Assuntos
Herpes Zoster da Orelha Externa/complicações , Dissinergia Cerebelar Mioclônica/diagnóstico , Aciclovir/administração & dosagem , Idoso , Herpesvirus Humano 3/patogenicidade , Humanos , Masculino , Dissinergia Cerebelar Mioclônica/tratamento farmacológico , Dissinergia Cerebelar Mioclônica/virologia , Prednisolona/administração & dosagemRESUMO
OBJECTIVE: We report our retrospective study of the recovery rate of auditory ossicles preserved facial nerve decompression surgery via the transmastoid approach in cases of both an electroneurography score of < 10% and a Yanagihara score of ≤8 in Bell's palsy and Ramsay Hunt syndrome. MATERIALS AND METHODS: We retrospectively reviewed 47 patients who we were able to follow-up for more than 6 months following the onset of palsy. The recovery rate was defined by the Japan Society for Facial Nerve Research or the Yanagihara score. RESULTS: Twelve months after palsy onset, the recovery rate was 48.8% (20/41) for all patients, 65.2% (15/23) for patients with Bell's palsy, and 27.8% (5/18) for patients with Ramsay Hunt syndrome. Comparing the clinical efficacy of surgical treatment at 12 months after palsy onset, we observed a statistically significant effect of age. Comparing the Yanagihara scores of patients aged < 60 years with those of patients aged ≥60 years revealed that patients aged ≥60 years had significant poor prognosis, particularly in patients with Ramsay Hunt syndrome, which showed a very low recovery rate (14.3%). We also analyzed six other factors, but none showed statistical significance. CONCLUSION: The clinical efficacy of surgical treatment of Ramsay Hunt syndrome was inferior to that of Bell's palsy, which is consistent with previous reports. There was a statistically significant difference in the Yanagihara score between patients aged < 60 years and those aged ≥60 years. Particularly, patients with Ramsay Hunt syndrome aged ≥60 years have a very low recovery rate.
Assuntos
Descompressão Cirúrgica/métodos , Ossículos da Orelha/cirurgia , Nervo Facial/cirurgia , Paralisia Facial/cirurgia , Dissinergia Cerebelar Mioclônica/cirurgia , Adulto , Idoso , Paralisia Facial/diagnóstico , Paralisia Facial/epidemiologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Dissinergia Cerebelar Mioclônica/diagnóstico , Dissinergia Cerebelar Mioclônica/epidemiologia , Complicações Pós-Operatórias , Prognóstico , Recuperação de Função Fisiológica/fisiologia , Estudos RetrospectivosRESUMO
BACKGROUND Ramsay Hunt syndrome is a rare otologic complication resulting from varicella zoster virus reactivation that can present with a myriad of clinical presentations. Most common being triad of ear pain, vesicles at auricle, and ear canal with same side facial palsy. CASE REPORT We report a case of a 29-year-old male with a human immunodeficiency virus (HIV) infection who presented with left facial palsy, vesicles, pain in the left ear, dysphagia, dizziness, and headache resulting from multiple cranial nerves involvement such as cranial nerve V, VII, VIII, IX, and X. CONCLUSIONS This case report raises awareness among general practitioners to investigate for Ramsay Hunt syndrome in HIV patients presenting with ear pain with a thorough neurological exam and emphasize on the interplay of different specialties in managing these patients.
