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Familial Recurrent Peripheral Facial Palsy


Presentation

  • It might rarely present with bilateral facial palsy. Case Report A 21 year old male patient presented with swelling of right half of the face since 10 months. There was inability to close both the eyes since 8 months.[bhj.org.in]
  • Bilateral facial palsy is much less frequent and occurs in less than 2% of patients presenting with an acute facial palsy ( Table 70–2 ). Bilateral involvement typically reflects a systemic disorder with multiple manifestations.[mhmedical.com]
  • Characteristic features Acute LMN palsy Acute LMN palsy can present at any age but is most frequently seen at age 15-60 years, affecting both sexes equally.[patient.info]
  • Página 51 - Selective IgA deficiency: presentation of 30 cases and a review of the literature. ‎ Página 188 - Dudding, BA, Gorlin, RJ, and Langer, LO: The oto-palato-digital syndrome.[books.google.es]
  • SOD1 (A4V)-mediated ALS presenting with lower motor neuron facial diplegia and unilateral vocal cord paralysis . Muscle Nerve 2009 ; 40 : 880 –2 46 Walling , AD . Bell's palsy in pregnancy and the puerperium .[cambridge.org]
Physician
  • As recurrent peripheral facial palsies carry a relatively poor prognosis, the physician should be alerted to this possibility in any child who has an idiopathic peripheral facial palsy and a positive family history.[ncbi.nlm.nih.gov]
  • In 2007 he received the Distinguished Physician Award from the American Association of Neuromuscular and Electrodiagnostic Medicine. Lahey Clinic’s Medical Staff Association recognized Dr.[books.google.com]
  • […] credited the Persian physician Ibn Sina (980–1037) for describing this condition before him.[en.wikipedia.org]
  • […] the Persian physician Ibn Sina (980–1037) for describing this condition before him.[ipfs.io]
  • Table 2 lists the investigations that may aid the physician in the diagnosis of facial paralysis.[primarypsychiatry.com]
Recurrent Peripheral Facial Palsy
  • As recurrent peripheral facial palsies carry a relatively poor prognosis, the physician should be alerted to this possibility in any child who has an idiopathic peripheral facial palsy and a positive family history.[ncbi.nlm.nih.gov]
  • V. 2011 6 Familial recurrent peripheral facial palsy. ( 6336357 ) Cole M. 1984 7 Familial recurrent peripheral facial palsy.[malacards.org]
  • Resumo: Recurrent peripheral facial palsy (PFP) is not uncommon and it is reported in 3 to 15% of idiopathic PFPs.[comum.rcaap.pt]
  • Familial recurrent peripheral facial palsy. Observations of the pediatric population. Arch Neurol. 1981 Jul; 38(7):463-4. PMID: 7247773. Read at: PubMed View all profiles[bumc.bu.edu]
  • Auerbach SH, Depiero TJ, Mejlszenkier J (1981) Familial recurrent peripheral facial palsy. Observations of the pediatric population. Arch Neurol 38: 463–464 Google Scholar 9.[link.springer.com]
Pharyngitis
  • Articles in this issue include Otitis Externa; Disease of the Internal Ear; Hearing Loss; Dizziness and Vertigo; Rhinitis; Sinusitis; Epistaxis; Laryngitis; Pharyngitis; Mouth Disease; and Neurological Syndromes pertaining to the Head and Neck.[books.google.de]
  • […] coloboma-cleft lip and palate-intellectual disability VACTERL with hydrocephalus Van den Bosch syndrome Variant of Guillain-Barré syndrome Vici syndrome Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome Vocal cord and pharyngeal[se-atlas.de]
Rhinitis
  • Articles in this issue include Otitis Externa; Disease of the Internal Ear; Hearing Loss; Dizziness and Vertigo; Rhinitis; Sinusitis; Epistaxis; Laryngitis; Pharyngitis; Mouth Disease; and Neurological Syndromes pertaining to the Head and Neck.[books.google.de]
Lacrimation
  • If the mastoid segment is involved, as with tympanic lesions, hearing and balance may be affected, and lacrimation is preserved.[ispub.com]
  • Also aberrant parasympathetic re-innervation may cause symptoms such as gustatory lacrimation ('crocodile tears'). Symptoms can be helped by subcutaneous or intramuscular injections of botulinum toxin. [ 25 ][patient.info]
  • […] nerve V Taste: To nucleus tractus solitarius Preganglionic parasympathetics Origin: Superior salivatory nucleus Innervate: Submandibular & Pterygopalatine ganglion Branches Greater & Lesser petrosal To: Pterygopalatine & Otic ganglia Parasympathetic Lacrimation[neuromuscular.wustl.edu]
  • It enters the apex of the petrous part of the temporal bone with the eight cranial nerve and gives branches to the lacrimal gland, and stapedius muscle in the inner ear, sensation to the auricular skin, and the sublingual/submandibular salivary glands[primarypsychiatry.com]
  • This is thought to be due to faulty regeneration of the facial nerve, a branch of which controls the lacrimal and salivary glands. Gustatorial sweating can also occur.[ipfs.io]
Myopathy
  • Distal myopathy with anterior tibial onset Distal myopathy with early respiratory muscle involvement Distal myopathy with posterior leg and anterior hand involvement Distal myopathy, Welander type Distal myotilinopathy Distal nebulin myopathy Distal[se-atlas.de]
