Cerebellar atrophy is frequently associated with non-paraneoplastic sensory neuronopathy

Sensory neuronopathies (SN) are peripheral nervous system disorders associated with degeneration of dorsal root ganglion neurons. Despite the evidence of a defective proprioceptive sensory input in SN, the prominent gait and truncal ataxia raises the question of a concomitant involvement of the cerebellum. Objective: To evaluate cerebellar atrophy in SN. Method: We analyzed MRI-based volumetry of anterior lobe (paleocerebellum) and total cerebellum in patients with non-paraneoplastic chronic SN and compared to age- and gender-matched controls. Results: Cerebellum and anterior lobe MRI volumetry were performed in 20 patients and nine controls. Mean anterior lobe and cerebellar volume were not statistically different. Three patients (15%), however, had an abnormal anterior lobe and cerebellar volume index (values outside 2.5 standard deviations). One of them also had a specific atrophy of the anterior lobe. All these patients had infectious or dysimmune associated SN. Conclusion: Cerebellar atrophy is infrequently associated with SN, but can be found in some patients with SN related to infectious or immune mediated conditions. It can be more prominent in the anterior lobe and may contribute to the ataxia seen in these patients.69460260

Bickerstaff's Encephalitis, Guillain-barré Syndrome And Idiopathic Intracranial Hypertension: Are They Related Conditions?

[No abstract available]663 B744746Hughes, R.A., Cornblath, D.R., Guillain-Barré syndrome. (2005) Lancet, 366, pp. 1653-1666Overell, J.R., Willison, H.J., Recent developments in Miller Fisher syndrome and related disorders (2005) Curr Opin Neurol, 18, pp. 562-566Odaka, M., Yuki, N., Yamada, M., Bickerstaff's brainstem encephalitis: Clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome (2003) Brain, 126, pp. 2279-2290Ball, A.K., Clarke, C.E., Idiopathic intracranial hypertension (2006) Lancet Neurol, 5, pp. 433-442Walker, R.W., Idiopathic intracranial hypertension: Any light on the mechanism of the raised pressure? (2001) J Neurol Neurosurg Psychiatry, 71, pp. 1-5Weiss, G.B., Bajwa, Z.H., Mehler, M.F., Co-occurrence of pseudotumor cerebri and Guillain-Barré syndrome in an adult (1991) Neurology, 41, pp. 603-604Ropper, A.H., Marmarou, A., Mechanism of pseudotumor in Guillain-Barré syndrome (1984) Arch Neurol, 41, pp. 259-261Pulitanò, S., Viola, L., Genovese, O., Miller-Fisher syndrome mimicking intracranial hypertension following head trauma (2005) Childs Nerv Syst, 21, pp. 473-476Fisher, M., An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia) (1956) N Engl J Med, 255, pp. 57-65Bickerstaff, E.R., Brain-stem encephalitisfurther observations on a grave syndrome with benign prognosis (1957) Br Med J, 1, pp. 1384-1387Al-Din, A.N., The nosological position of the ophthalmoplegia, ataxia and areflexia syndrome: "the spectrum hypothesis (1987) Acta Neurol Scand, 75, pp. 287-294Chiba, A., Kusunoki, S., Obata, H., Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: Clinical and immunohistochemical studies (1993) Neurology, 43, pp. 1911-1917Nagaoka, U., Kato, T., Kurita, K., Cranial nerve enhancement on three-dimensional MRI in Miller Fisher syndrome (1996) Neurology, 47, pp. 1601-1602Kornberg, A.J., Pestronk, A., Blume, G.M., Selective staining of the cerebellar molecular layer by serum IgG in Miller Fisher and related syndromes (1996) Neurology, 47, pp. 1317-1320Lo, Y.L., Chan, L.L., Pan, A., Ratnagopal, P., Acute ophthalmoparesis in the anti-GQ1b antibody syndrome: Electrophysiological evidence of neuromuscular transmission defect in the orbicularis oculi (2004) J Neurol Neurosurg Psychiatry, 75, pp. 436-440Yuki, N., Koga, M., Bacterial infections in Guillain-Barré and Fisher syndromes (2006) Curr Opin Neurol, 19, pp. 451-457Kwon, H.M., Hong, Y.H., Sung, J.J., A case of Bickerstaff's brainstem encephalitisthe evidence of cerebellum involvement by SPM analysis using PET (2006) Clin Neurol Neurosurg, 108, pp. 418-420Urushitani, M., Udaka, F., Kameyama, M., Miller Fisher-Guillain-Barré overlap syndrome with enhancing lesions in the spinocerebellar tracts (1995) J Neurol Neurosurg Psychiatry, 58, pp. 241-243Ogawara, K., Kuwabara, S., Yuki, N., Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems (2002) Muscle Nerve, 26, pp. 845-849Overell, J.R., Hsieh, S.T., Odaka, M., Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders (2007) Cochrane Database Syst Rev, 1. , CD00476

