^9,15,16 features of multiple systemic atrophy (MSA),^9,15 or a rare type of dystonia peripherally induced¹⁵ or of unknown origin.^9,15,17 Although some studies were the first to draw attention to the disorder,¹⁷ the nosological position and the origin of this unusual symptom in association with parkinsonism remain unclear, as do the precise clinical features of this entity.The aim of the present prospective study was to characterise a selected sample of patients with parkinsonism and camptocormia and to compare them with age‐matched patients with Parkinson''s disease without camptocormia.