Sjögren syndrome-related plasma cell disorder and multifocal nodular AL amyloidosis: clinical picture and pathological findings.

Background: Localized nodular deposits of AL amyloid are seen in different tissues/organs; however, the pathogenesis of this form of amyloidosis remains unclear. Recently, Sjögren syndrome combined with localized nodular AL amyloidosis has been noted. Here, we report Sjögren syndrome cases showing multifocal nodular AL amyloidosis and the followed benign course. Materials and methods: We investigated the clinical pictures and histopathological findings of three cases with both presence of Sjögren syndrome and localized nodular AL amyloidosis, paying a special attention to the distribution of amyloidoma. Results: All three cases were middle-aged females. In two of three cases localized deposits of AL amyloid preceded Sjögren syndrome. Amyloidoma was detected in scalp, eyelid, cheek, larynx, trachea, lung and breast, and around these amyloid-deposited lesions infiltration of plasma cells was seen. Pulmonary amyloidosis was consistently accompanied with parenchymal cystic lesions, but this amyloidosis did not produce any significant respiratory symptoms. Some of large pulmonary amyloidomas showed cavity formation and subsequent shrinkage. In two cases amyloid deposition was found on gastric mucosa. Two cases received small doses of oral prednisone, with no further appearance of amyloidoma. Conclusion: Sjögren syndrome-related plasma cell disorder may be responsible for the formation of this unique multifocal nodular AL amyloidosis.

Bioelectrical Impedance Analysis of Water Reduction in Lower-Limb Lymphedema by Lymphaticovenular Anastomosis.

BACKGROUND: Although lymphedema is fundamentally abnormal accumulation of excess water in the extracellular space, previous studies have evaluated the efficacy of physiological bypass surgery (lymphaticovenular anastomosis [LVA]) for lymphedema without measuring water volume. This study clarified the water reductive effect of LVA using bioelectrical impedance analysis (BIA). METHODS: The efficacy of LVA for unilateral lower-limb lymphedema was evaluated using BIA in a retrospective cohort. The water volume of affected and unaffected legs was measured using multifrequency BIA before and after LVA. Preoperative measurements were undertaken after compression therapy for at least 3 months. The follow-up period after LVA was a minimum of 6 months. RESULTS: Thirty consecutive patients with unilateral lower-limb lymphedema were enrolled. The mean water volume reduction of the affected leg by LVA (ΔLBW) was 0.86 L (standard deviation [SD]: 0.86, median: 0.65) with a mean number of 3.3 anastomoses (SD: 1.7). The mean reduction rate of edema was 45.1% (SD: 36.3). Multiple linear regression analysis revealed water volume difference between the affected and unaffected legs before LVA (excess LBW) as the strongest predictor of ΔLBW (R 2 = 0.759, p < 0.01; ß = 0.500, p < 0.01). CONCLUSION: The LVA reduces the volume of accumulated body water in lower-limb lymphedema. As excess LBW most strongly predicted the amount of water volume reduction by LVA, body water volume measurement by BIA before LVA might identify patients with low excess LBW not expected to benefit from LVA, regardless of apparent differences in limb circumference.

Near-Infrared Irradiation Increases Length of Axial Pattern Flap Survival in Rats.

Objective: We previously reported that near-infrared irradiation nonthermally induces long-lasting vasodilation of the subdermal plexus by causing apoptosis of vascular smooth muscle cells. To clarify the possible application of near-infrared irradiation to prevent skin flap necrosis, we evaluated the length of axial pattern flap survival in rats by near-infrared irradiation. Methods: A bilaterally symmetric island skin flap was elevated under the panniculus carnosus on the rat dorsum. Half of the flap was subjected to near-infrared irradiation just before flap elevation with a device that simulates solar radiation, which has a specialized contact cooling apparatus to avoid thermal effects. The length of flap survival of the near-infrared irradiated side was measured 7 days after flap elevation and compared with the nonirradiated side. Results: The irradiated side showed elongation of flap survival compared with the nonirradiated side (73.3 ± 11.7 mm vs 67.3 ± 14.9 mm, respectively, P = .03). Conclusions: Near-infrared irradiation increases the survival length of axial pattern flaps in rats.

Eyelid Opening with Trigeminal Proprioceptive Activation Regulates a Brainstem Arousal Mechanism.