Assuntos
Doenças dos Nervos Cranianos/diagnóstico , Doenças dos Nervos Cranianos/etiologia , Infecções por HIV/complicações , Dissinergia Cerebelar Mioclônica/complicações , Dissinergia Cerebelar Mioclônica/diagnóstico , Adulto , Doenças dos Nervos Cranianos/terapia , Humanos , Masculino , Dissinergia Cerebelar Mioclônica/terapiaRESUMO
The aim of this study was to compare clinical characteristics, electroneurography (ENoG) results, and functional outcomes of patients with Bell's palsy (BP) and Ramsay Hunt syndrome (RHS).Around 57 patients with BP and 23 patients with RHS were enrolled in this study from January 2010 and September 2015. Both clinical characteristics and ENoG results were recorded at hospital admission. The evaluations of functional outcomes were conducted with House-Brackmann (H-B) grading system at 6-month follow-up.There were no significant differences in age, gender proportion, initial H-B grades, time before commencement of treatment and the presence of comorbid disease in 2 groups. However, the final H-B grades at 6-month follow-up were significantly better in BP patients than RHS patients. The results of ENoG showed that degeneration index (DI) was significantly higher in the RHS group than the BP group. But no significant difference was found in the value of prolonged latency time (PLT) between the 2 groups. In multivariate analysis, age and ENoG DI were independently associated with functional outcome of recovery in the BP group (OR 0.167, 95% CI 0.038-0.622, Pâ=â0.009 and OR 0.289 95% CI 0.107-0.998, Pâ=â0.050, respectively). However, in the RHS group, only ENoG DI was related to the final H-B grades (OR 0.067, 95% CI 0.005-0.882, Pâ=â0.040). Spearman's rank correlation analysis showed that higher age and ENoG DI were related to poorer prognosis in 2 groups (Pâ<â0.05). PLT was related to functional outcomes only in the BP group (rsâ=â0.460, Pâ<â0.001). The receiver operating characteristic (ROC) of ENoG DI analysis revealed that the cutoff value was 67.0% for BP prognosis and 64.5% for RHS prognosis. What's more, patients with hypertension or diabetes mellitus had both higher final H-B grade and ENoG DI than those without the same comorbidity.Patients with RHS had poorer prognosis than those with BP. Some factors including age, ENoG DI, and the presence of disease influenced recovery from BP and RHS. The present study demonstrated that BP patients with ENoG DIâ<â67.0% and RHS patients with ENoG DI < 65.5% had a greater opportunity for recovery within half a year.
Assuntos
Paralisia de Bell/diagnóstico , Dissinergia Cerebelar Mioclônica/diagnóstico , Adulto , Fatores Etários , Idoso , Paralisia de Bell/fisiopatologia , Eletrodiagnóstico , Fenômenos Eletrofisiológicos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dissinergia Cerebelar Mioclônica/fisiopatologia , Prognóstico , Recuperação de Função FisiológicaRESUMO
BACKGROUND: Neurosyphilis is defined as any involvement of the central nervous system by the bacterium Treponema pallidum. Movement disorders as manifestations of syphilis have been reported quite rarely. CASE PRESENTATION: We report a case of a 42-year-old Russian man living in Estonia with rapidly progressive dementia and movement disorders manifesting as myoclonus, cerebellar ataxia and parkinsonism. The mini mental state examination score was 12/30. After excluding different neurodegenerative causes, further diagnostic testing was consistent with neurosyphilis. Treatment with penicillin was started and 6 months later his mini mental state examination score was 25/30 and he had no myoclonus, parkinsonism or cerebellar dysfunction. CONCLUSION: Since syphilis is easily diagnosed and treatable, it should be considered and tested in patients with cognitive impairment and movement disorders.
Assuntos
Antibacterianos/uso terapêutico , Dissinergia Cerebelar Mioclônica/diagnóstico , Neurossífilis/diagnóstico , Neurossífilis/tratamento farmacológico , Transtornos Parkinsonianos/diagnóstico , Penicilinas/uso terapêutico , Adulto , Diagnóstico Diferencial , Humanos , Masculino , Dissinergia Cerebelar Mioclônica/fisiopatologia , Neurossífilis/microbiologia , Neurossífilis/fisiopatologia , Transtornos Parkinsonianos/fisiopatologia , Resultado do Tratamento , Treponema pallidum/efeitos dos fármacos , Treponema pallidum/crescimento & desenvolvimento , Treponema pallidum/isolamento & purificaçãoRESUMO
Surgical approaches to the temporomandibular joint (TMJ) have been designed specifically to minimize injury to the temporal branch of the facial nerve. In spite of this, facial nerve dysfunction occurs in 1-32% of patients undergoing TMJ surgery. Ramsay Hunt syndrome is characterized by peripheral facial paralysis that often involves other cranial nerves, mostly cranial nerve VIII. The pathology is attributed to the reactivation of latent varicella zoster virus in the geniculate ganglion. The diagnosis is based mostly on history and physical findings. Surgical procedures have been known to reactivate varicella zoster virus, but Ramsay Hunt syndrome subsequent to TMJ surgery has not been described yet. This report describes a case of Ramsay Hunt syndrome associated with TMJ surgery. Because of the relatively high incidence of facial nerve dysfunction associated with TMJ surgery, patients with varicella zoster virus reactivation may initially be misdiagnosed with iatrogenic facial palsy, or vice versa.