Guillain-barré Syndrome In The Elderly: Clinical, Electrophysiological, Therapeutic And Outcome Features

There are few papers devoted to geriatric Guillain-Barré (GBS) and many related issues remain unanswered. Objective: To describe clinical, electrophysiological and therapeutic features in this age. Method: Clinico-epidemiological data and therapy of GBS patients older than 60 years were reviewed. Hughes scores were used to quantify neurological deficit and define outcome. Results: Among 18 patients (mean age 64.8 years), 9 had evident prodrome and 80% noticed initially sensory-motor deficit. Demyelinating GBS was found in 8 and axonal in 6 subjects. There was one Miller-Fisher and 3 unclassified cases. Plasmapheresis (PFX) was single therapy in 12 patients and intravenous immunoglobulin (IVIg) in 2. Disability scores just before therapy were similar in both groups, so as short and long term outcome. Conclusion: Axonal GBS seems to be more frequent in the elderly and this may have prognostic implications. PFX and IVIg were suitable options, but complications were noticed with PFX. Prospective studies are needed to better understand and manage GBS in the elderly.633 B772775Kuwabara, S., Guillain-Barré syndrome: Epidemiology, pathophysiology and management (2004) Drugs, 64, pp. 597-610Hughes, R.A.C., Rees, J.H., Clinical and epidemiological features of Guillain-Barré syndrome (1997) J Infect Dis, 176 (SUPPL. 2), pp. S92-S98Hartung, H.P., Willison, H.J., Kieseier, B.C., Acute immunoinflammatory neuropathy: Update on Guillain-Barré syndrome (2002) Curr Opin Neurol, 15, pp. 571-577Efficiency of plasma exchange in Guillain-Barré syndrome: Role of replacement fluids (1987) Ann Neurol, 22, pp. 753-761Greenwood, R.J., Newsom Davis, J.M., Hughes, R.A.C., Controlled trial of plasma exchange in acute inflammatory polyradiculoneuropathy (1984) Lancet, 1, pp. 877-879Osterman, P.O., Lundemo, G., Pirskanen, R., Beneficial effects of plasma exchange in acute inflammatory polyradiculoneuropathy (1984) Lancet, 2, pp. 1296-1299Plasmapheresis and acute Guillain-Barré syndrome (1985) Neurology, 35, pp. 1096-1104Plasma exchange in Guillain-Barré syndrome: One-year follow-up (1992) Ann Neurol, 32, pp. 94-97Van Der Meché, F.G.A., Schmitz, P.I.M., A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome (1992) N Engl J Med, 326, pp. 1123-1129Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome (1997) Lancet, 349, pp. 225-230Sridharan, G.V., Tallis, R.C., Gautam, P.C., Guillain-Barré syndrome in the elderly: A retrospective comparative study (1993) Gerontology, 39, pp. 170-175Winner, S.J., Evans, J.G., Guillain-Barré syndrome in Oxfordshire: Clinical features in relation to age (1993) Age Ageing, 22, pp. 164-170Yamashita, S., Morinaga, T., Matsumoto, K., Sakamoto, T., Kaku, N., Matsukura, S., Severe Guillain-Barré syndrome in aged patients: The effect of plasmapheresis (1992) Intern Med, 31, pp. 1313-1316Rana, S.S., Rana, S., Intravenous immunoglobulins versus plasmapheresis in older patients with Guillain-Barré syndrome (1999) J Am Geriatr Soc, 47, pp. 1387-1388Asbury, A.K., Cornblath, D.R., Assessment of current diagnostic criteria for Guillain-Barré syndrome (1990) Ann Neurol, 27 (SUPPL.), pp. S21-S24Seneviratne, U., Guillain-Barré syndrome (2000) Postgrad Med J, 76, pp. 774-782Hughes, R.A.C., Newsom-Davis, J.M., Perkin, G.D., Pierce, J.M., Controlled trial of prednisolone in acute polyneuropathy (1978) Lancet, 2, pp. 750-753Rocha, M.S.G., Brucki, S.M.D., Carvalho, A.A.S., Lima, U.W.P., Epidemiologic features of Guillain-Barre syndrome in São Paulo, Brazil (2004) Arq Neuropsiquiatr, 62, pp. 33-37Van Der Meche, F.G., Visser, L.H., Jacobs, B.C., Endtz, H.P., Meulstee, J., Van Doom, P.A., Guillain-Barré syndrome: Multifactorial mechanisms versus defined subgroups (1997) J Infect Dis, 176 (SUPPL. 2), pp. S99-S102Sheth, R.D., Riggs, J.E., Hobbs, G.R., Gutmann, L., Age and Guillain-Barré syndrome severity (1996) Muscle Nerve, 19, pp. 375-377Dias-Tosta, E., Kuckelhaus, C.S., Guillain-Barre syndrome in a population less than 15 years old in Brazil (2002) Arq Neuropsiquiatr, 60, pp. 367-37