Eyelid opening stretches mechanoreceptors in the supratarsal Müller muscle to activate the proprioceptive fiber supplied by the trigeminal mesencephalic nucleus. This proprioception induces reflex contractions of the slow-twitch fibers in the levator palpebrae superioris and frontalis muscles to sustain eyelid and eyebrow positions against gravity. The cell bodies of the trigeminal proprioceptive neurons in the mesencephalon potentially make gap-junctional connections with the locus coeruleus neurons. The locus coeruleus is implicated in arousal and autonomic function. Due to the relationship between arousal, ventromedial prefrontal cortex, and skin conductance, we assessed whether upgaze with trigeminal proprioceptive evocation activates sympathetically innervated sweat glands and the ventromedial prefrontal cortex. Specifically, we examined whether 60° upgaze induces palmar sweating and hemodynamic changes in the prefrontal cortex in 16 subjects. Sweating was monitored using a thumb-mounted perspiration meter, and prefrontal cortex activity was measured with 45-channel, functional near-infrared spectroscopy (fNIRS) and 2-channel NIRS at Fp1 and Fp2. In 16 subjects, palmar sweating was induced by upgaze and decreased in response to downgaze. Upgaze activated the ventromedial prefrontal cortex with an accumulation of integrated concentration changes in deoxyhemoglobin, oxyhemoglobin, and total hemoglobin levels in 12 subjects. Upgaze phasically and degree-dependently increased deoxyhemoglobin level at Fp1 and Fp2, whereas downgaze phasically decreased it in 16 subjects. Unilateral anesthetization of mechanoreceptors in the supratarsal Müller muscle used to significantly reduce trigeminal proprioceptive evocation ipsilaterally impaired the increased deoxyhemoglobin level by 60° upgaze at Fp1 or Fp2 in 6 subjects. We concluded that upgaze with strong trigeminal proprioceptive evocation was sufficient to phasically activate sympathetically innervated sweat glands and appeared to induce rapid oxygen consumption in the ventromedial prefrontal cortex and to rapidly produce deoxyhemoglobin to regulate physiological arousal. Thus, eyelid opening with trigeminal proprioceptive evocation may activate the ventromedial prefrontal cortex via the mesencephalic trigeminal nucleus and locus coeruleus.

Longitudinal slit procedure in addition to negative pressure wound therapy for a refractory wound with exposed achilles tendon.

OBJECTIVE: This case report reviews features of negative pressure wound therapy, particularly for the exposed Achilles tendon, and describes an additional effective procedure. METHODS: An 87-year-old man presented with a soft-tissue defect measuring 3×5 cm with the exposed Achilles tendon as a sequela of deep burn. The condition of his affected leg was ischemic because of arteriosclerosis. We used negative pressure wound therapy and made 2 longitudinal slits penetrating the tendon to induce blood flow from the ventral side to the dorsal surface. RESULTS: By this combination therapy, the surface of the exposed Achilles tendon was completely epithelialized and the tendon was spared without disuse syndrome. CONCLUSIONS: The authors conclude that this combination therapy is useful for covering the widely exposed tendon in aged patients.

Desensitization of the Mechanoreceptors in Müller's Muscle Reduces the Increased Reflex Contraction of the Orbicularis Oculi Slow-Twitch Fibers in Blepharospasm.

OBJECTIVE: Although the mixed orbicularis oculi muscle lacks the muscle spindles required to induce reflex contraction of its slow-twitch fibers, the mechanoreceptors in Müller's muscle function as extrinsic mechanoreceptors to induce reflex contraction. We hypothesize that strong stretching of these mechanoreceptors increases reflex contraction of the orbicularis oculi slow-twitch muscle fibers, resulting in blepharospasm. METHODS: We examined a 71-year-old man with right blepharospasm and bilateral aponeurosis-disinserted blepharoptosis to determine whether the patient's blepharospasm was worsened by increased trigeminal proprioceptive evocation via stretching of the mechanoreceptors in Müller's muscle owing to a 60° upward gaze and serrated eyelid closure, and whether local anesthesia of the mechanoreceptors via lidocaine administration to the upper fornix as well as surgical disinsertion of Müller's muscle from the tarsus and fixation of the disinserted aponeurosis to the tarsus decreased trigeminal proprioceptive evocation and improved patient's blepharospasm. RESULTS: Before pharmacological desensitization, 60° upward gaze and serrated eyelid closure exacerbated the patient's blepharospasm. In contrast, these maneuvers did not worsen his blepharospasm following lidocaine administration. One year after surgical desensitization, the blepharospasm had disappeared and a 60° upward gaze did not induce blepharospasm. CONCLUSIONS: Strong stretching of the mechanoreceptors in Müller's muscle appeared to increase reflex contraction of the orbicularis oculi slow-twitch muscle fibers, resulting in blepharospasm. In addition to botulinum neurotoxin injections into the involuntarily contracted orbicularis oculi muscle and myectomy, surgical desensitization of the mechanoreceptors in Müller's muscle may represent an additional procedure to reduce blepharospasm.

