Facial diplegia as a manifestation of neuroleptospirosis / Diplejía facial como manifestación de neuroleptospirosis

Abstract This case series describes the clinical and paraclinical findings in two young men with bilateral peripheral facial palsy or facial diplegia during the convalescent period of leptospirosis, who recovered neurologically without sequelae. This highlights the role of spirochetes in the development of an atypical and rare variant of Guillain-Barré syndrome. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.1947).

Resumen Esta serie de caso describe los hallazgos clínicos y paraclínicos observados en dos hombres jóvenes, con parálisis facial periférica bilateral o diplejía facial durante la fase de convalecencia de la leptospirosis, con recuperación neurológica sin secuela; resaltando así, el papel de la espiroqueta en el desarrollo de una variante atípica y poco frecuente del síndrome de Guillain-Barré. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.1947).

One-and-a-half syndrome with facial diplegia: A case report

One-and-a-half syndrome with facial diplegia, also referred to as the fifteen-and-a-half syndrome, is an extremely rare clinical entity caused by involvement of bilateral tegmentum of pons. Herein, we report a 52-year-old male who presented with one-and-a-half syndrome with left facial paralysis, which was consistent with the so-called eight-and-a-half syndrome. Brain magnetic resonance imaging showed pontine infarction. Five days after initiation of antiplatelet therapy, the patient developed right facial paralysis, a diagnosis of fifteen-and-a-half syndrome was made, repeat MR imaging revealed bilateral pontine tegmentum infarction. Fifteen-and-a-half syndrome is a newly proposed concept associated with pontine infarction. The clinicoradiological features of this specific disease are as yet unclear due to its extreme rarity. The current case would help advance the current understanding of the disease spectrum of pontine infarction.

Diplejía facial como variante inusual del síndrome de Guillain-Barré: primer caso reportado en Venezuela / Facial diplegia as unusual variant of Guillain-Barré syndrome: first case reported in Venezuela

El síndrome de Guillain-Barré (SGB) es la causa más común de parálisis generalizada aguda. El SGB es una polirradiculoneuropatia aguda inflamatoria desmielinizante que generalmente se presenta como una parálisis que inicia en miembros inferiores y luego progresa de forma ascendente y simétrica. El presente trabajo, tiene como objetivo, informar un caso de diplejía facial como manifestación inicial de SGB. Paciente masculino, 37 años de edad, diabético tipo 2, que luego de ocho días de haber padecido una sinusitis aguda, presentó de forma gradual, cefalea hemicraneana derecha, derramamiento salival y disartria. En la exploración neurológica se observó ausencia bilateral de los pliegues frontales, lagoftalmos bilateral acompañado de epífora, signo de Bell bilateral y derramamiento salival a través de ambas comisuras labiales. A las 48 horas de su ingreso hospitalario, presentó paresia en ambos miembros superiores. El estudio del líquido cefalorraquídeo reportó 1,1 células/mm³ representadas en su totalidad por linfocitos de aspecto normal, y proteínas totales 196,9mg/dL. La electromiografía fue compatible con polineuropatía desmielinizante aguda de predominio motor con mayor afectación facial. Con los hallazgos clínicos y paraclínicos se realizó el diagnóstico de SGB. Se inició tratamiento a base de plasmaféresis e inmunoglobulina endovenosa, con posterior mejoría de la clínica. La diplejía facial forma parte de las variantes regionales del SGB. A pesar que cerca del 60% de los pacientes con SGB presentan debilidad facial en el curso del trastorno, ésta habitualmente es precedida por debilidad en extremidades. El presente caso permite evidenciar que el SGB puede debutar clínicamente como una diplejía facial.

