Camptocormia Research Articles

Deep brain stimulation for camptocormia in dystonia and Parkinson’s disease

Camptocormia, or "bent spine syndrome", may occur in various movement disorders such as primary dystonia or idiopathic Parkinson's disease (PD). Although deep brain stimulation (DBS) is an established treatment in refractory primary dystonia and advanced PD, few data are available on the effect of DBS on camptocormia comparing these two conditions. Seven patients (4 with dystonia, 3 with PD; mean age 60.3years at surgery, range 39-73years) with camptocormia were included in the study. Five patients underwent bilateral GPi DBS and two patients underwent bilateral STN DBS guided by CT-stereotactic surgery and microelectrode recording. Pre- and postoperative motor assessment included the BFM in the dystonia patients and the UPDRS in the PD patients. Severity of camptocormia was assessed by the BFM subscore for the trunk at the last available follow-up at a mean of 17.3months (range 9-36months). There were no surgical complications. In the four patients with dystonia there was a mean improvement of 53% in the BFM motor score (range 41-79%) and of 63% (range 50-67%) in the BFM subscore for the trunk at the last available follow-up (mean 14.3months, range 9-18months). In the three patients with camptocormia in PD who underwent bilateral STN DBS (2 patients) or pallidal DBS (1 patient), the PD symptoms improved markedly (mean improvement in the UPDRS motor subscore stimulation on/medication off 55%, range 49-61%), but there was no or only mild improvement of camptocormia in the two patients who underwent STN DBS, and only moderate improvement in the patient with GPi DBS at the last available follow-up (mean 21months, range 12-36months). GPi DBS is an effective treatment for camptocormia in dystonia. The response of camptocormia to chronic STN or GPi DBS in PD is more heterogenous. The latter may be due to a variety of causes and needs further clarification.

Facioscapulohumeral muscular dystrophy presenting with isolated axial myopathy and bent spine syndrome

Several subtypes of facioscapulohumeral muscular dystrophy (FSHD) with atypical clinical presentation have been described. We report a new, distinct phenotype with progressive bent spine syndrome solely affecting the paraspinal muscles. Magnetic resonance imaging study of the lumbar spine revealed marked atrophy of the paraspinal muscles. The diagnosis was confirmed by DNA testing, which revealed shortened restriction fragments of the D4Z4 repeat on haplotype A in connection with a positive family history.

Acute camptocormia induced by olanzapine: a case report

IntroductionCamptocormia refers to an abnormal posture with flexion of the thoraco-lumbar spine which increases during walking and resolves in supine position. This symptom is an increasingly recognized feature of parkinsonian and dystonic disorders, but may also be caused by neuromuscular diseases. There is recent evidence that both central and peripheral mechanisms may be involved in the pathogenesis of camptocormia. We report a case of acute onset of camptocormia, a rare side effect induced by olanzapine, a second-generation atypical anti-psychotic drug with fewer extra-pyramidal side-effects, increasingly used as first line therapy for schizophrenia, delusional disorders and bipolar disorder.Case presentationA 73-year-old Caucasian woman with no history of neuromuscular disorder, treated for chronic delusional disorder for the last ten years, received two injections of long-acting haloperidol. She was then referred for fatigue. Physical examination showed a frank parkinsonism without other abnormalities. Routine laboratory tests showed normal results, notably concerning creatine kinase level. Fatigue was attributed to haloperidol which was substituted for olanzapine. Our patient left the hospital after five days without complaint. She was admitted again three days later with acute back pain. Examination showed camptocormia and tenderness in paraspinal muscles. Creatine kinase level was elevated (2986 UI/L). Magnetic resonance imaging showed necrosis and edema in paraspinal muscles. Olanzapine was discontinued. Pain resolved quickly and muscle enzymes were normalized within ten days. Risperidone was later introduced without significant side-effect. The camptocormic posture had disappeared when the patient was seen as an out-patient one year later.ConclusionsCamptocormia is a heterogeneous syndrome of various causes. We believe that our case illustrates the need to search for paraspinal muscle damage, including drug-induced rhabdomyolysis, in patients presenting with acute-onset bent spine syndrome. Although rare, the occurrence of camptocormia induced by olanzapine must be considered.

Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach

Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD-associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13-14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC.