Trigeminal Proprioception Evoked by Strong Stretching of the Mechanoreceptors in Müller's Muscle Induces Reflex Contraction of the Orbital Orbicularis Oculi Slow-Twitch Muscle Fibers.

OBJECTIVE: The mixed orbicularis oculi muscle lacks an intramuscular proprioceptive system such as muscle spindles, to induce reflex contraction of its slow-twitch fibers. We evaluated whether the mechanoreceptors in Müller's muscle function as extrinsic mechanoreceptors to induce reflex contraction of the slow-twitch fibers of the orbicularis oculi in addition to those of the levator and frontalis muscles. METHODS: We evaluated in patients with aponeurosis-disinserted blepharoptosis whether strong stretching of the mechanoreceptors in Müller's muscle from upgaze with unilateral lid load induced reflex contraction of the orbicularis oculi slow-twitch fibers and whether anesthesia of Müller's muscle precluded the contraction. We compared the electromyographic responses of the bilateral orbicularis oculi muscles to unilateral intraoperative direct stimulation of the trigeminal proprioceptive nerve with those to unilateral transcutaneous electrical stimulation of the supraorbital nerve. RESULTS: Upgaze with a unilateral 3-g lid load induced reflex contraction of the bilateral orbicularis oculi muscles with ipsilateral dominance. Anesthesia of Müller's muscle precluded the reflex contraction. The orbicularis oculi reflex evoked by stimulation of the trigeminal proprioceptive nerve differed from that by electrical stimulation of the supraorbital nerve in terms of the intensity of current required to induce the reflex, the absence of R1, and duration. CONCLUSIONS: The mechanoreceptors in Müller's muscle functions as an extramuscular proprioceptive system to induce reflex contraction of the orbital orbicularis oculi slow-twitch fibers. Whereas reflex contraction of the pretarsal orbicularis fast-twitch fibers functions in spontaneous or reflex blinking, that of the orbital orbicularis oculi slow-twitch fibers may factor in grimacing and blepharospasm.

Pulmonary microvascular remodeling after balloon pulmonary angioplasty in a patient with chronic thromboembolic pulmonary hypertension.

We herein report a case of peripheral type chronic thromboembolic pulmonary hypertension treated with medical therapy and subsequent balloon pulmonary angioplasty (BPA). After a series of BPA procedures, the patient's hemodynamics almost completely normalized. The patient was later diagnosed with lung carcinoma, and the vasculature of the resected lung demonstrated intimal thickening and luminal stenosis in the pulmonary arteries in both the areas where BPA was performed and not performed, in spite of a marked reduction in pulmonary arterial pressure. The present case is the first report on the histology of the pulmonary vasculature following BPA.

Prednisolone markedly reduced serum IgG4 levels along with the improvement of pituitary mass and anterior pituitary function in a patient with IgG4-related infundibulo-hypophysitis.

In 2011 a 76 year-old man with a medical history of diabetes, hypertension and autoimmune pancreatitis was admitted to our hospital because of anorexia, general malaise and repeated hypoglycemia. When he was 72 years old, he suffered from pancreatitis, and pancreas head tumor was operated. IgG4-related pancreatitis was diagnosed histopathologically. On admission anterior pituitary function test revealed impaired response of ACTH and cortisol to CRH, and no response of GH, TSH and gonadotropin to GHRH, TRH and LHRH, respectively. Baseline PRL level was elevated. Serum IgG and IgG4 levels were markedly elevated. Pituitary MRI showed significant enlargement of pituitary gland and stalk. Chest CT suggested IgG4-related lung disease. IgG4-related infundibulo-hypophysitis was diagnosed based on the above mentioned past history and results of present examinations. Twenty mg of hydrocortisone, followed by 20 mg of prednisolone (PSL) and 25 µg of levothyroxine markedly reduced serum IgG4 levels and ameliorated the symptom, the size of pituitary and stalk, and anterior pituitary function (TSH, GH and gonadotropin), although diabetes insipidus became apparent due to glucocorticoid administration. This is a typical case of IgG4-related hypophysitis in which PSL causes marked improvement of pituitary mass and pituitary function along with the reduction of serum IgG4 levels.

