Relationship between oral transit time and functional performance in motor neuron disease / Relação entre tempo de trânsito oral e desempenho funcional na doença do neurônio motor

ABSTRACT Oral phase swallowing impairment in motor neuron disease (MND) is caused by tongue weakness, fasciculation and atrophy, which may compromise oral transit time and total feeding time. Objective: To describe and correlate total oral transit time (TOTT) with functional performance in MND using different food consistencies. Methods: The study was conducted on 20 patients with MND, regardless of type or duration of the disease, of whom nine were excluded due to issues on the videofluoroscopic swallowing images. The remaining 11 patients (nine men and two women) ranged from 31 to 87 years of age (mean: 57 years) with scores on the Penetration Aspiration Scale ranging from ≤ 2 to ≤ 4. The Amyotrophic Lateral Sclerosis Functional Rating Scale - revised questionnaire was applied to classify individuals according to global, bulbar and bulbar/respiratory parameters. Videofluoroscopy of swallowing using 5ml of different consistencies was performed and a quantitative temporal analysis of the TOTT was carried out with the aid of specific software. Results: There was a wide variation in the TOTT within the same food consistency among MND patients. There was a correlation between the TOTT and overall functional performance for the thickened liquid consistency (r = −0.691) and between the TOTT and bulbar performance for the pureed consistency (r = −0.859). Conclusion: Total oral transit time in MND varies within the same food consistency and the longer the TOTT, regardless of food consistency, the lower the functional performance in MND.

RESUMO O comprometimento na fase oral da deglutição na doença do neurônio motor (DNM) é ocasionado por fraqueza, fasciculação e atrofia de língua, podendo comprometer o tempo de trânsito oral (TTO) e o tempo total de alimentação. Objetivo: Descrever e relacionar o tempo de trânsito oral total (TTOT) com o desempenho funcional na DNM em distintas consistências de alimento. Métodos: Participaram 20 indivíduos com DNM, independente do tipo ou tempo de doença. Foram incluídos 11 indivíduos, nove homens e duas mulheres, faixa etária de 31 a 87 anos (média de idade de 57 anos) e com Penetration Aspiration Scale (Rosenbek et al., 1996) de ≤ 2 a ≤ 4. Foram excluídos nove indivíduos por questões técnicas relacionadas às imagens videofluoroscópicas de deglutição. Aplicado o questionário Amyotrophic Lateral Sclerosis Functional Rating Scale - revised para classificação dos indivíduos de acordo com parâmetros Global, Bulbar e Bulbar/Respiratório. Realizada videofluoroscopia da deglutição com diferentes consistências de alimento no volume de cinco ml e análise quantitativa do TTOT por meio de software específico. Resultados: Houve ampla variação no TTOT dentro da mesma consistência de alimento na DNM. Houve correlação entre o TTOT e o desempenho funcional global na consistência líquida espessada (r = −0,691) e para o TTOT e o desempenho bulbar na pastosa (r = −0,859). Conclusão: O tempo de trânsito oral total na DNM varia dentro da mesma consistência de alimento e quanto mais longo o TTOT, independente da consistência do alimento, menor foi o desempenho funcional na DNM.

A Variant Guillain-Barré Syndrome with Anti-Ganglioside Complex Antibody

BACKGROUND: Recently, anti-ganglioside complex (GSC) antibodies were discovered among the various subtypes of Guillain-Barré syndrome. GSC is the novel glycoepitopes formed by two individual ganglioside molecules. CASE REPORT: We present a 36-year-old man with overlap Miller Fisher syndrome and acute bulbar palsy who had anti-GSC antibody that provided diagnostic robustness. CONCLUSION: Anti-GSC testing could be considered important in patients who show atypical manifestation with negative antibody reaction against each constituent ganglioside.

Epidemiological and clinical factors impact on the benefit of riluzole in the survival rates of patients with ALS / Impacto de fatores epidemiológicos e clínicos sobre o benefício do riluzol nas taxas de sobrevida de pacientes com ELA

ABSTRACT Objective To investigate the impact of epidemiological and clinical factors on the benefit of riluzole in patients with amyotrophic lateral sclerosis (ALS). Methods The survival rate of 578 patients with ALS (1999-2011) was analyzed by descriptive statistics and Kaplan-Meier curves. Considering the median of the sample survival time (19 months), patients were divided in two groups: below (B19) and above the median (A19). Kaplan-Meier curves compared the survival rates of patients treated with riluzole and with patients who did not take the medication. Results Riluzole increased the survival rates of patients with lower limb onset who were diagnosed after the first appointment in B19. Patients with bulbar onset and diagnosed on the first, or after the first appointment showed higher survival rates in A19. Males lived longer than females in both groups. Conclusion Epidemiological and clinical factors influenced the benefit of riluzole in the survival rates of patients with ALS.