Assuntos
Dissinergia Cerebelar Mioclônica/diagnóstico , Dissinergia Cerebelar Mioclônica/etiologia , Transtornos da Articulação Temporomandibular/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Dissinergia Cerebelar Mioclônica/terapia , Adulto JovemRESUMO
A 57-year-old man developed 3 days of left facial pain and swelling with left-sided hearing loss followed by a painful, unilateral, erythematous, and vesicular rash on the left anterior two-thirds of the tongue, external auditory canal, lip, and face typical of varicella-zoster virus reactivation (figure). Reactivation in the geniculate ganglion or facial nerve is uncommon and typically causes tongue and auricular lesions or facial palsy and was described by Hunt in 1907.(1) The patient received IV acyclovir and oral prednisone with rapid improvement in pain and resolution of lesions and improvement of hearing over 1 month.
Assuntos
Dissinergia Cerebelar Mioclônica/diagnóstico , Língua/patologia , Gânglio Geniculado , Herpes Zoster/complicações , Herpesvirus Humano 3/patogenicidade , Humanos , Masculino , Pessoa de Meia-Idade , Dissinergia Cerebelar Mioclônica/fisiopatologia , Dissinergia Cerebelar Mioclônica/virologiaRESUMO
Mevalonic aciduria (MA) represents the severest form of mevalonate kinase deficiency due to recessively inherited, loss-of-function MVK mutations. MA is an early-onset disorder characterized by a marked failure to thrive, diverse neurologic symptoms, dysmorphic features, and recurrent febrile episodes. However, significant clinical differences have been reported in the few cases published to date. Here we describe 2 unrelated Spanish patients with MA, emphasizing the clinical heterogeneity observed. One patient presented with the severe classic MA phenotype due to the homozygous p.Ile-268-Thr MVK genotype, with a poor response to conventional treatments. However, the anti-interleukin 1 agent anakinra in this patient resulted in improvement in many clinical and laboratory parameters. The second patient presented with an atypical milder phenotype because of an older age at disease onset, mild neurologic symptoms, absence of febrile episodes and dysmorphic features, and moderate-to-good response to conventional treatments. The novel p.Arg-241-Cys MVK mutation, associated with the already known p.Ser-135-Leu mutation, detected in this patient expands the genetic diversity of mevalonate kinase deficiency. This atypical presentation of MA suggests that it should be included in the differential diagnosis of unclassified patients with psychomotor retardation, failure to thrive or ataxia, even in the absence of febrile episodes.
Assuntos
Alelos , Análise Mutacional de DNA , Deficiência de Mevalonato Quinase/diagnóstico , Deficiência de Mevalonato Quinase/genética , Encéfalo/patologia , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/genética , Cerebelo/patologia , Criança , Diagnóstico Diferencial , Insuficiência de Crescimento/diagnóstico , Insuficiência de Crescimento/genética , Febre de Causa Desconhecida/diagnóstico , Febre de Causa Desconhecida/genética , Genes Recessivos/genética , Variação Genética , Genótipo , Humanos , Lactente , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Deficiência de Mevalonato Quinase/tratamento farmacológico , Ácido Mevalônico/urina , Dissinergia Cerebelar Mioclônica/diagnóstico , Dissinergia Cerebelar Mioclônica/genética , Fenótipo , Transtornos Psicomotores/diagnóstico , Transtornos Psicomotores/genéticaRESUMO
Mutations in the CACNA1A gene were described in familial hemiplegic migraine, episodic ataxia type 2, and spinocerebellar ataxia type 6. Familial hemiplegic migraine and episodic ataxia type 2 are caused by point mutations in the CACNA1A gene, and spinocerebellar ataxia type 6 develops as a result of a CAG triple expansion in exon 1 of the gene. Phenotypic variability and clinical overlap are well recognized. We describe a 3-year-old child with clinical and radiologic signs of early-onset cerebellar atrophy. The family history was significant for migraine, and in some members of the family, a diagnosis of hemiplegic migraine was established. The combination of cerebellar atrophy in our patient and the family history suggested involvement of the CACNA1A gene. The sequence analysis of genomic DNA from the proband identified heterozygosity for a mutation (Thr666Met) in the CACNA1A gene. Subsequently, his father, who was mildly affected, and two other relatives were demonstrated to carry the same mutation. Therefore, CACNA1A gene mutations should be considered in the differential diagnosis of congenital cerebellar atrophy.