CASE REPORT Penetrating Injury of the Orbit by a Needlefish.

OBJECTIVE: We present a very rare case of penetrating injury into the orbit by a needlefish. The patient underwent extirpation twice at another hospital. METHODS: We performed foreign body removal from a right subbrow incision under general anesthesia. RESULTS: The foreign body was successfully removed and the patient's diplopia recovered gradually after surgery. CONCLUSIONS: Treatment was similar to that used for penetrating injury. As there is a risk of secondary infection, it is important to completely remove the fish body, followed by vigorous irrigation and debridement.

Developed lower-positioned transverse ligament restricts eyelid opening and folding and determines Japanese as being with or without visible superior palpebral crease.

INTRODUCTION: We have reported that a developed lower-positioned transverse ligament between the superior-medial orbital rim and the lateral orbital rim on the lateral horn in the lower orbital fat space antagonizes eyelid opening and folding in certain Japanese to produce narrow eye, no visible superior palpebral crease, and full eyelid. In this study, we confirmed relationship between development of the lower-positioned transverse ligament and presence of the superior palpebral crease. METHODS: We evaluated whether (1) digital immobilization of eyebrow movement during eyelid opening and (2) a developed lower-positioned transverse ligament could classify Japanese subjects as being with or without visible superior palpebral crease. RESULTS: Digital immobilization of eyebrow movement restricted eyelid opening in all subjects without visible superior palpebral crease but did not restrict in any subject with visible superior palpebral crease. Macroscopic and microscopic evidence revealed that the lower-positioned transverse ligament behind the lower orbital septum in subjects without visible superior palpebral crease was significantly more developed than that in subjects with visible superior palpebral crease. CONCLUSIONS: Since a developed lower-positioned transverse ligament antagonizes opening and folding of the anterior lamella of the upper eyelid in subjects without visible superior palpebral crease, these individuals open their eyelids by lifting the eyebrow with the anterior lamella and the lower-positioned transverse ligament owing to increased tonic contraction of the frontalis muscle, in addition to the retractile force of the levator aponeurotic expansions. In subjects with visible superior palpebral crease, the undeveloped lower-positioned transverse ligament does not antagonize opening and folding of the anterior lamella, and so they open their eyelids by folding the anterior lamella on the superior palpebral crease via the retractile force of the levator aponeurotic expansions.

The Supraorbital Margin of Japanese Who Have No Visible Superior Palpebral Crease and Persistently Lift the Eyebrow in Primary Gaze is Higher and More Obtuse Than Those Who Do Not.

OBJECTIVE: Anthropological studies divided the Japanese into the Yayoi migrants, who had narrow eye, no visible superior palpebral crease, and high-positioned round supraorbital margins for cold tolerance, and the Jomon natives, who had wide eye, visible superior palpebral crease, and low-positioned straight supraorbital margins, thus suggesting an anatomical discrepancy between the vertical palpebral fissure and the height of the supraorbital margin. Because Japanese subjects without visible superior palpebral crease open their eyelids by lifting the eyebrows with the anterior lamella owing to increased tonic contraction of the frontalis muscle, we hypothesized that persistently lifting the eyebrows in primary gaze mechanically remodels the supraorbital margin to be high positioned and round. METHOD: We evaluated whether subjects without visible superior palpebral crease persistently lifted their eyelids more than subjects with visible superior palpebral crease, whether the presence of persistently lifted eyebrow in primary gaze affected the relative height of the supraorbital margin in coronal view 3-dimensional computed tomography imaging, and whether the shape of the supraorbital margin in the coronal view affected that in the sagittal view 3-dimensional computed tomography imaging. RESULTS: Eyebrow height in subjects without visible superior palpebral crease was significantly larger than that in subjects with visible superior palpebral crease. The supraorbital margin of subjects without visible superior palpebral crease who persistently lifted the eyebrows in primary gaze was higher (rounder) and more obtuse than that of Japanese subjects with visible superior palpebral crease who did not. CONCLUSIONS: The mechanical pressure applied to the supraorbital margin by persistently lifting the eyebrows appears to functionally, rather than genetically, create the high (round) and obtuse supraorbital margin.