Camptocormia: the bent spine syndrome, an update

Camptocormia, also referred to as bent spine syndrome (BSS) is defined as an abnormal flexion of the trunk, appearing in standing position, increasing during walking and abating in supine position. BSS was initially considered, especially in wartime, as a psychogenic disorder. It is now recognized that in addition to psychiatric syndromes, many cases of reducible BSS have a somatic origin related to a number of musculo-skeletal or neurological disorders. The majority of BSS of muscular origin is related to a primary idiopathic axial myopathy of late onset, appearing progressively in elderly patients. Diagnosis of axial myopathy first described by Laroche et al. is based upon CT/MRI examination demonstrating massive fatty infiltration of paravertebral muscles. The non-specific histological aspect includes an extensive endomysial fibrosis and fat tissue with irregular degenerated fibers. Weakness of the paravertebral muscles can be secondary to a wide variety of diseases generating diffuse pathologic changes in the muscular tissue. BSS can be the predominant and sometimes revealing symptom of a more generalized muscular disorder. Causes of secondary BSS are numerous. They must be carefully assessed and ruled out before considering the diagnosis of primary axial myopathy. The principal etiologies include on the one hand inflammatory myopathies, muscular dystrophies of late onset, myotonic myopathies, endocrine and metabolic myopathies, and on the other hand neurological disorders, principally Parkinson's disease. Camptocormia in Parkinsonism is caused by axial dystonia, which is the hallmark of Parkinson's disease. There is no specific pharmacologic treatment for primary axial myopathy. General activity, walking with a cane, physiotherapy, and exercises should be encouraged. Treatment of secondary forms of BSS is dependent upon the variety of the disorder generating the muscular pathology. Pharmacologic and general management of camptocormia in Parkinson's disease merge with that of Parkinsonism. Levodopa treatment, usually active on tumor rigidity and akinesia, has poor or negative effect on BSS.

New Details on Camptocormia in Parkinson Disease

Camptocormia is an abnormal truncal flexion associated with Parkinson disease (PD) and other disorders. This abnormal movement is present when the

D.P.1.13 Isolated paraspinal myopathy with bent spine syndrome: A distinct subtype of adult facioscapulohumeral muscular dystrophy

Objective: Characterizing adult patients presenting paraspinal atrophy and weakness. Patients/methods: Several clinical subtypes of facioscapulohumeral muscular dystrophy (FSHD) have been characterized. We present four cases (two men, two women) with genetically confirmed FSHD with a distinct phenotype solely affecting the paraspinal muscles. Three of these patients complained of lower back pain. On physical examination there was a marked atrophy of the paraspinal muscles, one patient showed slight weakness of hip abduction, another patient revealed slight weakness of the dorsal extensors of the foot.

Camptocormia and dropped head syndrome as a clinic picture of myotonic myopathy

Dropped head syndrome is primarily based on weakness localized at neck extensors. It may result from motor neuron disease, myasthenia gravis, and chronic inflammatory demyelinating polyneuropathy and also from various neuromuscular diseases including inflammatory, dystrophic and metabolic myopathies. Camptocormia (CC) on the other hand is an unusual condition characterized by progressive weakness of the extensor vertebral muscles and results in involuntary trunk flexion. CC may emerge as a clinical feature of many different conditions such as several myopathies and Parkinson's disease. The association of dropped head syndrome with CC has been rarely published in the literature. However, this is the only case presenting with concomitant dropped head syndrome and CC as a clinical picture of myotonic dystrophy (MD). In this report we aimed to represent a female patient, who was diagnosed as having myotonic dystrophy, with concurrent dropped head syndrome and CC.

An orthosis and physiotherapy programme for camptocormia: A prospective case study

Camptocormia, or bent spine syndrome, is an acquired postural disease leading to lumbar kyphosis observed when the patient stands up. Classic orthoses and physiotherapy programmes provide little correction and are often poorly tolerated. The aim of this study was to evaluate the effectiveness and tolerance of a new orthosis combined with physiotherapy for treating camptocormia. Fifteen patients (5 men and 10 women, 71.4 (standard deviation (SD) 7.3) years old) consulting for camptocormia were consecutively included in the study. Patients who had pain when they straightened up were excluded. Patients equipped with the orthosis were hospitalized for 5 days in order to learn a self-rehabilitation programme. They were evaluated before and after hospitalization, then at 30 and 90 days. In comparison with day 0 (without orthosis), the mean increase in lumbar lordosis with the orthosis was 10.1 degrees (SD 9.9) at day 30 (p < 0.05) and 12.5 degrees (SD 9.7) at day 90 (p < 0.001). Average pain values showed a reduction of 69% (SD 36) and 70% (SD 35) in the initial pain at days 30 and 90, respectively. The average increase in quality of life was 87% and 92% at days 30 and 90, respectively. This new orthosis, relying on the principle of thoraco-pelvic anterior distraction, gives excellent results in the treatment of camptocormia.

