title: Oculopalatal tremor, facial myokymia and truncal ataxia in a patient with neurosarcoidosis.
abstract: Symptomatic palatal tremor (SPT) is the result of a structural lesion, in the form of stroke, trauma or demyelinating disease. SPT is due to contractions of the levator veli palatini and can be accompanied by simultaneous movements of the facial and ocular muscles. Facial myokymia (FM) is a persistent quivering of the facial muscles. FM is usually encountered with conditions involving the pontine tegmentum. We report, to our knowledge, the first patient with neurosarcoidosis with simultaneous SPT and FM. A 49-year-old African American woman, with non-caseating granulomas in a paratracheal lymph node biopsy, presented with progressive gait disturbances for the last 3 years. Neurological examination revealed ataxic speech, bilateral rotatory nystagmus, myokymia of the chin and perioral muscles, palatal tremor without ear click and marked truncal ataxia. MRI demonstrated a lesion involving the facial nucleus and the right middle cerebellar peduncle. Based on exclusion of alternative etiologies, a diagnosis of neurosarcoidosis was made and the patient was started on methotrexate for 9 months, with minimal improvement. She was then switched to intravenous infliximab without major adverse events. The patient's speech and gait ataxia improved and follow up MRI demonstrated resolution of the enhancing lesions. To our knowledge, this is the first reported case of the combination of palatal tremor and FM due to neurosarcoidosis. Methotrexate may fail to produce clinical or radiographic response in up to 39% of patients. Tumor necrosis factor-α inhibitors, such as infliximab, should be considered in refractory cases.
authors.affiliation: Department of Neurology, Henry Ford Hospital, 6777 West Maple Road, West Bloomfield, Detroit, MI 48322, USA. Electronic address: csidiro1@hfhs.org.
authors.forename: Christos
authors.initials: C
authors.lastname: Sidiropoulos
authors.affiliation: Department of Neurology, Henry Ford Hospital, 6777 West Maple Road, West Bloomfield, Detroit, MI 48322, USA.
authors.forename: Naganand
authors.initials: N
authors.lastname: Sripathi
authors.affiliation: Department of Neurology, Henry Ford Hospital, 6777 West Maple Road, West Bloomfield, Detroit, MI 48322, USA.
authors.forename: Khalil
authors.initials: K
authors.lastname: Nasrallah
authors.affiliation: Department of Neurology, Henry Ford Hospital, 6777 West Maple Road, West Bloomfield, Detroit, MI 48322, USA; Wayne State University, School of Medicine, Detroit, MI, USA.
authors.forename: Panayiotis
authors.initials: P
authors.lastname: Mitsias
mesh_terms: D001741:Black or African American; D001259:Ataxia / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D002493:Central Nervous System Diseases / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D065837:Middle Cerebellar Peduncle / Q000473:pathology / Q000503:physiopathology; D010156:Palatal Muscles / Q000503:physiopathology; D012507:Sarcoidosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D014202:Tremor / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology
keywords: Infliximab; Myokymia; Neurosarcoidosis; Oculopalatal tremor