The Guillain-Barré syndrome (GBS) is the most common cause of acute generalized paralysis. GBS is an acute inflammatory demyelinating polyradiculoneuropathy. It usually presents as a paralysis that starts in the lower limbs and then progresses symmetrically upward. The present study reports a case of bilateral facial palsy as the initial manifestation of GBS. This is a report of a case of a 37-year-old male, diabetic, that eight days after having suffered acute sinusitis, gradually presented with right hemicranial headache, dysarthria and sialorrhea. The neurological examination disclosed the absence of the bilateral frontal folds, accompanied by epiphora, bilateral lagophthalmos, bilateral Bell sign and salivary drooling through both commissures of lips. At 48 hours after hospital admission the patient showed paresis in both upper limbs. The cerebrospinal fluid analysis reported 1.1cells/mm³, fully represented by lymphocytes of normal aspect and total proteins were 196.9 mg/dL. The electromyography was consistent with acute demyelinating polyneuropathy, with a predominant motor component and a major facial involvement. With the clinical and laboratory findings, a diagnosis of GBS was established. Treatment was started with plasmapheresis and intravenous immunoglobulin, with the subsequent improvement of the clinic. The facial diplegia is part of the regional variants of GBS. Although about 60% of GBS patients present with facial weakness, it is usually preceded by weakness in the limbs. This case makes evident that GBS may present clinically as a facial diplegia.

Facial diplegia: etiology, clinical manifestations, and diagnostic evaluation / Diplegia facial: etiologia, manifestações clínicas, e avaliação diagnóstica

ABSTRACT Objective Facial diplegia (FD) is a rare neurological manifestation with diverse causes. This article aims to systematically evaluate the etiology, diagnostic evaluation and treatment of FD. Method The study was performed retrospectively and included 17 patients with a diagnosis of FD. Results Patients were diagnosed with Guillain-Barré syndrome (GBS) (11), Bickerstaff’s brainstem encephalitis (1), neurosarcoidosis (1), non-Hodgkin’s Lymphoma (1), tuberculous meningitis (1) herpes simplex reactivation (1) and idiopathic (1). In addition, two patients had developed FD during pregnancy. Conclusion Facial diplegia is an ominous symptom with widely varying causes that requires careful investigation.

RESUMO Objetivo Diplegia facial (DF) é uma manifestação neurológica rara proveniente de diferentes causas. Este artigo visa avaliar sistematicamente a etiologia, avaliação diagnóstica e tratamento de DF. Método O estudo foi retrospectivo e incluiu 17 pacientes com diagnóstico de FD. Resultados Os pacientes foram diagnosticados como casos de síndrome de Guillain-Barré (SGB) (11), encefalite de tronco de Bickerstaff (1), neurosarcoidose (1), linfoma não-Hodgkin’s (1), meningite tuberculosa (1) reativação de herpes simplex (1) e causa idiopática (1). Além disto, duas pacientes haviam desenvolvido DF durante a gestação. Conclusão Diplegia facial é uma manifestação com diversas causas que requer investigação cuidadosa.

Diplejia facial una variante del síndrome de guillain barre / Facial diplegia a variant of guillain barre syndrome / Diplegia facial uma variante da síndrome de guillain-barre

El Síndrome de Guillain Barre es una polineuropatía autoinmune que causa desmielinización motora y sensitiva, frecuentemente con antecedente de una infección. El diagnóstico se realiza mediante sospecha clínica, aunque el líquido cefalorraquídeo y estudios electrodiagnósticos ayudan a soportarlo. El Médico debe estar familiarizado con las variantes clínicas tales como la diplejía facial para hacer un diagnóstico preciso. La inmunoglobulina intravenosa y la plasmaferesis son tratamientos eficaces. El cuidado de soporte durante y después de la hospitalización es crucial.

Guillain Barre Syndrome is an autoimmune polyneuropathy that causes motor and sensory demyelination, often with a history of infection. The diagnosis is made by clinical suspicion, although the cerebrospinal fluid and electrodiagnostic studies help support it. The Physician should be familiar with the clinical variants such as facial diplegia and make an accurate diagnosis. IVIG and plasmapheresis are effective treatments. Supportive care during and after hospitalization is crucial.

A síndrome de Guillain Barre é uma polineuropatia autoimune que provoca desmielinização motora e sensitiva, frequentemente com antecedentes de infecção. O diagnostico é realizado através de suspeita clinica, embora o líquido cefalorraquidiano e estudos eletrodiagnósticos ajudam a apoiá-lo. O médico deve estar familiarizado com as variantes clínicas tais como a diplegia facial para estabelecer um diagnóstico preciso. A imunoglobulina intravenosa e a plasmaférese são tratamentos eficazes. O tratamento de suporte durante e após a hospitalização é crucial.

