- Email: [email protected]
P300 and PET in cortical and subcortical dementia
Society supposed illness duration. The modification gest an initial CNS impairment. 43.
of this parameter
can sug-
Ascending paralysis in AIDS patient: Guillain Barre syndrome or cytomegalovirus (CMV) polyradiculopathy? - S. Medaglini, C. Fornara, L. Maderna, G. Galardi, L. Fumagallia, P. Cinquea, A. Lazzarina, N. Canal, G. Comi (Neurologic Department and aInfectious Diseases Department, IRCCS San Raffaele, University of Milan, Italy)
Polyradiculopathy caused by cytomegalovirus is a rare but serious neurological disorder that occurs late in the course of HIV infection. Recently the identification of CMV infection by PCR (polymerase chain reaction) methodology consents an early diagnosis and treatment. We describe the case of a patient 25 years old, ex i.v. drug abuser, severely immunocompromised (CDC Atlanta IVCl) who consulted for limb weakness, generalized pain and sphincter disorders of a 2 weeks evolution. The EMG-ENG neurophysiologic study executed at hospital admission suggested a diagnosis of acute pohneuropathy. Neutrophilic pleiocytosis (N83%, L12%, M5%) and high levels of proteins (9lmg/dl) were observed on CSF. CMV was identified on CSF by PCR and positive were blood titres of CMV p65/p72 (130-3). Eight days after, the patient developed paraplegia of lower limbs with tendon areflexia, weakness in the thoracic and cervical segments, mononeuritis of VI cranic nerve. Suspecting a CMV polyradiculopathy the patient started therapy with ganciclovir at induction dose (5 mg/kg, iv., every 12 h). A significant clinical improvement was evident after 1 month of therapy and the patient reached a complete recovery after 2 months of therapy. The CSF pleocytosis normalized upon treatment, while proteins persisted elevated. At follow-up of 6 months CMV-DNA was positive on CFS, while blood CMV titres maintained negative. The described case takes relevant problems for the differential diagnosis while PCR-based methods for detecting CMV in CSF can be considered sensitive and specific techniques. 44.
P300 and PET in cortical and subcortical dementia. - T. Locatelli, S. Medagliui, V. Martinelli, D. Perani, M. Rovaris, G. Magnani, M. Cursi, F. Grassi, F. Fazio, G. Comi (Neurology and Medicine Nuclear Depts., IRCCS H.S.Raffaele, University of Milan)
We analyzed changes of acoustic P300, recorded with a classical odd-ball paradigm, and cerebral metabolism (18F-FDG-PET) occurring in two different kinds of dementia in order to better clarify the specific role of these two techniques. We studied 10 patients affected by probable Alzheimer’s disease (AD) and 30 patients affected by multiple sclerosis (MS). in the AD group P300 was recognizable only in 4 patients, while PET showed a significant hypometabolism over parietotemporal regions in all patients and frequently a frontal involvement also; the patients with P300 had lower degree of hypomethabolism. In the MS group, P300 was abnormal in 57% of the cases and PET in 26%. A positive relationship was present between glucose consumption and P300 amplitude. Moreover patients with an abnormal P300 had lower rates of metabolic consumption, particularly in thalamic and temporo-parietal regions. Differently from what happens in AD, in subcortical dementia P300 is more sensitive than PET in the detection of functional abnormalities and the involvement of specific relays, as a thalamic structure, plays an important role in the correct genesis of this component.
Proceedings
group. Some of these patients were categorized together by Welander and Miyoshi because of their shared clinical features and geographic distribution. We present a 28-year-old Italian woman without a family history of neuromuscular disease with similar clinical and pathological features as seen in Welander’s cases. Her neuroEogica1 examination revealed: bilateral pes cavus, distal symmetrical leg muscular atrophy without fasciculations and marked extensor and mild flexor weakness of legs. All deep tendon reflexes were elicited only with facilitatory maneuvers. The sensory exam was normal. Investigation revealed: slightly elevated serum CK levels; normal cerebrospinal fluid analysis; ECG: incomplete A-V conduction defect; echocardiography: slight mitral insufficiency; ECG Holter: diurnal sinoatrial tachycardia. The EMG showed: (1) abundant spontaneous activities (fibrillations, PSWs,); (2) low amplitude and polyphasic motor units in the affected muscles; (3) myopathic interference pattern; (4) normal motor and sensory conductions. The sural nerve biopsy was normal. Muscle biopsy of the right tibial anterior revealed myopathic features with rimmed vacuoles. Our case resembles Welander’s cases based on similar clinical features, mild CK elevation, and the presence of rimmed vacuoles on muscle biopsy. However our case has a sporadic ptesentation and an unusual geographic distribution. 46.