RESUMO Objetivo Investigar o impacto de fatores epidemiológicos e clínicos sobre o benefício do riluzole em pacientes com esclerose lateral amiotrófica (ELA). Métodos A sobrevida de 578 pacientes com ELA (1999-2011) foi analisada por estatística descritiva e curvas de Kaplan-Meier. Considerando a mediana do tempo de sobrevida (19 meses), a amostra foi subdividida em dois grupos: sobrevida abaixo (B19) e acima de 19 meses (A19). As curvas de Kaplan-Meier compararam a sobrevida de pacientes tratados com riluzole e com pacientes que não receberam tratamento. Resultados O riluzole aumentou a sobrevida de pacientes com início nos membros inferiores e diagnosticados após a primeira consulta no grupo B19. Pacientes com início bulbar e diagnosticados na primeira/ após a primeira consulta apresentaram maior sobrevida em A19. Os homens apresentaram sobrevida maior do que as mulheres. Conclusão Foram encontradas diferenças epidemiológicas e clínicas no benefício do riluzole em pacientes com ELA.

Effective acupoints for bulbar paralysis by professor GAO Weibin / 中国针灸

Professor GAO Weibin academically advocates, based on basic theory of TCM and theories of different schools, modern science technology should be used for the methods and principles of acupuncture and Chinese medicine for neuropathy, so as to explore and summarize the rules, characteristics and advantages of TCM for nervous system disease, especially bulbar paralysis. During the treatment of bulbar paralysis, professor GAO creatively proposes the effective acupuncture points such as Gongxue, Tunyan-1, Tunyan-2, Fayin, Tiyan and Zhifanliu from the aspects of neuroanatomy, and analyzes their anatomical structure and action mechanism.

Clinical observation on medulla oblongata palsy after brainstem infarction treated with electroacupuncture at eight-neck-occiput points / 中国针灸

<p><b>OBJECTIVE</b>To compare the differences in the efficacy on speech and swallowing dysfunction of medulla oblongata palsy (MOP) after brainstem infarction between electroacupuncture at eight-neck-occiput points and routine acupoints.</p><p><b>METHODS</b>Seventy-two patients were randomized into a neck-occiput points group and a meridian points group, 36 cases in each one. In the neck-occiput points group, the eight-neck-occiput points (Neck 1-4 points, Occiput 1-4 points) were selected. In the meridian points group, Lianquan (CV 23), Futu (LI 18), Tongli (HT 5), Hegu (LI 4) and the others were selected. Electroacupuncture was used in the two groups, dense-dispersion wave, retaining for 30 min. The treatment was given once a day, 5 treatments a week. Totally, 4 weeks were required. The symptom scores of speech and swallowing dysfunction were observed before and after treatment in the two groups. The efficacy was compared between the two groups.</p><p><b>RESULTS</b>The scores of speech and swallowing dysfunction were improved significantly after treatment in the two groups (P < 0.05, P < 0.01). The results in the neck-occiput points group were better than those in the meridian points group (both P < 0.01). The curative rate of speech dysfunction was 30.6% (11/36) and that of swallowing dysfunction was 22.2% (8/ 36) in the neck-occiput points group, which were better than 11.1% (4/36) and 5.6% (2/36) in the meridian points group, respectively. The differences were significant in comparison of the two groups (P < 0.01, P < 0.05).</p><p><b>CONCLUSION</b>Electroacupuncture at eight-neck-occiput points achieves a better efficacy on speech and swallowing dysfunction of MOP after brainstem infarction as compared with the routine acupoints. This therapy is characterized as more accurate point localization and safer operation.</p>

Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report

Central pontine myelinolysis (CPM) classically occurs in alcoholics, malnourished individuals, chronic liver diseases, and rapid correction of hyponatremia. This report presents locked-in syndrome due to CPM following rapid correction of hyponatremia. A 44-year-old male came to the hospital due to a short period of loss of consciousness. He was alert and had no focal neurological abnormalities at admission. The serum sodium concentration was 118 mEq/L and was corrected to 134 mEq/L in the first 18 hours. One week later, progressive weakness in limbs developed and he progressed to a complete quadriplegic state and bulbar palsy, with only eye blinking preserved. Brain magnetic resonance imaging revealed a characteristic hyperintense signal abnormality in both pons, so he was diagnosed to locked-in syndrome caused by CPM. The patient gradually improved following continuous intensive rehabilitation for more than 2 years. He was able to move all joint muscles against gravity in generally and he could gait under supervision.