Assuntos
Canais de Cálcio/genética , Dissinergia Cerebelar Mioclônica/diagnóstico , Dissinergia Cerebelar Mioclônica/genética , Mutação Puntual/genética , Pré-Escolar , Humanos , Masculino , LinhagemRESUMO
Methylenetetrahydrofolate reductase catalyzes the formation of 5-methyltetrahydrofolate from 5,10-methylentetrahydrofolate and produces folate for the methylation of homocysteine to methionine. Due to insufficient conversion of homocysteine to methionine, plasma homocysteine levels increase in methylenetetrahydrofolate reductase deficiency. Homocysteine is an amino acid that contains a neurotoxic sulfur molecule and can induce neuronal apoptosis. Methylenetetrahydrofolate reductase deficiency is 1 of the etiological factors that causes neurological symptoms and signs in the newborn and childhood period. Here, we report a premature baby with prenatal onset diffuse multicystic encephalomalacia and cerebellar atrophy due to homozygous methylenetetrahydrofolate reductase mutation.
Assuntos
Análise Mutacional de DNA , Encefalomalacia/genética , Doenças do Prematuro/genética , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Dissinergia Cerebelar Mioclônica/genética , Encéfalo/patologia , Hemorragia Cerebral/genética , Hemorragia Cerebral/patologia , Ecoencefalografia , Encefalomalacia/diagnóstico , Humanos , Recém-Nascido , Doenças do Prematuro/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Metilenotetra-Hidrofolato Redutase (NADPH2)/deficiência , Dissinergia Cerebelar Mioclônica/diagnóstico , Tomografia Computadorizada por Raios XRESUMO
UNLABELLED: Autoantibodies to glutamic acid decarboxylase (GAD-Ab) have been described in stiff-man syndrome, type 1 diabetes mellitus and in patients with auto-immune polyglandular failure. In addition, a few patients with progressive cerebellar ataxia show high titres of GAD-Ab, suggesting an auto-immune origin. AIM: This is a report of a patient presenting with cerebellar ataxia associated to late-onset type 1 diabetes and polyendocrine auto-immunity. CASE REPORT: A 47-year-old woman with a past medical history of vitiligo and Graves' disease presented with late-onset type 1 diabetes. For two years, she had complained of progressive gait instability and oscillopsia. Neurological examination revealed multidirectional, horizontal rotatory fixation and gaze nystagmus, gait ataxia and mild limb ataxia in the left upper arm. METHODS: Imaging studies, electrophysiological studies, routine biological and detailed immunological screening as well as a study of cerebrospinal fluid (CSF) were performed. RESULTS: Brain magnetic resonance imaging showed cerebellar atrophy. Routine biological screening was normal. Immunological screening showed positivity for numerous antibodies (Ab), including GAD-Ab, thyroid peroxidase-Ab, thyroglobulin-Ab, 21-hydroxylase (adrenal)-Ab, gastric parietal cell-Ab and GM1 ganglioside IgG-Ab. CSF was normal, with no oligoclonal bands detected. GAD-Ab were positive in CSF, suggesting an auto-immune origin of the cerebellar ataxia. Treatment with intravenous immunoglobulin led to a slight improvement in nystagmus and gait instability. CONCLUSION: Auto-immune cerebellar ataxia related to GAD-Ab is a rare condition that typically affects women with late-onset type 1 diabetes or other auto-immune disorders, including auto-immune polyendocrinopathy. Immunomodulatory treatment may be effective.
Assuntos
Autoanticorpos/sangue , Doenças Autoimunes/complicações , Ataxia Cerebelar/complicações , Diabetes Mellitus Tipo 1/complicações , Glutamato Descarboxilase/imunologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/imunologia , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/imunologia , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/imunologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Dissinergia Cerebelar Mioclônica/diagnósticoAssuntos
Cóclea/patologia , Dissinergia Cerebelar Mioclônica/diagnóstico , Cóclea/microbiologia , Diagnóstico Diferencial , Herpes Zoster da Orelha Externa/complicações , Herpes Zoster da Orelha Externa/virologia , Humanos , Imageamento por Ressonância Magnética , Dissinergia Cerebelar Mioclônica/complicaçõesRESUMO
Tacrolimus is a macrolide immunosuppressant frequently used after solid-organ transplantation. Moderate and severe neurologic side effects have been reported in patients receiving tacrolimus. Cerebral neurotoxicity is a rare but fatal calcineurin inhibitor-related complication, especially in kidney and liver transplant recipients. Often a reduction or a change in immunosuppressive regimen is the only means of clinical management. Herein we report a case of a 31-year-old man who developed cerebellar atrophia while under immunosuppressive therapy 9 years after heart transplantation. His neurologic constitution ameliorated after an immunosuppressant switch from tacrolimus to sirolimus.
