Pathology and radiology correlation of idiopathic interstitial pneumonias.

By nature, idiopathic interstitial pneumonias have been diagnosed in a multidisciplinary manner. As classifications have been subject to significant refinement over the last decade, the importance of correlating clinical, radiologic, and pathologic information to arrive at a diagnosis, which will predict prognosis in any given patient, has become increasingly recognized. In 2013, the American Thoracic Society and European Respiratory Society updated the idiopathic interstitial pneumonias classification scheme, addressing the most recent updates in the field. The purpose of this review is to highlight the correlations between radiologic and pathologic findings in idiopathic interstitial pneumonias while using updated classification schemes and naming conventions.

[Case of oromandibular dystonia presenting with severe impairment of mouth-opening, and a marked effect by administration of baclofen].

We report a 67-year-old woman with idiopathic oromandibular dystonia (OMD). She could neither open the mouth nor take meals due to involuntarily strong mouth-closing. The movement of face, pharynx and tongue were normal, and she could open the mouth slightly when jaw and cheek were touched (sensory trick). Chvostek sign and Trousseau sign were negative, and opisthotonus was not recognized. The laboratory data including calcium, phosphorous and cerebrospinal fluid were within normal limits, head and cervical MRI, temporomandibular joints-Xp and needle electromyography were normal. The surface electromyography revealed that masseter and chin muscles contracted synchronously. This result meant dystonia around the mouth. The clinical course and physical examination did not support the diagnosis of tetanus, tetany or bulldog response. She was diagnosed as OMD. She had peroral administration of baclofen, because this drug is a GABA-derivative and acts as a muscular relaxant. Her clinical symptoms and dystonic pattern on the surface electromyography improved markedly after the administration. Baclofen is an effective drug for treatment of oromandibular dystonia.

[Spasmodic dysphonia in course of Meige's syndrome--case report]. / Przypadek dysfonii spastycznej, wystepujacej w przebiegu zespolu Meige'a.

INTRODUCTION: The Meige's syndrom is characterized by the presence of bilateral, symmetrical, dystonic cramp of face muscles or muscles of middle line of body, the respiratory muscles and muscles of throat. The etiology of Meige's syndrome is uncertain. The disorders of basal ganglia function and neurotransmitters' imbalance (dopamine and acetylocholine) can be with reason of pronouncement of symptoms presumably. MATERIAL AND METHOD: Our aim was to introduce a case of 71 years old patient in whom we diagnosed spasmodic dysphonia in course of Meige's Syndrom. Patient has been treated by 3 years with Botulin toxin. The spasmodic Dysphonia occurred after over 2 years from appearing of first syndrom's symptoms. RESULTS: Sonorous voice during rehabilitation was got during expressing syllables and short bisyllabic words. Patient stays still under phoniatric care. CONCLUSIONS: Patients with spasmodic dysphonia ought to be examined by a interdisciplinary medical team.

[Meige's syndrome or segmental craniocervical dystonia: terminology, history and contemporary view].

The term "Meige's syndrome" or "Breughel's syndrome" is used by specialists for the description of blepharospasm with the involuntary movements in the lower part of the face and/or masseter muscle. These eponyms "Meige's syndrome" and "Breughel's syndrome" make some mess in the terminology. The term "segmental craniocervical dystonia" joins different blepharospam-plus phenotypes and reflects contemporary perceptions about its genetic and pathophysiological community. Botulinotherapy is in fact the only way of symptomathic treatment of craniocervical dystonias.

Brueghel syndrome: its distinction from Meige syndrome.

A critical historical evaluation of the cranial dystonias supports the separation of the dystonia of the motor trigeminal nerve producing a widely opened mouth (Brueghel syndrome) from the more common facial dystonias with blepharospasm (Meige syndrome). In a patient with Brueghel syndrome, paroxysmal hyperpnea coincided with dystonic gaping; the finding of upbeating nystagmus suggests pontine localization in the pathogenesis of this rare disorder.

Sensory symptoms in cranial dystonia: a potential role in the etiology?

Cranial dystonia is normally considered as a pure movement disorder. Sensory symptoms have not received much attention, but we found ill-defined pain, discomfort, distortion of sensory modalities, 'phantom' kinetic or postural sensations in the orofacial areas subsequently involved by the dyskinesia in all of 11 consecutive patients, preceding by weeks or months the motor syndrome. Physicians were often mislead, initially making diagnoses such as trigeminal neuralgia, dental problems, sicca syndrome, chronic conjunctivitis, glossitis or stomatitis. The patients reported that the orofacial movements were at first willingly performed in order to decrease the discomfort which was felt in these facial areas before the movements finally escaped voluntary control and became socially disturbing. We suspect that the sensory symptoms, for which no objective substrate could be found, and which were always reported before and in the exact location of the subsequent dyskinesia, could be the earliest manifestation of an evolving process in cranial and perhaps other focal dystonias.