  • […] research laboratories of Boston Children’s and other institutions, focuses on the description of novel mutations in patients with neuromuscular diseases and the application of new technologies to explore the pathogenesis and treatment of congenital myopathies[books.google.com]
  • Glycogen storage disease type XI Glycogen storage disease type XII Glycogen storage disease type XV Glycogen storage disease with hypertrophic cardiomyopathy Glycogen storage disease with severe cardiomyopathy due to glycogenin deficiency Glycogen storage myopathy[orpha.net]
  • […] syndrome Möbius syndrome & Congenital facial paresis An-α-lipoproteinemia (Tangier) Infection Leprosy HIV infection : May occur before seroconversion Lyme disease Guillain Barré : Variant Other peripheral causes Motor neuron disorders Myasthenia gravis Myopathies[neuromuscular.wustl.edu]
  • Fulminant form of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes: A diagnostic challenge. J Med Cases 2011;2(2):87-90. Akaza M, Kanouchi T, Inaba A, Numasawa Y, Irioka T, Mizusawa H, Yokota T.[tmd.ac.jp]
Hyperacusis
  • Hyperacusis and a decrease in tearing and in salivation with loss of taste can be detected.[ispub.com]
  • ; Dysgeusia Signs Facial weakness All branches of nerve: Upper & Lower Unilateral Degree: Partial (30%); Complete (70%) Side: Right in 63% Stapedius dysfunction (33%): Hyperacusis Lacrimation: Mildly affected in some patients Taste No clinically significant[neuromuscular.wustl.edu]
  • There may be hyperacusis. Patients with lesions proximal to the geniculate ganglion may be unable to produce tears and have loss of taste.[patient.info]
  • Old age, post-auricular pain, dysgeusia, hyperacusis, dry eye and delayed onset of recovery affect a bad prognosis. Enog, TMS and eye blink reflex response are the other useful prognostic indices.[jpma.org.pk]
  • […] may help in localizing the site of the lesion along the course of the facial nerve include lacrimation, which can be quantified using the Schirmer tear test (dry eyes indicate involvement of the facial nerve at or proximal to the geniculate ganglion), hyperacusis[primarypsychiatry.com]
Hearing Impairment
  • impairment Niemann-Pick disease type A Niemann-Pick disease type C Niemann-Pick disease type C, adult neurologic onset Niemann-Pick disease type C, juvenile neurologic onset Niemann-Pick disease type C, late infantile neurologic onset Niemann-Pick disease[se-atlas.de]
Epistaxis
  • Articles in this issue include Otitis Externa; Disease of the Internal Ear; Hearing Loss; Dizziness and Vertigo; Rhinitis; Sinusitis; Epistaxis; Laryngitis; Pharyngitis; Mouth Disease; and Neurological Syndromes pertaining to the Head and Neck.[books.google.de]
  • Hyperthyroidism Pregnancy Hypertension Alcoholic neuropathy Bulbopontine paralysis Oculopharyngeal muscular dystrophy Vascular Anomalous sigmoid sinus Benign intracranial hypertension Intratemporal aneurysm of internal carotid artery Embolization for epistaxis[mhmedical.com]
  • Bilateral facial nerve paralysis following arterial embolization for epistaxis . Otolaryngol Head Neck Surg 1983 ; 91 : 299 – 303 39 Hattori , T , Schlagenhauff , RE . Bilateral facial palsy: occurrence with diabetes mellitus .[cambridge.org]
Vertigo
  • Articles in this issue include Otitis Externa; Disease of the Internal Ear; Hearing Loss; Dizziness and Vertigo; Rhinitis; Sinusitis; Epistaxis; Laryngitis; Pharyngitis; Mouth Disease; and Neurological Syndromes pertaining to the Head and Neck.[books.google.de]
  • There was no history of ear discharge, ear pain, tinnitus or vertigo. There was no past history of facial weakness. There was no family history of the cough, fever, weight loss, tuberculosis, diabetes, and hypertension.[bhj.org.in]
  • It can include deafness and vertigo and other cranial nerves can be affected.[patient.info]
  • Special attention must be paid to examining the central nervous system for evidence of brainstem and other cranial nerves involvement (eg, deafness, vertigo, dizziness, vomiting, difficulty swallowing).[primarypsychiatry.com]
Dizziness
  • Articles in this issue include Otitis Externa; Disease of the Internal Ear; Hearing Loss; Dizziness and Vertigo; Rhinitis; Sinusitis; Epistaxis; Laryngitis; Pharyngitis; Mouth Disease; and Neurological Syndromes pertaining to the Head and Neck.[books.google.de]
  • Special attention must be paid to examining the central nervous system for evidence of brainstem and other cranial nerves involvement (eg, deafness, vertigo, dizziness, vomiting, difficulty swallowing).[primarypsychiatry.com]
Myelopathy
  • ほぼ全長にわたる脊髄病変と肺病変をともなった急速進行性HTLV-1-associated myelopathy (HAM) の1例. 臨床神経学 2011; 51(2): 130-133. 三宅敦子、山口実菜、杉山美帆、神山隆治、泉山肇、入岡隆、水澤英洋、平田結喜緒. 意識障害にて入院中に発症したと考えられる劇症1型糖尿病の1例. 糖尿病. 2011; 54(2):107-111 三木一徳,石原正一郎,山浦千春,沼沢祥行,嶋田謙,新谷周三. [tmd.ac.jp]
Involuntary Movements
  • Cerebrovascular accidents usually weaken voluntary movement, often sparing involuntary movements (eg, spontaneous smiling).[patient.info]