Assuntos
Rejeição de Enxerto/prevenção & controle , Transplante de Coração , Imunossupressores/efeitos adversos , Dissinergia Cerebelar Mioclônica/induzido quimicamente , Tacrolimo/efeitos adversos , Adulto , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Dissinergia Cerebelar Mioclônica/diagnóstico , Tacrolimo/uso terapêutico , Fatores de TempoRESUMO
A huge number of neurological disorders are associated with myoclonus. This paper describes these disorders whose diagnosis partly relies on the presence of myoclonus. The diagnostic approach is related to certain clinical features of myoclonus, which, after their integration in the clinical context, help orientate towards diagnosis. Myoclonus is frequent during dementia. Although its presence is well-known to take part in the diagnosis of Creutzfeldt-Jakob disease (CJD), myoclonus can also be present to a significant degree in Alzheimer's disease and Lewy body dementia (LBD), which raises a diagnostic issue. Both its clinical and electrophysiological features may help differential diagnosis, given that myoclonus with fast-evolving dementia and focal neurological signs should favor the diagnosis of CJD. Myoclonus in a context of progressive ataxia suggests one clinical form of the Ramsay-Hunt syndrome (progressive myoclonic ataxia, PMA), whose most frequent causes are: coeliac disease, mitochondriopathies, some spino-cerebellar degenerations, and some late metabolic disorders. In addition to ataxia and myoclonus, the presence of opsoclonus directs diagnosis toward the opsoclonus-myoclonus syndrome (OMS), whose origin, in adult, is idiopathic or paraneoplastic. Palatal tremor (myoclonus) with ataxia may represent either a sporadic pattern, which often reflects the evolution of degenerative or lesional disorders, or a familial pattern in some degenerative affections or metabolic diseases. Of more recent knowledge is the association of progressive ataxia, myoclonus, and renal failure, which corresponds to a recessive autosomic disease. In a context of encephalopathy, myoclonus is frequent in metabolic or hydro-electrolytic disorders, and in brain anoxia. One should distinguish these various forms of myoclonus which may occur in the acute post-anoxic phase, from those occurring as sequels at a later stage, i.e. the Lance and Adams syndrome whose clinical aspects are also multiple. Myoclonus is less frequent during toxic or drug exposures. Irrespective of its acute or insidious onset, Hashimoto's encephalopathy is accompanied by myoclonus and tremor. Myoclonus may also be present during encephalic and/or spinal infectious disorders. Myoclonus with focal neurological signs may be observed in thalamic lesions, responsible for unilateral asterixis or unilateral myoclonus superimposed on dystonic posture. Segmental spinal myoclonus or propriospinal myoclonus may be associated with several spinal-cord disorders. Myoclonus associated with peripheral nerve lesions is exceptional or even questionable for some of these.
Assuntos
Mioclonia/etiologia , Doenças do Sistema Nervoso/complicações , Adolescente , Adulto , Idoso , Encefalopatias Metabólicas/complicações , Encefalopatias Metabólicas/diagnóstico , Demência/complicações , Demência/diagnóstico , Humanos , Hipóxia Encefálica/complicações , Hipóxia Encefálica/diagnóstico , Infecções/complicações , Dissinergia Cerebelar Mioclônica/complicações , Dissinergia Cerebelar Mioclônica/diagnóstico , Mioclonia/genética , Doenças do Sistema Nervoso/diagnóstico , Síndromes Neurotóxicas/complicações , Síndromes Neurotóxicas/diagnóstico , Síndrome de Opsoclonia-Mioclonia/diagnóstico , Insuficiência Renal/complicações , Tremor/etiologiaRESUMO
Two hundred and forty-eight patients from 116 Italian families with dominant ataxia were studied for CAG expansion within SCA1, 2, 3, 6, 7 (spinocerebellar ataxia) and DRPLA (dentatorubropallidoluysian atrophy) genes. Fifty-six percent of the families originated from Southern, 19% from Central and 25% from Northern Italy. SCA2 was the commonest mutation, accounting for 47% of the families, followed by SCA1 (24%), SCA6 (2%), SCA7 (2%) and DRPLA (1%). No SCA3 family was found. Twenty-four percent of the families carried a still unidentified mutation. When occurrence of mutations was evaluated according to the geographic origin, SCA1 was the commonest in Northern (72%), whereas SCA2 was prevalent (63%) in Southern Italy. The number of CAG repeats in SCA1 normal alleles was higher in Northern than in Central-Southern Italy.