Diplejía facial con parestesias: Una variante atípica del síndrome de Guillain-Barré

El síndrome de Guillain-Barré es una enfermedad desmielinizante aguda con una forma clásica que se presenta con debilidad muscular y ausencia de reflejos. Existen múltiples variantes y formas atípicas de la enfermedad, entre otras la diplejía facial con parestesias. Asimismo, la ausencia de reflejos en este síndrome es característico pero no constante, ya que en un 10% de los pacientes los reflejos están presentes. Se presenta aquí el caso de una mujer de 33 años con paresia facial bilateral, parestesias y debilidad de miembros inferiores e hiperreflexia, una forma de presentación infrecuente de este síndrome.

Guillain-Barré syndrome is an acute demyelinating disease which presents in a classic form with muscular weakness and the lack of reflexes. There are multiple variations and atypical forms of the disease, being facial diplegia with paresthesia one of them. Also, the absence of reflexes in this syndrome is typical but not constant, since 10% of patients present reflexes. We describe a case of atypical presentation with bilateral facial palsy, paresthesia, brisk reflexes and weakness in the lower limbs in a 33 year old woman.

Acute Inflammatory Demyelinating Polyradiculoneuropathy following Plasmodium vivax malaria- a case report.

Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) is usually preceded by infection with certain bacteria and viruses. Parasitic infection has rarely been reported as a causal factor for AIDP. Neurological manifestations following malaria is commonly seen with P. falciparum. There are only few case reports of Guillain–Barré Syndrome or facial diplegia following P. vivax infection. Here we are reporting a patient who developed AIDP and facial diplegia within two weeks following successful treatment of P. vivax infection.

A rare case of Moebius sequence.

We report a case of an 18-year-old male who presented with watering and inability to close the left eye completely since 6 months and inability to move both eyes outward and to close the mouth since childhood. Ocular, facial, and systemic examination revealed that the patient had bilateral complete lateral rectus and bilateral incomplete medial rectus palsy, left-sided facial nerve paralysis, thickening of lower lip and inability to close the mouth, along with other common musculoskeletal abnormalities. This is a typical presentation of Moebius syndrome which is a very rare congenital neurological disorder characterized by bilateral facial and abducens nerve paralysis. This patient had bilateral incomplete medial rectus palsy which is suggestive of the presence of horizontal gaze palsy or occulomotor nerve involvement as a component of Moebius sequence.

Reporte de caso clínico: síndrome de Moebius asociado al uso de misoprostol en el embarazo / Clinical case report: Moebius syndrome associated with the use of misoprostol in pregnancy

El síndrome de Moebius conocido también como diplejia facial congénita o agenesia nuclear, es una enfermedad neurológica congénita poco frecuente en la que se ven afectados los núcleos de origen de los pares craneales VI y VII, impidiendo su total desarrollo. Aunque su etiología es idiopática se asocia al uso de sustancias teratógenas, tales como cocaína, talidomida, misoprostol, etc. Se describe el caso clínico de un paciente de 11 meses de edad cuya madre usó tabletas de misoprostol por vía vaginal (400 mcg),durante el primer trimestre de gestación, con fines abortivos, presentando al nacimiento defectos congénitos. El misoprostol es un análogo de la prostaglandina E1 y fue creado para el tratamiento de la úlcera gástrica, pero, además posee acción uterotónica y tiene capacidad de maduración del cuello uterino. Si se lo usa al final de la gestación colabora como buen inductor del parto, no siendo así en el primer trimestre de gestación, donde los efectos son devastadores provocando un aborto, o en su defecto, genera déficit en la irrigación sanguínea, afectando el desarrollo embriológico del nuevo ser. En países donde el aborto no es legal, el misoprostol se emplea para la interrupción del embarazo, sin embargo la infrecuente supervisión profesional, llevan a que algunos de los embarazos continúen produciendo exposición prenatal al misoprostol, lo cual está asociado con la ocurrencia de anomalías congénitas. El presente trabajo demuestra la injerencia del misoprostol como agente teratógeno durante el primer trimestre de embarazo y posible inductor al desarrollo del síndrome de Moebius.