Workup

  • Figure 3 Figure 4 Despite a thorough workup, 60 to 75 percent of all cases of facial paralysis are of unknown etiology and are called Bell's palsy 1, 7, 15, 16, 17, 18.[ispub.com]

Treatment

  • Apply the latest treatment options in pediatric care with new chapters on pediatric sleep disorders, pediatric infectious disease, and evaluation and management of the infant airway.[books.google.com]
  • Factors and Bell's Palsy Unknown status NCT01377766 25 Prednisone and Acupuncture for the Treatment of Facial Neuritis: a Multiple Center, CER in China Unknown status NCT01201642 Not Applicable Prednisolone 26 Quantification, Analysis and Simulation[malacards.org]
  • Facial nerve palsy in children: clinical aspects of diagnosis and treatment. Acta Otolaryngol. 1994;(suppl 511):150-152. 9. Prescott CA. Idiopathic facial nerve palsy in children and the effect of treatment with steroids.[primarypsychiatry.com]

Prognosis

  • As recurrent peripheral facial palsies carry a relatively poor prognosis, the physician should be alerted to this possibility in any child who has an idiopathic peripheral facial palsy and a positive family history.[ncbi.nlm.nih.gov]
  • Hauser WA, Karnes WE, Annis J, Kurland LT (1971) Incidence and prognosis of Bell's palsy in the population of Rochester, Minnesota. Mayo Clin Proc 46: 258–264 Google Scholar 3.[link.springer.com]
  • […] age group, the prognosis for full recovery is excellent in approximately 95% of cases.[primarypsychiatry.com]
  • Prognosis Predicting prognosis of Bell’s palsy is difficult, particularly in the early phase of the disease.[jpma.org.pk]
  • […] is variable but usually good. [ 23 ] Prognosis [ 14, 24 ] 85% improve spontaneously within three weeks of its onset. 71% recover fully. 16% have significant sequelae, 5% severe: Facial asymmetry Gustatory lacrimation Inadequate lid closure Brow ptosis[patient.info]

Etiology

  • However, the first familial occurrence was found in 1887, hence hereditary factors have been considered to play a role in the etiology of the disease.[hnppwellbeing.com]
  • Other etiologies include intracranial compressive tumors, parotid gland tumors or inflammation, recurrent otitis media, head trauma, multiple sclerosis, sarcoidosis, tuberculosis, Lyme disease, HIV, and the rare Melkersson-Rosenthal Syndrome (MRS).[comum.rcaap.pt]
  • Etiology, frequency, prognosis. J Neurol 216: 119–125 Google Scholar 7. Waterman GA (1909) Facial paralysis: a study of three hundred and thirty-five cases. J Nerv Ment Dis 36: 65–76 Google Scholar 8.[link.springer.com]
  • Common etiologies include acoustic neuromas 14 and meningiomas. In the labyrinthine segment, facial palsy is seen with ipsilateral hearing loss and vestibular weakness.[ispub.com]
  • Facial palsy: unusual etiology . Laryngoscope 1977 ; 87 : 26 – 34 61 May , M , Hughes , GB . Facial nerve disorders: update 1987 . Am J Otol 1987 ; 8 : 167 –80 62 Schwaber , MK , Larson , TC , Zealear , DL , Creasy , J .[cambridge.org]