Usefulness of infrared thermal imaging camera for screening of postoperative surgical site infection after the nuss procedure.

Introduction and Objective. The Nuss procedure is widely used in the treatment of pectus excavatum worldwide. Postoperative pectus bar infection is one of the most serious complications associated with this procedure. Therefore, early detection of signs of implant infection is very important. However, this is difficult, and effective methods have yet to be established. Methods. We use a handheld infrared thermal imaging camera to screen patients for postoperative infection following the Nuss procedure. Here, we report a 28-year-old man with recurrent postoperative (Ravitch procedure) pectus excavatum. Results. Infrared thermography camera clearly indicated slight cellulitis in the right chest. Conclusion. Our technique may assist in preventing postoperative bar infection and removal caused by severe bar infection. Furthermore, this camera is potentially suitable for many situations in infection monitoring following subcutaneous implant surgery.

Eyebrow ptosis after blowout fracture indicates impairment of trigeminal proprioceptive evocation that induces reflex contraction of the frontalis muscle.

OBJECTIVE: The mixed levator and frontalis muscles lack the interior muscle spindles normally required to induce involuntary contraction of their slow-twitch fibers. To involuntarily move the eyelid and eyebrow, voluntary contraction of the levator nonskeletal fast-twitch muscle fibers stretches the mechanoreceptors in Müller's muscle to evoke trigeminal proprioception, which then induces reflex contraction of the levator and frontalis skeletal slow-twitch muscle fibers. The trigeminal proprioceptive nerve has a long intraorbital course from the mechanoreceptors in Müller's muscle to the superior orbital fissure. Since external force to the globe may cause impairment of trigeminal proprioceptive evocation, we confirmed how unilateral blowout fracture due to a hydraulic mechanism affects ipsilateral eyebrow movement as compared with unilateral zygomatic fracture. METHODS: In 16 unilateral blowout fracture patients, eyebrow heights were measured on noninjured and injured sides in primary and 60° upward gaze and statistically compared. Eyebrow heights were also measured in primary gaze in 24 unilateral zygomatic fracture patients and statistically compared. RESULTS: In the blowout fracture patients, eyebrow heights on the injured side were significantly smaller than on the noninjured side in both gaze. In the zygomatic fracture patients, eyebrow heights on the injured side were significantly larger than on the noninjured side in primary gaze. CONCLUSION: Since 60° upward gaze did not recover the eyebrow ptosis observed in primary gaze in blowout fracture patients, such ptosis indicated impairment of trigeminal proprioceptive evocation and the presence of a hydraulic mechanism that may require ophthalmic examination.

A ringed fascia lata graft without peritendinous areolar tissue encircling the levator veli palatini and superior pharyngeal constrictor muscles gradually shrinks to reduce velopharyngeal incompetence, functioning as an intravelar palatal lift.

INTRODUCTION: We have previously reported that fascia lata grafts with peritendinous areolar tissue used to treat severe congenital blepharoptosis gradually shrink within 6 weeks postoperatively and maintain long-term shrinkage of 15.5% on average. Accordingly, it seemed possible that a fascia lata graft without peritendinous areolar tissue would shrink more than the one with peritendinous areolar tissue in a clinical setting. We evaluated this possibility in a patient with Klippel-Feil syndrome having postoperative deep atonic nasopharynx. METHODS: In combination with intravelar veloplasty and palatal lengthening with modified bilateral buccinator sandwich pushback, a ringed fascia lata without peritendinous areolar tissue encircling the levator veli palatini and superior constrictor muscles was grafted to cure severe velopharyngeal incompetence. RESULTS: Obstructive sleep apnea did not occur following surgery. Pharyngoscopy, videofluoroscopy, and nasometry showed no amelioration of velopharyngeal incompetence at 1 month postoperatively, but marked velopharyngeal incompetence reduction was evident at 4 months and 2 years after surgery. CONCLUSIONS: The extended recovery period suggests that the anticipated postoperative shrinkage of the ringed fascia lata without peritendinous areolar tissue played a more prominent role than intravelar veloplasty and palatal lengthening, which posteroinferiorly elongated the atonic soft palate. Although the pharyngeal flap procedure is the most popular technique for treatment of velopharyngeal incompetence, it is sometimes accompanied by respiratory complications. Thus, the gradual postoperative shrinkage of a ringed fascia lata graft encircling the velopharyngeal muscles functions as an intravelar palatal lift and may be an additional surgical method with less respiratory complications to narrow atonic nasopharyngeal port.