Efficacy on dysphagia induced by bulbar palsy treated with acupuncture at Tiantu (CV 22) with different needling techniques / 中国针灸

<p><b>OBJECTIVE</b>To compare the efficacy between elongated needle therapy and regular needle therapy at Tiantu (CV 22) on the basis of xingnao kaiqiao (activiting brain and regaining consciousness) acupuncture therapy so as to explore the effective therapeutic method in treatment of dysphagia induced by bulbar palsy.</p><p><b>METHODS</b>Seventy one cases of dysphagia induced by bulbar palsy were randomized into two groups. The xingnao kaiqiao acupuncture therapy was applied at Shuigou (GV 26), Neiguan (PC 6), Sanyinjiao (SP 6) and the others in the two groups. In the elongated needle therapy group, on the basis of xingnao kaiqiao acupuncture therapy, the elongated needle was used to puncture Tiantu (CV 22). In the regular needle therapy, the regular acupuncture technique was used at Tiantu (CV 22). In both groups, the treatment was given once a day in a week except Sunday and lasted for 4 weeks totally. Before and after treatment, the swallowing condition and the standardized swallowing assessment (SSA) were observed in the patients and the efficacy was compared between the two groups.</p><p><b>RESULTS</b>The total effective rate was 97.2% (35/36) in the elongated needle therapy group, which was better than 77.1% (27/35) in the regular needle therapy group (P<0.05). After treatment, SSA score was reduced significantly as compared with that before treatment in the two groups (both P<0.05). SSA score in the elongated needle therapy group was reduced much more apparently as compared with that in the regular needle therapy group after treatment (P<0.05).</p><p><b>CONCLUSION</b>On the basis of the xingnao kaiqiao acupuncture therapy, the elongated needle therapy at Tiantu (CV 22) achieves the superior effect on bulbar palsy-induced dysphagia as compared with the regular acu- puncture at Tiantu (CV 22).</p>

Multiple Cranial Neuropathies Without Limb Involvements: Guillain-Barre Syndrome Variant?

Acute multiple cranial neuropathies are considered as variant of Guillain-Barre syndrome, which are immune-mediated diseases triggered by various cases. It is a rare disease which is related to infectious, inflammatory or systemic diseases. According to previous case reports, those affected can exhibit almost bilateral facial nerve palsy, then followed by bulbar dysfunctions (cranial nerves IX and X) accompanied by limb weakness and walking difficulties due to motor and/or sensory dysfunctions. Furthermore, reported cases of the acute multiple cranial neuropathies show electrophysiological abnormalities compatible with the typical Guillain-Barre syndromes (GBS). We recently experienced a patient with a benign infectious disease who subsequently developed symptoms of variant GBS. Here, we describe the case of a 48-year-old male patient who developed multiple symptoms of cranial neuropathy without limb weakness. His laboratory findings showed a positive result for anti-GQ1b IgG antibody. As compared with previously described variants of GBS, the patient exhibited widespread cranial neuropathy, which included neuropathies of cranial nerves III-XII, without limb involvement or ataxia.

A síndrome de Villaret e o espaço retroparotídeo / Villaret syndrome and retroparotid space

A partir do relato de um caso sobre a síndrome de Villaret, o autor ressalta a importância da relação anátomo-clínica, aspectos clínicos e etiologias das síndromes que cursam com o comprometimento dos quatro últimos nervos cranianos quando da sua passagem extracranial.

From the case study of a patient with the Villaret syndrome the author emphasizes the importance of the anatomical-clinical relationship, clinical aspects, and etiologies of syndromes that are associated with the impairment of the last four cranial nerves during its out of skull passage.

Brown-vialetto-Van Laere syndrome: Egyptian case report

We report an Egyptian case with Brown-Vialetto-Van Laere syndrome. A five-year-oldgirl with progressive hearing loss was documented by brainstem auditory evoked potentials [BAEP] as sensorineural deafness, bilateral facial palsy and bilateral partial vocal cord paralysis in the paramedian position. There was no evidence of involvement of the upper motor neurons or spinalmotor nerves. There is no similar case in the family

Misdiagnosis of myasthenia gravis and subsequent clinical implication: a case report and review of literature