Haptoglobin study in myasthenia gravis

Objective: A cross-sectional study of haptoglobin (Hp) in myasthenia gravis (MG) was designed, with, the objective to identify its values and correlate them with different disease status. Method: 46 patients were enrolled in the study, all having disease severity established according to the quantitative myasthenia gravis strength scores (QMGSS). Based on the functional scale determined by Myasthenia Gravis Foundation of America (MGFA) recommendations, patients were classified as having: complete stable remission (CSR; n=10); minimal manifestations-O (MMO; n=6), minimal manifestations-1 (MM1; n=4); pharmacological remission (PR; n=6). Two other groups participated: thymomatous patients (T; n=10) and patients without imunosuppression or thymectomy, until the assessment for Hp (WIT; n=10). Hp dosage was done by immunonephelometry, blindly to clinical data. Student's t-test, Anova test and linear regression were employed for statistical analyses. Results: Statistically significant differences occurred between CSR+MMOxWIT groups (86.62x157.57, p < 0.001) and PR+MM1xWIT groups (73.93x157.57, p < 0.001). Linear regression showed correlation between Hp levels and QMGSS (r=0.759, p < 0.001). Conclusion: Our results suggest that Hp may be useful in clinical practice as a disease severity marker in MG.662A22923

Myositis with sensory neuronopathy

Inflammatory myopathies (IM) are a heterogeneous group of diseases characterized by immune-mediated damage to skeletal muscle. Sensory abnormalities are rare in patients with IM. We report two patients, one with dermatomyositis and the other with inclusion-body myositis, who presented with unexpected sensory abnormalities due to probable immune-mediated damage to dorsal root ganglia. We emphasize the importance of combined neuroimaging and neurophysiological assessment for proper diagnosis.36572172

Brainstem Involvement in Sjogren's Syndrome-Related Sensory Neuronopathy

BACKGROUND AND PURPOSE Sensory neuronopathies (SN) are peripheral nervous system disorders associated with degeneration of dorsal root ganglion neurons. Magnetic resonance imaging (MRI) studies have shown abnormalities limited to T2-weighted high signal intensity in the posterior columns. METHODS AND RESULTS A 65-year-old woman with Sjogren syndrome had slowly progressive unsteadiness of gait and limb paresthesias. A blink reflex examination suggested a paramedian brainstem lesion, confirmed by MRI. CONCLUSIONS Sjogren's syndrome-related SN may be associated with a more diffuse immune-mediated aggression, involving also the brainstem, and leading to some of the blink reflex abnormalities observed in nonparaneoplastic SN.20439739