Assuntos
Globo Pálido , Dissinergia Cerebelar Mioclônica/genética , Núcleo Rubro , Degenerações Espinocerebelares/genética , Repetições de Trinucleotídeos/genética , Alelos , Análise Mutacional de DNA , Feminino , Frequência do Gene/genética , Genes Dominantes/genética , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Dissinergia Cerebelar Mioclônica/diagnóstico , Exame Neurológico , Degenerações Espinocerebelares/diagnósticoRESUMO
In 1921 Ramsay-Hunt first described the syndrome of dyssynergia cerebellaris myoclonica (DCM), characterized by the clinical triad of action myoclonus, progressive ataxia and epilepsy with cognitive impairment, subsequently also referred to as the "Ramsay-Hunt syndrome". The cause of the symptoms of this rare degenerative syndrome (incidence: 500,000) is the impairment of a regulatory mechanism between nucleus dentatus, nucleus ruber and the bulbar olive. We present two sisters, aged 29 and 30 years, who were investigated for oculomotor abnormalities. The patients were diagnosed as having DCM according to clinical symptomatology, which was confirmed by neurophysiological and radiological findings. In both cases saccadic velocity was markedly reduced, whereas saccadic latency showed a significant increase. In addition, smooth pursuit eye-movements were abnormal and presented reduced gain. These findings suggest that pontine areas and the vestibulocerebellum also seem to be affected in DCM.
Assuntos
Dissinergia Cerebelar Mioclônica/genética , Transtornos da Motilidade Ocular/genética , Adulto , Cerebelo/patologia , Eletroculografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Dissinergia Cerebelar Mioclônica/diagnóstico , Dissinergia Cerebelar Mioclônica/fisiopatologia , Exame Neurológico , Transtornos da Motilidade Ocular/diagnóstico , Transtornos da Motilidade Ocular/fisiopatologia , Acompanhamento Ocular Uniforme/fisiologia , Tempo de Reação/fisiologia , Movimentos Sacádicos/fisiologiaRESUMO
It is clear, from our clinical experience, that the facial nerve in patients with facial palsy is enhanced on magnetic resonance (MR) imaging after intravenous administration of gadolinium diethylenetriamine. However, some problems with clinical reliability persist. There have been reports that normal facial nerves often show enhancement on MR imaging. We also question whether there are any differences in the degree of enhancement between Bell's palsy and Ramsay Hunt syndrome. To solve these problems, analyses were conducted using a personal computer by means of digital image-processing to measure the gray scale levels of enhanced facial nerves on MR imaging films. Seventeen cases of Bell's palsy, eight cases of Ramsay Hunt syndrome and fourteen normal subjects whose facial nerves showed enhancement on MR imaging were selected for the analyses. The concept of a facial nerve/whole image ratio (F/W ratio), analyzing the degree of enhancement of the facial nerve quantitatively, is introduced in this paper. The F/W ratio is the ratio of the gray scale level of the facial nerve region to the highest gray scale level in the skull at the MR imaging film. When the F/W ratios of these subjects were analyzed, no significant differences were found between Bell's Palsy and Ramsay Hunt syndrome in the degree of enhancement; facial palsy cases showed quantitatively larger F/W ratios than normal subjects.
Assuntos
Nervo Facial/patologia , Paralisia Facial/diagnóstico , Compostos Organometálicos , Ácido Pentético/análogos & derivados , Gadolínio DTPA , Humanos , Injeções Intravenosas , Imageamento por Ressonância Magnética , Dissinergia Cerebelar Mioclônica/diagnósticoRESUMO
An autopsied case of Ramsay Hunt syndrome with progressive dementia was reported. The clinical symptoms included progressive intellectual decline, myoclonus, generalized convulsive seizure, cerebellar ataxia and positive pyramidal signs. Neuropathological examination disclosed cerebral white matter demyelination marked in the frontal lobe and fibrillary gliosis predominantly in the subcortical U-fibers, grumose degeneration in the dentate nucleus and inferior olivary nucleus lesion. The skeletal muscle showed no ragged-red fiber. The present case can be included in Ramsay Hunt syndrome because of the absence of pathological hallmark of mitochondrial encephalomyopathy and of the presence of the degenerative lesions in the olivary and dentate nucleus without cerebellar Purkinje cell loss. The intellectual decline is a result of extensive frontal white matter change, and myoclonus and ataxia are closely associated with dentate grumose degeneration. The cerebral white matter change is an unusual finding and the present case might be a variant in Ramsay Hunt syndrome.