Electromyographic comparison of forearm muscle movements for fine skin suturing between an enlarged pen needle holder and a webster needle holder.

BACKGROUND: For surgical suturing, a Webster needle holder uses wrist supinating with supinator and extrinsic muscles, whereas a pen needle holder uses finger twisting with intrinsic and extrinsic muscles. Because the latter is better suited to microsurgery, which requires fine suturing with less forearm muscle movement, we have recently adopted an enlarged pen needle holder scaled from a micro needle holder for fine skin suturing. In this study, we assessed whether the enlarged pen needle holder reduced forearm muscle movement during fine skin suturing as compared with the Webster needle holder. METHODS: A fine skin-suturing task was performed using pen holding with the enlarged micro needle holder or scissor holding with the Webster needle holder by 9 experienced and 6 inexperienced microsurgeons. The task lasted for 60 seconds and was randomly performed 3 times for each method. Forearm flexor and extensor muscular activities were evaluated by surface electromyography. RESULTS: The enlarged pen needle holder method required significantly less forearm muscle movement for experienced microsurgeons despite it being their first time using the instrument. There was no significant difference between 2 methods for inexperienced microsurgeons. CONCLUSIONS: Experienced microsurgeons conserved forearm muscle movement by finger twisting in fine skin suturing with the enlarged pen needle holder. Inexperienced microsurgeons may benefit from the enlarged pen needle holder, even for fine skin suturing, to develop their internal acquisition model of the dynamics of finger twisting.

Lateral vermilion border transposition flap to correct vermilion deformities with unilateral or bilateral cleft lip.

Previously, bilateral lateral vermilion border transposition flaps were reported to correct whistling lip deformity in patients with cleft lip. This study examined the long-term outcome in these patients and modified the procedure. Here, the use of this procedure is reported in 69 cleft lip patients. The follow-up period after the operation ranged from 4-14 years. In addition to the standard method, a unilateral flap was applied for asymmetrical vermilion and small bilateral flaps were applied to loosen the transverse tightness of the vermilion. The asymmetry of the tightness was improved in all patients. The reconstructed tubercle diminished in volume gradually over a few years postoperatively. Secondary deformities after its use were subtly everted tubercle during smiling (18.8%) and irregularities on the labial-free margin (21.7%). They were corrected by minor follow-up revision. Although it is necessary to wait several years until the postoperative shape has stabilised, this is a simple and widely available method to reconstruct a naturally-shaped vermilion in cleft lip patients.

Surgical desensitisation of the mechanoreceptors in Müller's muscle relieves chronic tension-type headache caused by tonic reflexive contraction of the occipitofrontalis muscle in patients with aponeurotic blepharoptosis.

Proprioceptively innervated intramuscular connective tissues in Müller's muscle function as exterior mechanoreceptors to induce reflex contraction of the levator and occipitofrontalis muscles. In aponeurotic blepharoptosis, since the levator aponeurosis is disinserted from the tarsus, stretching of the mechanoreceptors in Müller's muscle is increased even on primary gaze to induce phasic and tonic reflexive contraction of the occipitofrontalis muscle. It was hypothesised that in certain patients with aponeurotic blepharoptosis, the presence of tonic reflexive contraction of the occipitofrontalis muscle due to the sensitised mechanoreceptors in Müller's muscle, can cause chronic tension-type headache (CTTH) associated with occipitofrontalis tenderness. To verify this hypothesis, this study evaluated (1) what differentiates patients with CTTH from patients without CTTH, (2) how pharmacological contraction of Müller's smooth muscle fibres as a method for desensitising the mechanoreceptors in Müller's muscle affects electromyographic activity of the frontalis muscle, and (3) how surgical aponeurotic reinsertion to desensitise the mechanoreceptors in Müller's muscle electromyographically or subjectively affects activities of the occipitofrontalis muscle or CTTH. It was found that patients had sustained CTTH when light eyelid closure did not markedly reduce eyebrow elevation. However, pharmacological contraction of Müller's smooth muscle fibres or surgery to desensitise the mechanoreceptor electromyographically reduced the tonic contraction of the occipitofrontalis muscle on primary gaze and subjectively relieved aponeurotic blepharoptosis-associated CTTH. Over-stretching of the mechanoreceptors in Müller's muscle on primary gaze may induce CTTH due to tonic reflexive contraction of the occipitofrontalis muscle. Therefore, surgical desensitisation of the mechanoreceptors in Müller's muscle appears to relieve CTTH.