Autonomic dysfunction in non-paraneoplastic sensory neuronopathy: beyond sensory abnormalities

Sensory neuronopathies (SN) represent a subgroup of peripheral nervous system disorders which are becoming increasingly recognized. Despite a few reports of autonomic dysfunction, this complication has not been fully appreciated. Autonomic function was quantified through tests of sympathetic and parasympathetic function (forming the Autonomic Tests Score, ATS), and through a 40-item questionnaire assessing autonomic symptoms (constituting the Autonomic Questionnaire Score, AQS). Twenty patients were enrolled. Forty-six age- and gender-matched controls were evaluated for the AQS and 15 for the ATS. All patients reported symptoms of autonomic dysfunction. Of the patients, 60% had one or more abnormal cardiovagal test, 60% orthostatic hypotension and 20% abnormal pupillary function. Their ATS was significantly different from the controls (p < 0.0001). Neither the ATS nor AQS were different between groups of SN associated disorders. Autonomic dysfunction is a frequent and important complication in SN, and it does not seem to be related to a specific etiology, as previously thought.258223123

Motor Nerve Hyperexcitability And Muscle Cramps In Machado-joseph Disease - Reply

[No abstract available]661139140Kanai, K., Kuwabara, S., Arai, K., Sung, J.Y., Ogawara, K., Hattori, T., Muscle cramp in Machado-Joseph disease: Altered motor axonal excitability properties and mexiletine treatment (2003) Brain, 126 (PART 4), pp. 965-97

Muscle excitability abnormalities in Machado-Joseph disease

Objectives: To estimate the frequency of and to characterize muscle excitability abnormalities in Machado-Joseph disease (MJD). Design: Machado-Joseph disease is a common autosomal dominant cerebellar ataxia caused by an unstable CAG trinucleotide repeat expansion. Muscle cramps and fasciculations are frequent and sometimes disabling manifestations. However, their frequency and pathophysiological mechanisms remain largely unknown. Symptomatic patients with MJD (hereinafter MJD patients) with molecular confirmation were assessed prospectively. A standard questionnaire addressing clinical features of muscle cramps and fasciculations was used. The Cramps Disability Scale was used to quantify cramps-related disability. Patients underwent neurophysiological testing with routine techniques. F waves of the right median nerves were obtained, and persistence indexes were calculated. Four muscles (deltoid, first dorsal interossei, tibialis anterior, and vastus lateralis) were examined by needle electromyography. A semiquantitative scale (from 0 [no activity] to 4 [continuous activity]) was used to determine the frequency of rest fasciculations in each muscle. Results: Fifty MJD patients (29 men) were included in the study. Their mean age at examination was 46.3 years, their mean age at onset of the disease was 35 years, and the mean duration of disease was 11.2 years. Abnormal CAG(n) varied from 59 to 75 repeats. Forty-one patients presented with muscle cramps; in 10, this was their first symptom. The frequency of cramps varied between I and 90 episodes a week. For 15 patients, cramps were the chief complaint, frequently disturbing sleep or work (Cramps Disability Scale score, 2 or 3). Lower limbs were affected in 37 individuals, but unusual regions, such as the face and abdominal muscles, were also involved. Fasciculations were found in 25 individuals; in 8 patients, they included facial muscles. However, fasciculations were not a significant complaint for any of these patients. The clinical and neurophysiological profile of MJD patients with and without cramps was not significantly different. However, MJD patients with fasciculations; had more severe damage to their peripheral nerves. Conclusions: Muscle excitability abnormalities were found in 41 MJD patients (82%), and they were the presenting complaint in 10 (20%). They are related to altered excitability of peripheral motor axons, but mechanisms underlying cramps and fasciculations are possibly distinct in MJD patients.65452552