Dropped-head syndrome due to steroid responsive focal myositis: A case report and review of the literature

Isolated severe weakness of the paraspinal musculature, either at the cervical level leading to a "dropped head syndrome" or at the thoracic level leading to a "bent-spine syndrome", is a rare disorder. Etiologically it may be present in a variety of neurological diseases including Parkinson's disease, multiple system atrophy, neuromuscular or motor neuron diseases, as well as non-inflammatory, inflammatory, dystrophic or metabolic myopathies. We present a previously healthy 74-year-old man with a 2-month history of progressive difficulty in lifting his chin off his chest. Magnetic resonance imaging and skeletal muscle biopsy revealed an isolated myositis of the neck extensor and trapezius muscles, which responded well to steroid therapy. This case and other rare reports obtained from a systematic review of the literature indicate that in a subgroup of patients "dropped head syndrome" or "bent spine syndrome" is caused by a myositis, which emphasizes the necessity to obtain a MRI examination, as well as a muscle biopsy to diagnose this potentially treatable disease.

Camptocormia with Parkinson Disease

Camptocormia is a forward bending of the thoracolumbar spine that appears during standing and walking but abates on lying down. Whether its genesis is

Myasthenia Gravis with Concomitant Severe Paraspinal Muscle Degeneration and Mitochondrial DNA4977 Deletion

Recent reports have discussed the many causes of dropped head syndrome and bent spine syndrome. We described a case of myasthenia gravis with concomitant severe degeneration of spinal muscle, mitochondrial DNA4977 deletion and sensorineural deafness. These associations were thought to be independent, however this is an important case to consider the etiology of bent spine syndrome.

Sporadic, Adult-Onset Nemaline Myopathy Presenting as Camptocormia (Bent-Spine Syndrome)

Sporadic, Adult-Onset Nemaline Myopathy Presenting as Camptocormia (Bent-Spine Syndrome)

Alleviation of camptocormia by bilateral subthalamic nucleus stimulation in a patient with Parkinson's disease

Camptocormia, known also as “bent spine syndrome”, is characterized by involuntary truncal flexion of the thoraco-lumbar spine that appears in the sitting or standing position. Although Parkinson's disease (PD) is the most frequent etiologic factor, this postural disorder responds poorly to levodopa or other medications. We report a PD patient in whom chronic bilateral subthalamic nucleus stimulation produced a striking alleviation of camptocormia.

Camptocormia: Pathogenesis, classification, and response to therapy

Camptocormia: Pathogenesis, classification, and response to therapy

Camptocormia associated with focal myositis in multiple‐system atrophy

Camptocormia (CC) or pronounced forward flexion of the trunk is a common symptom of Parkinson's disease. We describe 2 patients with probable, respectively possible multiple-system atrophy and CC. Magnetic resonance imaging of the erector trunci showed focal patchy hyperintensities with gadolinium enhancement and muscle biopsy was indicative of variably pronounced focal myositis. CC was progressive and the major handicap for both patients after 1 and 1.5 years of follow-up, respectively. The therapeutic response was poor. Similarities with the dropped-head syndrome suggest that the muscle pathology may be either the primary cause of CC, a focal reaction to the CC posture, or a coincident syndrome of old age.

The bent spine syndrome: myopathy + biomechanics = symptoms

Background context The bent spine syndrome, which mimics spinal stenosis, is thought to be a focal paraspinal myopathy, but because paraspinal fatigue with ambulation is not a feature of more severe myopathies, the cause of symptoms is not clear. Purpose To evaluate electromyographic and biomechanical aspects of the bent spine syndrome. Study design/setting University spine clinic. Methods A patient with severe disability from the bent spine syndrome was compared with a fortuitously discovered asymptomatic research subject with the syndrome, in terms of physical examination, magnetic resonance imaging, and electrodiagnostic testing. Results Both subjects had fatty paraspinal replacement on magnetic resonance imaging and electromyography. More detailed electromyography of the patient showed abnormalities medially and caudally, but changes including apparent myopathic motor units up to the high thoracic region. The research subject had no hip flexion contracture, whereas the patient had severe contracture. Correction of contracture increased ambulation from 20 to 300 meters. Conclusions Bent spine syndrome is likely a paraspinal myopathy, but symptoms do not occur unless there is also a hip flexion contracture.

Response of the dropped head/bent spine syndrome to treatment with intravenous immunoglobulin

Weakness of neck extension causing a dropped head may result from many neuromuscular disorders. One etiology is isolated neck extensor myopathy. A similar focal myopathy of the lower axial muscles may cause the bent spine syndrome, which manifests as flexion of the trunk and inability to stand upright. Combination of both dropped head and bent spine myopathies is uncommon. Inflammation is usually not pronounced in these conditions and response to immunosuppressive treatment is rare. We present an 81-year-old man who developed progressive weakness of neck and trunk extension over several months, with a prominent inflammatory process in the thoracic paraspinal muscles, which responded dramatically to treatment with intravenous immunoglobulin (IVIg). This case, together with other rare reports, suggests that the presence of inflammation in the biopsy of an affected muscle may predict treatment response.

Camptocormia: A Heterogeneous Disorder

Camptocormia, a condition marked by abnormal bending of the trunk, is an unusual, often misdiagnosed disorder. Parkinson disease (PD) frequently

Camptocormia

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.