Postherpes simplex type 1 neuralgia simulating postherpetic neuralgia.

Patients with prodromal neuralgia associated with recurrent herpes simplex type 1 (HST1) infection and chronic facial pain following years of relapsing HST1 have been described. Chronic neuralgia following a single clinical HST1 infection and simulating postherpetic neuralgia has not been previously reported. Such a case is described: A 49-yr-old woman with a 2-mo history of oral-facial dyskinesia developed burning pain and hypersensitivity of the left side of the tongue, lower gum, and inner cheek, followed 1 day later by a vesicular rash in the same painful distribution. Viral cultures of the lesions identified HST1 but not herpes zoster. Cerebrospinal fluid analyses during the vesicular lesion stage and 1 mo later were normal with no viral growth. Oral and facial lesions resolved after 10 days; acyclovir was given for 3 wk. Brain and brainstem magnetic resonance imaging (MRI), electroencephalogram, and brainstem evoked potential studies were normal. Hyperesthesias, allodynia, and burning pain persisted despite nonsteroidal antiinflammatory agents, codeine and hydrocodone. Oral opioids were administered until sedation occurred, with no relief of pain. The burning pain and hyperesthesia resolved after the 16th day of amitriptyline use, 75 mg/day. A trial off amitriptyline 6 mo later resulted in recurrence of pain, and amitriptyline was restarted with good pain control. Post-HST1 neuralgia may simulate postherpetic neuralgia clinically, and painful symptoms may respond to amitriptyline.

Dry eye and Meige's syndrome.

AIMS: To determine the relation between dry eye and Meige's syndrome. METHODS: 325 patients with dry eye were divided into those responsive to topical and other forms of treatment (n = 276) and those who were not (n = 49). A neuropsychiatric examination was performed to check for Meige's syndrome in the latter group. RESULTS: Twenty eight (57%) of the treatment unresponsive patients were diagnosed with Meige's syndrome. CONCLUSIONS: There is a subgroup of patients with dry eye who do not respond to simple therapy. More than half of these patients have Meige's syndrome and need psychiatric, as well as ophthalmic, care.

Blepharospasm--patient's perspective.

It is difficult to determine which age group is hardest hit by BEB-Meige's: 30 to 45 years (lowest number although increasing), 45 to 60 years (largest group), or 60 to 75 years (second largest group). Each group feels it is hardest hit for different reasons.

Blepharospasm and Meige syndrome: a review of diagnostic, aetiological and treatment approaches.

No known pathophysiological mechanism can explain the majority of cases of blepharospasm, i.e. spasm of the orbicularis oculi muscle; it may also affect the lower face, neck and jaw--Meige syndrome. Only symptomatic treatment is possible, and surgery should be a last resort for severe cases. Much more clinical research will be required before promising behavioural interventions, including biofeedback, can be considered treatments of choice.

Acquired blepharoptosis.

A review is given of the aetiology and possible treatment of acquired (non-congenital), blepharoptosis, which is a common but not specific sign of neurological disease. The diagnostic categories of upper eyelid drooping are scheduled as (a) pseudo-ptosis due to a local process or overactivity of eye closure, including blepharospasm, and (b) true ptosis due to a paresis of the eyelid levators (m. tarsalis superior or m. levator palpebrae) or to a disinsertion of the m. levator palpebrae (aponeurotic ptosis). A paresis of the m. tarsalis is due to a lesion in the central, intermediate or peripheral neuron of the sympathetic chain and constitutes one of the components of Horner's syndrome. A paresis of the m. levator palpebrae may be due to a failure in central innervation, in oculomotor (n.III) function, in neuromuscular transmission or to a lesion in the muscle itself.

Meige's disease misdiagnosed as anxiety disorder.

A woman in her late 40s with a 5 year history of anxiety was treated with relaxation training and cognitive restructuring. Her anxiety was manifested by facial twitching, hand fidgeting, vocal tremor, loss of self-esteem, and depression. Therapy seemed to reduce motor symptoms and improve her self-esteem, confidence, and mood. Six months after the start of therapy the client was found to have Meige's Disease. Following treatment with botulinum toxin, motor symptoms disappeared. This case highlights the need for psychotherapists to be more aware of neurological and medical problems which may mimic psychological ones.

