What you feel is not always what you've got. Jean Lhermitte (1877-1959) and the phantom limb phenomenon.

Jean Lhermitte (1877-1959), the French neurologist and psychiatrist, is most often associated with the sign he described in three patients with multiple sclerosis, back in 1927. In 1937, Lhermitte analytically studied a series of 28 amputees experiencing phantom limb sensations further to amputations dating between 1891 and 1934. After having described the main clinical characteristics of this unpublished series, we will detail the ideas advanced by Jean Lhermitte regarding the phenomenon of the phantom limb. Lhermitte will use these observations to develop conceptions of consciousness and the body schema encompassing very modern resonances.

French validation of the Quality of life in Essential Tremor Questionnaire (QUEST) and the Essential Tremor Embarrassment Assessment (ETEA).

Two scales have been developed and validated in English to evaluate the impact of tremor on daily life, namely Quality of life in Essential Tremor Questionnaire (QUEST) and Essential Tremor Embarrassment Assessment (ETEA). The psychometric properties of the French version of these two scales were assessed for 117 patients with head tremor. Both scales showed excellent acceptability, very good internal consistency (Cronbach's alpha coefficient>0.8) and reproducibility (Lin concordance coefficient>0.8), satisfactory external validity and satisfactory sensitivity to change. In conclusion, the French versions of QUEST and ETEA are comprehensive, valid and reliable instruments for assessing patients with head tremor.

Tabes dorsalis in the 19th century. The golden age of progressive locomotor ataxia.

Tabes dorsalis, a late neurological complication of syphilis, is nowadays almost extinct. The path to understanding this disease and its pathophysiology was long and winding, spanning multiple centuries. The 19th century was a crucial period for understanding it. In the first third of the century, German and French physicians defined the semiology of tabes dorsalis, renamed in France "ataxie locomotrice progressive [progressive locomotor ataxia]." Nevertheless, the multiplicity of ancient and recent terms and the description of sometimes unclear nosological concepts (tabes nervosa, tabes spasmodic, nervo-tabes, etc.) were a hindrance to understanding it. Tabes dorsalis was a fertile ground for the description of many clinical signs that have become classics in medicine. No real treatment was available and various unusual therapies were performed. For a long time, the etiology of this disease remained unknown. The link between syphilis and tabes dorsalis was slowly established in the second part of the 19th century from epidemiologic observations. We present an overview of the concept of tabes dorsalis in the medical context of the 19th century and discuss the medical observations of some famous patients suffering from the disease such as Édouard Manet (1832-1883) and Alphonse Daudet (1840-1897).

Unusual clinical and topographical midbrain stroke revealed by pure sensory disturbance.

We describe a rare midbrain stroke presenting with predominantly sensory symptoms. A 71-year-old woman was hospitalized due to the recurrence of faciobrachial sensory loss. Magnetic resonance imaging showed an infarct involving the right side of the middle portion of midbrain at the level of the inferior colliculus. In our case we tried to make some clinico-anatomical correlations. The main anatomical structures involved were the medial lemniscal tract, the ventral part of the spinothalamic tract and the trigeminothalamic tract. The location of the stroke would suggest there are overlaps between arterial territories of the midbrain. Clinicians should look for midbrain infarcts in cases of pure sensory stroke.

Ulnar neuropathy at the elbow: Reappraisal of the wrist-upper arm latency difference between ulnar and median nerves.