The autoimmune disease, myasthenia gravis [MG], can mimic a variety of neurological disorders leading to a delay in diagnosis and treatment. On occasions, misdiagnosis of MG could lead to unnecessary and potentially harmful therapeutic interventions. We report on a 12 year-old boy, in whom MG was mistaken for meningitic sequelae and subsequently for critical neuropathy/myopathy resulting in considerable morbidity for nearly a decade. Subsequent correct diagnosis and optimal management resulted in significant improvement in his functional status. We discuss the importance of considering MG as one of the potential differential diagnoses among cases of recurrent respiratory pump failure, or unexplained bulbar symptoms where documentary proof of the previous diagnoses including work-up for MG is lacking. We also review the literature on MG misdiagnosis and highlight the potential pitfalls in MG diagnosis

Treatment of Dysphagia with Pyridostigmine Bromide in a Patient with the Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome

A 24-year-old male developed bulbar palsy, ophthalmoplegia, ptosis, and shoulder weakness bilaterally 2 weeks after he had experienced an upper respiratory infection. The electrodiagnostic study demonstrated axonal polyradiculoneuropathy. The repetitive nerve stimulation study (RNS) showed no significant decrement of the compound muscle action potentials (CMAPs). The videofluoroscopic swallowing study (VFSS) showed severe impairment of the pharyngeal phase of swallowing. He was diagnosed as having the pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. The patient's dysphagia was not improved for 3 months. A follow up RNS showed a significant decrement of the CMAPs. Pyridostigmine bromide was tried to improve the dysphagia. The patient showed immediate improvement of his dysphagia on the VFSS after the trial with pyridostigmine bromide. Pyridostigmine bromide was given before each meal for 8 days and he showed continuous improvement of his dysphagia. The follow up VFSS after 3 months showed complete recovery of dysphagia.

Observation on theraputic effect of five-needle-in-nape acupuncture for treatment of poststroke pesudobulbar paralysis dysphagia / 中国针灸

<p><b>OBJECTIVE</b>To compare the therapeutic effect of five-needle-in-nape acupuncture treatment and the routine acupuncture treatment for poststroke pesudobulbar paralysis dysphagia.</p><p><b>METHODS</b>Sixty cases were randomly divided into a five-needle-in-nape (FNN) group and a routine acupuncture (RA) group (n=30 in each group). Ya-men (GV 15), Tianzhu (BL10), Zhiqiang acupoint (Extra) were needled and the training of swallowing was practiced when needling in FNN group. Lianquan (CV 23), Tongli (HT 5), Zhaohai (KI 6) were needled in RA group. The course of acupuncture treatment consisted of needling once time every day, six times a week for two weeks. The therapeutic effects were evaluated after two courses of acupuncture treatment.</p><p><b>RESULTS</b>The effective rate was 93.3% in FNN group, better than that of 80.0% in RA group (P < 0.05).</p><p><b>CONCLUSION</b>The therapeutic effect in FNN group was better than that of RA group for poststroke pesudobulbar paralysis dysphagia.</p>

Clinical presentations and complcations of Guilain barre syndrome in Children Welfare Teaching hospital

To study the clinical presentations and complications in patients with Guillain Barre syndrome [GBS]. A retrospective study was done on 70 patients with GBS from different parts of Iraq, who were admitted to children Welfare Teaching Hospital in Medical City, Baghdad in the period between January 2002 to December 2006. Of 70 patients with GBS, 40 [57.14%] were males and 30 [42.86%] were females, with a male to female ratio of 1.33:1. Antecedent events were found in 44 patients [62.86%], wherease 26 patients [37.14%] had no history of antecedent events. There is no specific pattern of motor weakness involvement and all cases show symmetrical muscle weakness. Bulbar nerves involvement is the commonest cranial nerve involvements. Autonomic nerves and respiratory muscles involvement are the main cause of death in GBS

Two Cases of Delayed Post-radiation Lower Cranial Nerve Palsies

Radiation-induced lower cranial neuropathy shows a clinical presentation similar to tumor recurrence or amyotrophic lateral sclerosis. We experienced two patients with bulbar palsies several years after radiotherapy for nasopharyngeal cancer. Brain magnetic resonance imaging showed no evidence of tumor recurrence. Electrophysiologic studies demonstrated mild denervation changes and myokymic discharges in muscles innervated by cranial nerves. Bulbar palsies progressed for 1 year then became stable. We emphasize the importance of myokymic discharges in the differential diagnosis of radiation-induced cranial neuropathy as radiation plexopathies.

A Brazilian family with Brown-Vialetto-van Laere syndrome with autosomal recessive inheritance

We report the first Brazilian family with Brown-Vialetto-van Laere syndrome. The presence of consanguineous marriages and illness affecting three sisters and one niece support an autosomal recessive transmission. The age at onset of the illness ranged from 12 to 20 years old. The time interval between hearing loss and involvement of other cranial nerves varied from 3 to 12 years. MRI demonstrated bulbar atrophy and also high intensity signal at T2 weighted and fluid attenuated inversion recovery (FLAIR) sequences.