Epidemiology

  • Epidemiology of Bell's palsy in Isfahan, Iran. Neurosciences (Riyadh). 2009;14(2):186-7. PubMed PMID: 21048610. Adour KK, Byl FM, Hilsinger RL, Jr., Kahn ZM, Sheldon MI. The true nature of Bell's palsy: analysis of 1,000 consecutive patients.[journals.sbmu.ac.ir]
  • […] tightly together: Difficulty keeping food in mouth Facial muscle atrophy (Late) Electrophysiology EMG Denervation Synkinesis: Late Blink reflex Abnormal ipsilateral R1 (early, disynaptic) R2 (late multisynaptic) responses Synkinesis (Late) Bell's Palsy 8 Epidemiology[neuromuscular.wustl.edu]
  • D. (2002) Taxonomy and epidemiology of spinal cord injury pain. In: Yezierski, R.P. and Burchiel, K. (eds.)[shiga-med.ac.jp]
  • The epidemiology and treatment of Bell’s palsy in the UK. Eur J Neurol. 2002;9:63-67. 2. Bell C. On the nerves, giving an account of some experiments on their structure and functions which lead to a new arrangement of the system.[primarypsychiatry.com]
  • Epidemiology The number of new cases of Bell's palsy is about 20 per 100,000 population per year. [42] The rate increases with age. [42] Bell’s palsy affects about 40,000 people in the United States every year.[ipfs.io]
Sex distribution
Age distribution

Pathophysiology

  • It is the labyrinthine portion where it is most likely that a vicious cycle of edema from infection, vascular compromise, and ischemia, thought to be the pathophysiologic process in Bell's palsy, can occur.[ispub.com]
  • Pathophysiology of Bell’s Palsy Many patients with Bell’s palsy have been found to have rising antibody titers to herpes simplex virus. 31 Different pathophysiological processes have been suggested, 31 including active viral invasion or immune processes[primarypsychiatry.com]
  • Saito 0, Electrophysiological study on pathophysiology of Bell’s palsy -Distribution of nerve conduction velocities in facial nerve with collision method, Nippon-Jibiinkoka-Gakkai-Kaiho, 1991 ;94(7):906-14. 8. Sugita, 1.[jpma.org.pk]
  • This may lead to an improved understanding of the pathophysiologic mechanisms in febrile seizures and eventually in other seizure disorders. Febrile seizures are known to aggregate in families.[mafiadoc.com]
  • […] produce facial palsy. [19] Sometimes the facial palsy occurs at the same time as the classic erythema migrans rash. [19] Other times, it occurs later. [19] In areas where Lyme disease is common, it may be the cause of facial palsy in half of cases. [19] Pathophysiology[ipfs.io]

Prevention

  • […] treatment to prevent febrile seizure recurrences.[mafiadoc.com]
  • Paralysis caused by neuritis frequently disappears when the disorder causing it is corrected. paralysis of accommodation paralysis of the ciliary muscles of the eye so as to prevent accommodation. ascending paralysis spinal paralysis that progresses upward[medical-dictionary.thefreedictionary.com]
  • Centers for Disease Control and Prevention. Retrieved April 12, 2019. "Lyme disease rashes and look-alikes". Lyme Disease. Centers for Disease Control and Prevention. Retrieved April 18, 2019. Wright WF, Riedel DJ, Talwani R, Gilliam BL (June 2012).[en.wikipedia.org]
  • Eye care: Ophthalmologists play an important role in preventing irreversible blindness from corneal exposure. This may be successfully achieved by using lubricating drops hourly and eye ointment at night an eye patch.[patient.info]
  • Not yet recruiting NCT03781700 Phase 4 Prednisolone;Placebo Oral Tablet 5 Reinnervation of Facial Muscles After Lengthening Temporalis Myoplasty Terminated NCT01833221 Phase 4 injection of 1% lidocaine, 2mL for facial nerve block 6 Corticosteroids in Prevention[malacards.org]

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