OBJECTIVES: To evaluate the sensitivity and specificity of the latency difference (DLat) between ulnar and median nerves of the arm after stimulation at the wrist; one of the easiest techniques proposed for recognizing ulnar neuropathy at the elbow (UNE). As latency difference is not a standardized technique, we set up a multicenter study to recruit large numbers of normal subjects and patients with UNE or generalized neuropathy. METHODS: Six centers participated in the study with data obtained from three groups of participants, controls (CTRLs), patients with UNE and patients with generalized neuropathy (GNP). We first verified the anatomical superposition of the ulnar and median nerves in cadaver examination. The optimal recording site for these two nerves was found to be 10 cm above the medial epicondyle. We then standardized the position of the arm with full extension of the elbow and stimulated first the median and then the ulnar nerves at the wrist. CTRLs were examined on both arms at two consecutive visits. RESULTS: We recorded 32 idiopathic UNE cases, 44 GNP patients and 62 controls. We demonstrated that a DLat cut-off value of 0.69 ms brings a sensitivity of 0.86 and specificity of 0.89 to discriminate CTRLs from UNE. We also validated that intra-examiner reproducibility was good. CONCLUSION: We report a lower normal value for DLat than reported in several non-standardized studies and CTRL and UNE groups have clearly separated DLat values. SIGNIFICANCE: Due to its high sensitivity, our standardized technique could be used as a first-line diagnostic tool when UNE is suspected.

Brueghel Syndrome or Meige Syndrome? Two Sides of a Same Disease.

Different eponyms such as "Wood syndrome," Meige syndrome, "Brueghel syndrome," "Blepharospasm plus syndrome" have been used to describe segmental craniocervical dystonias. These facial and/or oromandibular movement disorders are characterized by muscle contractions and spasms involving eyes, facial region, and sometimes pharynx, jaw, floor of the mouth, and tongue. The pathophysiology of craniocervical dystonia is poorly understood, but abnormal plasticity and impaired inhibition are suspected. Injection of botulinum toxin appears to be the best therapeutic option for treating segmental craniocervical dystonia. The objective of this chapter is to depict the history of segmental craniocervical dystonia in order to delineate the phenotypic spectrum of the disorders and to distinguish this entity from other facial and/or oromandibular movement disorders.

The medical itineraries of Blaise Cendrars. Neuropsychiatry marks life and literature.

Neuropsychiatry had a profound impact on the life and work of one of the most influential French writers of the 20th century, Frédéric Sauser, better known by his pen name Blaise Cendrars (1887-1961). Cendrars, whose right writing hand was amputated after a battlefield wound in 1915, described with acuity his stump pain and phantom limb syndrome. He became a left-handed writer. Between 1956 and his death in 1961, he also suffered two strokes that progressively paralyzed his left side and greatly diminished his ability to speak. Cendrars had started medical school in his youth and found that his ideas about the genesis of mental disorders conflicted with the generally accepted psychiatric conceptions of hysteria or psychoanalysis. His theories were greatly enriched by his observations of fellow World War I soldiers, victims of neuropsychiatric disorders. In his novels, many of his characters had borderline conditions, including two spectacularly mad serial killers, Moravagine and Fébronio. The case of Moravagine, fashioned after a patient with a brain tumor, allowed Cendrars to examine the nebulous frontier between neurological and psychiatric diseases.

Anatomy and cervical dystonia : "Dysfunction follows form".

At first glance, cervical dystonia might be an illustration of the well-known proposition "function follows form". Nevertheless, cervical dystonia is a highly non-physiological condition, which cannot be reproduced by healthy subjects and does not respond to the usual physiological rules. "Dysfunction follows form" might be the most accurate aphorism to define cervical dystonia. Taking into account this situation and recent insights, the anatomic approach needs to be adapted to allow a better understanding of semiology and to improve botulinum toxin therapy. In this review dealing with a new approach to cervical dystonia, we develop some practical anatomical concepts concerning the head and neck complex. Knowledge of cervical spine and muscular dysfunctions in cervical dystonia is an essential stage in treating cervical dystonia patients with botulinum toxin.

Successive motor nerve blocks to identify the muscles causing a spasticity pattern: example of the arm flexion pattern.