The Moebius syndrome, also known as congenital facial diplegia or nuclear agenesis, is a rare congenital neurological disorder which affects the nuclei of origin of cranial nerves VI and VII, preventing their full development. Although etiology is idiopathic, it is associated with the use of teratogenic substances such as cocaine, thalidomide, misoprostol, etc. The clinical case of an 11-month old patient is described, whose mother used misoprostol tablets vaginally (400 mcg) during the first trimester of pregnancy with abortive purposes, presenting birth defects. Misoprostol is a prostalglandin E1 analogue and was created for the treatment of gastric ulcer, but also has an uterotonic action and the capacity to ripen the cervix. If it is used at the end of the pregnancy, it works as a good inducer of birth, not so in the first trimester of pregnancy, where the effects are devastating causing an abortion, or otherwise, creating deficits in blood supply, affecting the embryological development of the new being. In countries where abortion is illegal, misoprostol is used for the termination of pregnancy, but the lack of professional supervision causes some of the pregnancies to continue, producing prenatal exposure to misoprostol, which is associated with the occurrence of congenital anomalies. This study demonstrates the interference of misoprostol as a teratogen in the first trimester of pregnancy and as thepossible inducer of the development of the Moebius syndrome

Facial Diplegia in Plasmodium vivax Malaria

BACKGROUND: Facial diplegia has diverse etiologies, including viral and bacterial infections such as diphtheria, syphilis and Lyme disease, and also protozoal infection in very rarely cases. CASE REPORT: A 20-year-old male patient was admitted to our hospital due to bilateral weakness of the upper and lower facial muscles. Examination revealed that the patient had a facial diplegia of the peripheral type. A peripheral blood smear demonstrated the presence of the asexual trophozoite stage of Plasmodium vivax with ring-form trophozoites, which led to a diagnosis of malaria. A serum work-up revealed increased IgG titers of antibodies to myelin-associated glycoprotein and ganglioside GD1b. The patient was administered antimalarial treatment, 1 week after which he showed signs of recovery. To our knowledge, this is the first case of facial diplegia after malaria infection, providing evidence that the mechanism underlying the condition is related to immune-mediated disease. CONCLUSIONS: Facial diplegia can manifest after P. vivax infection.

Aspectos odontológicos en paciente portador del síndrome de Mobius: relato de caso / Dental aspects of a patient with Mobius syndrome: case report

El síndrome de Mobius se caracteriza por la parálisis congénita y no progresiva de los nervios craneanos facial y abducentes cuyas manifestaciones clínicas principales son la apariencia facial estática y poco expresiva, el estrabismo bilateral convergente y la hipoplasia de miembros, entre otras. En la cavidad bucal puede observarse micrognacia, implantación heterotrófica de la lengua, anquiloglosia, úvula bífida, fisura palatina y anomalías dentales. La etiología del síndrome de Mobius es poco conocida y algunos relatos de la literatura señalan, como la hipótesis más probable, una isquemia fetal transitoria, durante el período de formación de los núcleos craneanos. Los posibles factores causales de esta isquemia serian los de orden ambiental, los disturbios fisiopatológicos y genéticos, o el uso ilícito de drogas como el Misoprostol, durante la gestación. El presente trabajo relata el tratamiento odontológico realizado en una paciente portadora de este síndrome enfatizando las particularidades observadas durante la atención, en función de las características propias de esa entidad clínica

Mobius syndrome is characterized by congenital and non-progressive paralysis of the facial and abducent cranial nerves. The principal manifestations of this syndrome are lack of facial expression, convergent bilateral strabismus and hypoplastic members. The oral findings include micrognatia, heterotrophic implantation of the tongue, ankyloglossia, bifid uvula, cleft palate and dental anomalies. The etiology of Mobius syndrome is unknown and some reports relate, as a main cause, the transitory fetal ischemia during the formation of cranial nucleus. The factors that lead to this ischemia are from ambient, physiopathological or genetic disturbs and use of drugs like Misoprostol during the pregnancy. This paper reports the dental treatment of a female patient with Mobius syndrome, emphasizing the peculiarities observed due to the characteristics of this clinical entity

A Case of Regional Variant of Guillain-Barre Syndrome

Neurologists are occasionally confronted with patients who have unique symptoms of bilateral but regional weaknesses that do not conform to the typical case with Guillain-Barre syndrome (GBS). Acute facial diplegia is a very uncommon neurologic manifestation that can be the presenting symptom in a wide range of diseases. We describe a 32-year-old male patient with acute facial diplegia and distal limb paresthesias without diminished reflexes. His neurophysiologic studies, CSF albuminocytologic dissociation and the clinical course are in keeping with a regional variant of GBS. The absence of hyporeflexia does not necessarily exclude the diagnosis of a GBS variant.