Electrical stimulation to the trigeminal proprioceptive fibres that innervate the mechanoreceptors in Müller's muscle induces involuntary reflex contraction of the frontalis muscles.

The levator and frontalis muscles lack interior muscle spindles, despite consisting of slow-twitch fibres that involuntarily sustain eyelid-opening and eyebrow-raising against gravity. To compensate for this anatomical defect, this study hypothetically proposes that initial voluntary contraction of the levator fast-twitch muscle fibres stretches the mechanoreceptors in Müller's muscle and evokes proprioception, which continuously induces reflex contraction of slow-twitch fibres of the levator and frontalis muscles. This study sought to determine whether unilateral transcutaneous electrical stimulation to the trigeminal proprioceptive fibres that innervate the mechanoreceptors in Müller's muscle could induce electromyographic responses in the frontalis muscles, with monitoring responses in the orbicularis oculi muscles. The study population included 27 normal subjects and 23 subjects with aponeurotic blepharoptosis, who displayed persistently raised eyebrows on primary gaze and light eyelid closure. The stimulation induced a short-latency response in the ipsilateral frontalis muscle of all subjects and long-latency responses in the bilateral frontalis muscles of normal subjects. However, it did not induce long-latency responses in the bilateral frontalis muscles of subjects with aponeurotic blepharoptosis. The orbicularis oculi muscles showed R1 and/or R2 responses. The stimulation might reach not only the proprioceptive fibres, but also other sensory fibres related to the blink or corneal reflex. The experimental system can provoke a monosynaptic short-latency response in the ipsilateral frontalis muscle, probably through the mesencephalic trigeminal proprioceptive neuron and the frontalis motor neuron, and polysynaptic long-latency responses in the bilateral frontalis muscles through an unknown pathway. The latter neural circuit appeared to be engaged by the circumstances of aponeurotic blepharoptosis.

Cell bodies of the trigeminal proprioceptive neurons that transmit reflex contraction of the levator muscle are located in the mesencephalic trigeminal nucleus in rats.

Since the levator and frontalis muscles lack interior muscle spindles despite being antigravity mixed muscles to involuntarily sustain eyelid opening and eyebrow lifting, this study has proposed a hypothetical mechanism to compensate for this anatomical defect. The voluntary contraction of fast-twitch fibres of the levator muscle stretches the mechanoreceptors in Müller's muscle to evoke proprioception, which continuously induces reflex contraction of slow-twitch fibres of the levator and frontalis muscles. This study confirmed the presence of cell bodies of the trigeminal proprioceptive neurons that transmit reflex contraction of the levator and frontalis muscles. After confirming that severing the trigeminal proprioceptive fibres that innervate the mechanoreceptors in Müller's muscle induced ipsilateral eyelid ptosis, Fluorogold was applied as a tracer to the proximal stump of the trigeminal proprioceptive nerve in rats. Fluorogold labelled the cell bodies of the trigeminal proprioceptive neurons, not in any regions of the rat brain including the trigeminal ganglion, but in the ipsilateral mesencephalic trigeminal nucleus neighbouring the locus ceruleus. Some Fluorogold particles accumulated in the area of the locus ceruleus. The trigeminal proprioceptive neurons could be considered centrally displaced ganglion cells to transmit afferent signal from the mechanoreceptors in Müller's muscle to the mesencephalon, where they may be able to make excitatory synaptic connections with both the oculomotor neurons and the frontalis muscle motoneurons for the involuntary coordination of the eyelid and eyebrow activities, and potentially to the locus ceruleus.