Botulinum Toxin A has been the main treatment for spasticity since the beginning of the 1990s. Surprisingly, there is still no consensus regarding injection parameters or, importantly, how to determine which muscles to target to improve specific functions. The aim of this study was to develop a systematic approach to determine this, using the example of the arm flexion pattern. We first determined anatomical landmarks for selective motor block of the brachialis nerve, using 20 forearms from 10 fresh cadavers in Ecole Européenne de Chirurgie and a university-based dissection centre, Paris, France. We then carried out selective blocks of the motor nerves to the brachialis, brachioradialis and biceps brachii in patients with stroke with an arm flexion pattern, in a University Rehabilitation Hospital, Garches, France. We measured: the resting angle of the elbow angle in standing (manual goniometer), active and passive range of extension, and spasticity using the Held and Tardieu and the Modified Ashworth scales. Range of passive elbow extension was also measured with the shoulder in 90° of flexion. The resting angle of the elbow in standing decreased by 35.0° (from 87.6 ± 23.7 to 52.6 ± 24.2°) with inhibition of brachialis, by a further 3.9° (from 52.6 ± 24.2 to 48.7 ± 23.7°) with inhibition of brachioradialis and a further 14.5° (from 48.7 ± 23.7to 34.2 ± 20.7°) with inhibition of biceps brachii. These results were consistent with the clinical evaluation of passive elbow range of motion with the shoulder at 90°. Sequential blocking of the nerves to the three main elbow flexors revealed that the muscle that limited elbow extension the most, was brachialis. This muscle should be the main target to improve the arm flexion pattern. These results show that it is important not simply to inject the most superficial or powerful muscles to treat a spastic deformity. A comprehensive assessment is required. The strategy proposed in this paper should increase the effectiveness of botulinum toxin injections by ensuring that the relevant muscles are targeted.

Anatomical and computed tomography study of the eighth costochondral junction: topography for costochondral graft harvesting.

INTRODUCTION: Costochondral grafts have long been used in maxillofacial reconstruction, but have been little used in trauma and orthopedic cases. This surgical technique requires that a graft be harvested from the thorax in the area of the eighth rib. Pleuropulmonary complications are very rare. Although the harvesting technique is simple, it needs to be demystified. GOAL OF STUDY: This study was performed to define anatomical relationships in the eighth costochondral junction and identify topographical and anatomical landmarks that will make it easier to harvest this structure. METHOD: This was a two-part study. First, an anatomical study was carried out on human cadaver thoraxes to define topographical landmarks and study the anatomical surroundings of the eighth costochondral junction. Second, an imaging study was performed using a database of existing patient computed tomography (CT) scans of the chest and abdomen to confirm the topographical landmarks defined in the first part of the study. The spine was used as a reference for both studies. The location of the eighth costochondral junction was defined relative to the spinal processes along with its location on the lower rib cage hemiperimeter in the transverse plane starting at the corresponding spinous process. RESULTS: The eighth costochondral junction was in line with the spinal process of the twelfth thoracic vertebra in the vast majority of cases and located at two-thirds of the lower rib cage hemiperimeter from the posterior median sulcus, regardless of the patient's chest shape, age and gender. This junction was always located under a single muscle (external oblique) and protected by a thick perichondrium layer, which separates it from the intercostal pedicles, endothoracic fascia and parietal pleura. DISCUSSION: This two-part study has identified reliable landmarks for harvesting of an osteochondral graft at the eighth costochondral junction and, by describing its anatomical surroundings, helps take the mystery out of its harvesting. These landmarks were identified in supine cadavers and in free-breathing patients lying in supine for the CT portion. This position must be used when identifying these landmarks in a patient undergoing costochondral autograft harvesting for cartilage reconstruction.

Dimensions of the trapezium bone: a cadaver and CT study.

PURPOSE: The primary purpose of this study was to define the size of the trapezium bone through measurements on cadaver specimens and CT scans of living subjects. The secondary purpose of this study was to determine if any correlation existed between the size of the trapezium and local anatomical parameters. METHODS: The radio-ulnar length (L), dorsopalmar width (ℓ) and height (h) of the distal surface of the trapezium were measured by two independent observers on 20 cadaver specimens. The same measurements were carried out by two other observers on anonymized CT scans from 18 patients. The inter- and intra-observer agreement was determined using the intraclass correlation coefficient. RESULTS: In the cadavers, the mean length, width and height of the trapezium were 22.8, 15.5 and 15.2 mm, respectively. On the CT scans, these same dimensions were 19.2, 11.4 and 11.6 mm. Inter-observer agreement was statistically significant in both parts of the study. DISCUSSION: The dimensions of the trapezium bone were about 3.33 mm larger in cadavers than on CT scans. These differences can be explained partially by a systematic under-sizing error on the CT scans and the fact that the cartilage layer cannot be directly visualized. CONCLUSION: This study was able to define the dimensions of the trapezium bone. It may be possible to predict the trapezium height from the length of the forearm or the width of the radial epiphysis. Our data can be used to adjust the size of trapezium implants to the dimensions of the patient's bone.