Idiopathic blepharospasm-oromandibular dystonia syndrome (Meige's syndrome) presenting as chronic temporomandibular joint dislocation.

A case of idiopathic blepharospasm-oromandibular dystonia (Meige's syndrome) is reported, presenting as chronic bilateral dislocation of the temporomandibular joints. The nature of the syndrome is discussed, together with the difficulties in diagnosis and management.

Meige's syndrome.

A case of Meige's syndrome is reported who presented with blepharospasm and oromandibular dystonia along with dislocation of jaw, due to severe dystonia. Such severe form of dystonia are very rare.

[Laryngeal dystonia--personal experience with botulin toxin treatment]. / Dystonia krtaniowa--wlasne doswiadczenia w leczeniu toksyna botuliowa.

Laryngeal dystonia is a focal dystonia occurring more often than it is diagnosed. Adductor type dystonia was described in 5 patients. In 3 cases it manifested itself as blepharospasm, which later developed into Meige's syndrome. The patients were treated with botulinum toxin A injections under EMG control administering 10 i.m. into laryngeal muscles on both sides with good and very good results. There were no serious side effects.

[A case of Meige's syndrome associated with post head trauma].

The pathogenesis of Meige's syndrome (MS) is controversial and has yet to be determined up to today. We studied a case of MS associated with post head trauma. The patient was a 52-year-old female. At the age of 46, she began to suffer from oro-lingual dystonia after head trauma induced by a traffic accident and the brief administration of neuroleptics to the delusion deteriorated the dystonia. She showed a wry appearance after 1 year and 6 months of the trauma and began to exhibit blepharospasms, oro-mandibular dystonia and cervical dystonia after 2 years and 3 months. For these symptoms her daily life became difficult. These symptoms were resistant to various drug therapies, although trihexyphenidyl relieved the symptoms transiently. Laboratory examinations and cranial MRI findings were normal. By surface electromyogram of ocular orbicular muscles, bilateral continuous discharge was observed. This patient was diagnosed as MS by clinical symptoms and surface electromyogram findings. It was inferred that the head trauma was associated with the development of MS. We discussed the pathogenesis of MS in the present case and it was speculated that MS was presented by a minute lesion of the brain stem which was produced at the time of the head trauma.

[Blepharospasm and Meige's syndrome--a contribution to it's pathogenesis]. / Kurcz powiek i zespól Meige'a--przyczynek do patogenezy.

Two cases with blepharospasm followed by Meige syndrome were described. In the first magnetic resonants revealed bilateral hypodensive foci in thalamus. During the few years general dystonia has been developed after several relapses of disease diagnosed as multiple infarcts followed by aphasia, hemiparesis and asynergia. In the other one, who died, loss of neurons in striatum, especially in caudate nuclei was found. It is concluded that blepharospasm could be a syndrome of different origin and only the phase of Meige syndrome.

[Posterior laryngeal stenosis in patient with Meige's Syndrome]. / Estenosis laríngea posterior en paciente con síndrome de Meige.

Meige's Syndrome is a combination of blepharospasm and oromandibular dystonia (cranial dystonia) that can affect the pharyngeal and respiratory musculature. We are reporting a case of a 61 year-woman with this diagnosis who presented a laryngeal stenosis due to sinequia on posterior commissure. The symptoms were an association of hoarseness and dyspnea with a clear movement in all pharyngo-laryngeal structures because of her illness. She was operated by Laser surgery with section of such sinequia and going on treatment in Neurology.

Methylphenidate-induced acute orofacial and extremity dyskinesia.

Methylphenidate is a short-acting stimulant. In this article, the authors report a 7-year-old male patient who presented with orofacial and limb dyskinesia after his first dose of methylphenidate treatment for a diagnosis of attention-deficit/hyperactivity disorder; he was also receiving sodium valproate treatment for epilepsy. Orofacial dyskinesia appeared 5 hours after methylphenidate administration, persisted for 10 hours, and had completely resolved within 2 days. Although limb dyskinesia after methylphenidate is a commonly reported side effect, to the authors' knowledge this is only the second reported case to develop both orofacial and limb dyskinesia in the acute period after the first dose of methylphenidate. This case is reported to emphasize the potential side effects of methylphenidate, individual differences in drug sensitivities, and drug-receptor interactions via different mechanisms.