title: Steroid-responsive intracranial germinoma presenting as Holmes' tremor: importance of a tissue diagnosis.
abstract: Holmes' tremor (rubral or midbrain outflow tremor) refers to a hyperkinetic movement disorder characterized by mild resting and more severe postural and action tremor often with associated brainstem symptoms, dystonia and cerebellar deficits. This syndrome should prompt lesional evaluation with neuroimaging focused on the dorsal midbrain, cerebellar outflow tracts, and thalamus. Herein we report a 26-year-old previously healthy male who presented with 4 years of progressive horizontal diplopia, right Parinaud syndrome, and appendicular ataxia. Neuroimaging revealed a right dorsal midbrain enhancing lesion which completely resolved with intravenous methylprednisolone prompting a diagnosis of neuroinflammatory syndrome. Subsequent clinical and radiographic evaluations, however, revealed steadily progressive left dorsal midbrain syndrome with an expansile enhancing lesion which culminated 4 years from symptom onset with a right upper extremity low-frequency rest, postural and action tremor, ataxic dysarthria, and mild right dystonia with dysdiadochokinesia. Uncomplicated brainstem biopsy confirmed intracranial germinoma and the patient underwent definitive radiation therapy with dramatic radiographic response and partial clinical improvement. This case, which to our knowledge is only the second report of intracranial germinoma presenting as Holmes' tremor, highlights the critical importance of definitive tissue diagnosis in the evaluation of lesional brainstem pathology presenting as Holmes' tremor. Steroid responsiveness can be seen in non-inflammatory pathology including intracranial germinoma. Prompt evaluation and appropriate treatment are important as Holmes' tremor responds poorly to symptomatic therapies and response to radiation therapy is favorable for germinomas.
authors: Department of Neurology, Johns Hopkins Hospital, David H. Koch Cancer Research Building II, 1550 Orleans Street, Room 1M16, Baltimore, MD 21287, USA. Electronic address: rstrowd1@jhmi.edu.
authors: Roy E
authors: RE
authors: Strowd
authors: Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA.
authors: Peter
authors: P
authors: Burger
authors: Department of Oncology, The Johns Hopkins Hospital, Baltimore, MD, USA.
authors: Matthias
authors: M
authors: Holdhoff
authors: Department of Radiation Oncology, The Johns Hopkins Hospital, Baltimore, MD, USA.
authors: Lawrence
authors: L
authors: Kleinberg
authors: Department of Neurology, University of Florida, Gainsville, FL, USA.
authors: Michael S
authors: MS
authors: Okun
authors: Department of Neurosurgery, The Johns Hopkins Hospital, Baltimore, MD, USA.
authors: Alessandro
authors: A
authors: Olivi
authors: Department of Neurology, Johns Hopkins Hospital, David H. Koch Cancer Research Building II, 1550 Orleans Street, Room 1M16, Baltimore, MD 21287, USA.
authors: Carlos
authors: C
authors: Pardo-Villamizar
authors: Department of Neurology, Johns Hopkins Hospital, David H. Koch Cancer Research Building II, 1550 Orleans Street, Room 1M16, Baltimore, MD 21287, USA.
authors: Nicoline
authors: N
authors: Schiess
mesh_terms: D000328:Adult; D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D001933:Brain Stem / Q000473:pathology*; D003937:Diagnosis, Differential; D018237:Germinoma / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D014202:Tremor / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology
chemical_list: D005938:Glucocorticoids; D008775:Methylprednisolone
keywords: Behçet’s disease; Germinoma; Holmes tremor; Multiple sclerosis; Rubral tremor