Cadaver study of the topography of the musculotendinous junction of the finger extensor muscles: applicability to tendon rupture following closed wrist trauma.

Rupture of the extensor pollicis longus (EPL) tendon in the wrist is a delayed complication that can occur after wrist injury. Several etiology-related hypotheses have been made to explain these ruptures. The one most commonly accepted is necrosis at the musculotendinous junction of the EPL, which is compressed between the extensor retinaculum and dorsal aspect of the radius. To confirm this hypothesis, we performed an anatomical study to show the close relationship between the extensor retinaculum and the musculotendinous junction of the EPL muscle. We calculated the distance between the musculotendinous junction of the various finger extensor muscles and the proximal edge of the extensor retinaculum. We were able to show that this junction is located under the extensor retinaculum for the extensor indicis (EI) and EPL muscles, but the latter is in the third extensor compartment, which is a tight, confined space. Any pressure increase in this space following trauma, for example, can bring about compartment syndrome at this musculotendinous junction, which some authors have found to be poorly vascularized.

An unusual case of Y-shaped right renal vein.

Vascular renal anomalies are frequent, multiple and well described and result from errors in vessel embryogenesis between the 6th and 10th week of gestation. Historically, variations are described in anatomic dissection and currently mostly in image interpretation. We report an anatomic variation concerning the right renal vein which, to our knowledge, has never been described in the literature either by dissection or by radiological examination. This variation was discovered during the routine dissection of an embalmed male body. It consists of a Y-shaped right renal vein and is associated with multiple retroperitoneal variations: a bilateral accessory renal artery, a trident ending of the right renal artery and a left testicular vein variation. Venous and arterial renal anatomy and its variations are fundamentally important in renal surgery, especially concerning living donor renal grafts. These variations may be diagnosed thanks to injected tomodensitometry which has a good sensitivity and specificity for anomalies. Preoperative diagnosis of an anatomic vascular renal variation may reduce morbidity during surgery, which is why precise examination of injected tomography should be mandatory.

The piriformis muscle syndrome: an exploration of anatomical context, pathophysiological hypotheses and diagnostic criteria.

INTRODUCTION: The piriformis muscle syndrome (PMS) has remained an ill-defined entity. It is a form of entrapment neuropathy involving compression of the sciatic nerve by the piriformis muscle. Bearing this in mind, a medical examination is likely to be suggestive, as a classical range of symptoms corresponds to truncal sciatica with frequently fluctuating pain, initially in the muscles of the buttocks. PATHOPHYSIOLOGICAL HYPOTHESES: The piriformis muscle is biarticular, constituting a bridge in front of and below the sacroiliac joint and behind and above the coxo-femoral joint. It is essentially a lateral rotator but also a hip extensor, and assumes a secondary role as an abductor. Its action is nonetheless conditioned by the position of the homolateral coxo-femoral joint, and it can also function as a hip medial rotator, with the hip being flexed at more than 90°. The main clinical manoeuvres are derived from these types of biomechanical considerations. For instance, as it is close to the hip extensors, the piriformis muscle is tested in medial rotation stretching, in resisted contraction in lateral rotation. On the other hand, when hip flexion surpasses 90°, the piriformis muscle is stretched in lateral rotation, and we have consequently laid emphasis on the manoeuvre we have termed Heel Contra-Lateral Knee (HCLK), which must be prolonged several tens of seconds in order to successfully reproduce the buttocks-centred and frequently associated sciatic symptoms. CONCLUSION: A PMS diagnosis is exclusively clinical, and the only objective of paraclinical evaluation is to eliminate differential diagnoses. The entity under discussion is real, and we favour the FAIR, HCLK and Freiberg stretching manoeuvres and Beatty's resisted contraction manoeuvre.