Descrevemos a primeira família brasileira com síndrome de Brown-Vialetto-van Laere. Os pacientes são três irmãs e uma sobrinha provenientes de casamentos consangüíneos, o que fortalece a hipótese de transmissão autossômica recessiva. A idade de aparecimento dos sintomas variou entre 12 e 20 anos. A latência entre a perda auditiva e o envolvimento de outros nervos cranianos variou de 3 a 12 anos. O estudo de imagem por ressonância magnética demonstrou atrofia bulbar além de alteração de sinal nas seqüências ponderadas em T2 e FLAIR (fluid attenuated inversion recovery).

Evaluación fonodeglutoria en pacients con esclerosis lateral amiotrófica / Evaluation of speech and seallowing disorders in patients with amyotrophic lateral sclerosis

En la Esclerosis lateral Amiotrófica (ELA) el treinta por ciento de los pacientes comienza con síntomas bulbares que incluyen disfagia, disartria y alteraciones fonatorias. No es claro si el comporomiso bulbar implica el deterioro simultáneo de las tres funciones o si ellas pueden tener una evolución independiente. Existen escalas para evaluar individualmente esas funciones que son de dificil cuantificación clínica. La detección y la mensura adecuada de sus alteraciones permiten apreciar adecuadamente la discapacidad existente

Progressive bulbur paralysis (Fazio-Londe disease).

Progressive bulbar paralysis of childhood is characterised by progressive paralysis of muscles innervated by cranial nerves. The authors report a case of progressive bulbar paralysis of childhood in a 12-year-old child. Child was admitted with the complaints of drooping of eyelids, difficulty in swallowing and hoarse voice. She had involvement of III, VII, IX, X, XI and XII cranial nerves and the corticospinal tracts. Electromyography revealed spontaneous activity in the form of fasciculations, giant motor unit potential and discrete recruitment of motor neurons suggestive of denervation pattern. Hearing assessment was normal. Muscle biopsy was also suggestive of neurogenic atrophy.

Paralisia bulbar progressiva juvenil doença de Fazio-Londe: relato de caso / Progressive bulbar palsy (Fazio-Londe disease): case report

A paralisia bulbar progressiva, também denominada doença de Fazio-Londe, caracteriza-se pelo acometimento degenerativo progressivo de nervos cranianos bulbares em crianças. Foi descrita primeiramente por Fazio em 1892 e até a presente data somente 30 casos foram relatados na literatura. Acomete ambos os sexos, assumindo dois padröes clínicos, um de início precoce (idade <6 anos, predomínio de comprometimento respiratório) e outro de início tardio (6-20 anos, predomínio de comprometimento motor nos membros superiores). Descrevemos o caso de um menino com 4 anos, cujo primeiro sintoma foi estridor e disfunçäo respiratória grave, tendo inicialmente recebido diagnóstico de asma brônquica. O quadro evoluiu progressivamente, em aproximadamente 12 meses, para paralisia do V, VII, VIII, IX e X nervos cranianos, comprovada por acompanhamento clínico, exame físico e estudo eletrofisiológico de tronco cerebral

Motor neuron diseases in the University Hospital of Fortaleza (Northeastern Brazil): a clinico-demographic analysis of 87 cases

In this retrospective (1980-1998) study, we have analyzed clinico-demographically, from the records of the University Hospital of Fortaleza (Brazil), a group of 87 patients showing signs and symptoms of motor neuron diseases (MNDs). Their diagnosis was determined clinically and laboratorially. The WFN criteria were used for amyotrophic lateral sclerosis (ALS) diagnosis. The clinico-demographic analysis of the 87 cases of MNDs showed that 4 were diagnosed as spinal muscular atrophy (SMA), 5 cases as ALS subsets: 2 as progressive bulbar paralysis (PBP), 2 as progressive muscular atrophy (PMA) and 1 as monomelic amyotrophy (MA), and 78 cases of ALS. The latter comprised 51 males and 27 females, with a mean age of 42.02 years. They were sub-divided into 4 groups according to age: from 15 to 29 years (n= 17), 30 to 39 years (n= 18), 40 to 69 years (n= 39) and 70 to 78 years (n= 4). From the 78 ALS patients, 76 were of the classic sporadic form whilst only 2 were of the familial form. The analysis of the 87 patients with MNDs from the University Hospital of Fortaleza showed a predominance of ALS patients, with a high number of cases of juvenile and early onset adult sporadic ALS