Piriformis muscle syndrome: diagnostic criteria and treatment of a monocentric series of 250 patients.

OBJECTIVES: Piriformis Muscle Syndrome (PMS) is caused by sciatic nerve compression in the infrapiriformis canal. However, the pathology is poorly understood and difficult to diagnose. This study aimed to devise a clinical assessment score for PMS diagnosis and to develop a treatment strategy. MATERIAL AND METHODS: Two hundred and fifty patients versus 30 control patients with disco-radicular conflict, plus 30 healthy control subjects were enrolled. A range of tests was used to produce a diagnostic score for PMS and an optimum treatment strategy was proposed. RESULTS: A 12-point clinical scoring system was devised and a diagnosis of PMS was considered 'probable' when greater or equal to 8. Sensitivity and specificity of the score were 96.4% and 100%, respectively, while the positive predictive value was 100% and negative predictive value was 86.9%. Combined medication and rehabilitation treatments had a cure rate of 51.2%. Hundred and twenty-two patients (48.8%) were unresponsive to treatment and received OnabotulinumtoxinA. Visual Analogue Scale (VAS) results were 'Very good/Good' in 77%, 'Average' in 7.4% and 'Poor' in 15.6%. Fifteen of 19 patients unresponsive to treatment underwent surgery with 'Very good/Good' results in 12 cases. CONCLUSIONS: The proposed evaluation score may facilitate PMS diagnosis and treatment standardisation. Rehabilitation has a major role associated in half of the cases with botulinum toxin injections.

Time course of odorant- and trigeminal-induced activation in the human brain: an event-related functional magnetic resonance imaging study.

It is well known that most odorants stimulate the trigeminal system but the time course of the brain regions activated by these chemical stimulations remains poorly documented, especially regarding the trigeminal system. This functional magnetic resonance imaging (fMRI) study compares brain activations resulting from the contrast between two odorant conditions (one bimodal odor and one relatively pure olfactory stimulant) according to the duration of the stimulation (i.e. one inhalation, or three or six successive inhalations). The results show striking differences in the main brain regions activated according to these durations. The caudate nucleus and the orbitofrontal cortex are only involved in short-duration stimulations, and the posterior insular cortex and post-central gyrus (SI) are only activated by long duration stimulations. Different regions of the frontal, temporal and occipital lobe are activated depending on the duration but mainly during medium-duration stimulations. These results expand on the findings of previous studies and contribute to the description of temporal networks in trigeminal perception.

[Transient elastography of calcaneal tendon: preliminary results and future prospects]. / Élastographie transitoire du tendon calcanéen: résultats préliminaires et perspectives.

PURPOSE: To evaluate transient ShearWave elastography of the normal Achilles' tendon. PATIENTS AND METHODS: The Achilles' tendon of 30 normal subjects were prospectively assessed using a Rubi V1Sq prototype (Supersonic Imagine). Quantitative elastography maps displayed in kilopascals with a scale of 0 to 600kPa were generated from transverse and longitudinal images at 3 different levels of plantar flexion. Subgroups were compared and analyzed based on proven or suspected variation factors (age, gender, level of physical activity). RESULTS: On sagittal images, mean elasticity was 104±46kPa during extension, 464±144kPa in neutral position and 410±196kPa during maximum dorsiflexion. There was significant increase in elasticity when the Achilles' tendon was maximally stretched (p<0.01). The intra-individual correlation between right and left tendons during ankle extension was good (Pearson Coefficient 0.8; p<0.01). Finally, physically active subjects showed significantly greater elasticity than non-active subjects (p<0.05). CONCLUSION: Transient ShearWave elastography of the Achilles' tendon is a simple technique that provides real-time information about tissue elasticity.