Distal myophatby with rimmed vacuoles: a sporadic case. (M. Santangelo, V. Miele, V. Lolli, A.P. Strafellaa, A. Tropeanf’, D. Lucchi”, F. Valzanlaa (Divisione di Neurologia, Ospedale Civile, Carpi. aDivisione di Neurologia, Ospedale Bellaria, Bologna) Myopathic
disorders
with
distal
distribution
am a heterogeneous
Modulation of postural tremor with traitscranial magnetic stimulation in Parkinson’s disease. - F. Valzania, R. Quatralea, A.P. Strafella, M. Santangelob, A. Tropeani, D. Lucchi, R. Bombardia, P. Passadorea, C.A. Tassinari, D. De GraudisC @ivisione di Neurologia, Ospedale Bellaria, Bologna. aClinica Neurologica, Ospedale S. Anna, Ferrara. bDivisione di Neurologia, Ospedale Civile, Carpi. ‘Divisione di Neurologia, Ospedale S. Anna, Ferrara)
In 10 patients with Parkinson’s disease (PD) (6M, 4F), mean age 62.8, we have studied the effect of transcranial magnetic stimulation (SMT) on postural tremor of the wrist, and its relation with the silent period (SP). In 4 of these patients, we also studied the response to paired transcranial magnetic stimuli during the tremor at intervals of interstimulus (ISI) 50, 100, 150 ms. The intensity of stimulation was of 12&140% of threshold and the tremor response was recorded from ECR and FCR muscles. Results: mean period of postural tremor: 156.2 ms (range 109-243); mean latency of first bust EMG post-MEP: 160.5 ms (118-255); mean duration of SP: 163 ms (I 16-262). A significant linear correlation was shown between the period of the tremor and the latency of first burst EMG post-MEP (r 0.8 I ; P < 0.001) as well as between the period of the tremor and the duration of SP (r 0.75; P < 0.001). With the paired stimulus test the conditioning MEP amplitude changed in relation to phase of tremor while the MEP test, at the same ISI, had a constant amplitude. The significant relationship between the period of tremor and the EMG rebound post-SMT suggests that the magnetic stimulus produces a ‘reset’ of the oscillatory mechanism which is responsible for the postural tremor. This reset occurs repeatedly at the same point at the cycle, and is fallowed by a resumption of the oscillatory mechanism. This phenomenon was confirmed by paired stimulus test. The relationship between tremor and SP suggests two considerations: (1) probably the SMT might produce a reset of the postural tremor acting on the cortical interneuron population, which is responsible for the late component of SP; (2) the SP could be strongly influenced by pallido-thalamo-cortical projections which produce the postural tremor. 47.
45.
63P
Sleep-wake transition spinal myoclonus, - F. Provini, G. Plazzi, R. Liguori, S. Macri, R. Corsini, K. Donati, E. Lugaresi, P. Montagna (Clinica Neurologica, Univ. Bologna)
We describe 3 healthy males of 41, 50 and 7 1 years presenting from 5, 20, and 31 years, respectively, with massive muscle jerks upon relaxation and drowsiness. Involuntary movements typically arise when patients lie down, causing insomnia. Two patients responded to
of this parameter
can sug-
Ascending paralysis in AIDS patient: Guillain Barre syndrome or cytomegalovirus (CMV) polyradiculopathy? - S. Medaglini, C. Fornara, L. Maderna, G. Galardi, L. Fumagallia, P. Cinquea, A. Lazzarina, N. Canal, G. Comi (Neurologic Department and aInfectious Diseases Department, IRCCS San Raffaele, University of Milan, Italy)
Polyradiculopathy caused by cytomegalovirus is a rare but serious neurological disorder that occurs late in the course of HIV infection. Recently the identification of CMV infection by PCR (polymerase chain reaction) methodology consents an early diagnosis and treatment. We describe the case of a patient 25 years old, ex i.v. drug abuser, severely immunocompromised (CDC Atlanta IVCl) who consulted for limb weakness, generalized pain and sphincter disorders of a 2 weeks evolution. The EMG-ENG neurophysiologic study executed at hospital admission suggested a diagnosis of acute pohneuropathy. Neutrophilic pleiocytosis (N83%, L12%, M5%) and high levels of proteins (9lmg/dl) were observed on CSF. CMV was identified on CSF by PCR and positive were blood titres of CMV p65/p72 (130-3). Eight days after, the patient developed paraplegia of lower limbs with tendon areflexia, weakness in the thoracic and cervical segments, mononeuritis of VI cranic nerve. Suspecting a CMV polyradiculopathy the patient started therapy with ganciclovir at induction dose (5 mg/kg, iv., every 12 h). A significant clinical improvement was evident after 1 month of therapy and the patient reached a complete recovery after 2 months of therapy. The CSF pleocytosis normalized upon treatment, while proteins persisted elevated. At follow-up of 6 months CMV-DNA was positive on CFS, while blood CMV titres maintained negative. The described case takes relevant problems for the differential diagnosis while PCR-based methods for detecting CMV in CSF can be considered sensitive and specific techniques. 44.
P300 and PET in cortical and subcortical dementia. - T. Locatelli, S. Medagliui, V. Martinelli, D. Perani, M. Rovaris, G. Magnani, M. Cursi, F. Grassi, F. Fazio, G. Comi (Neurology and Medicine Nuclear Depts., IRCCS H.S.Raffaele, University of Milan)
We analyzed changes of acoustic P300, recorded with a classical odd-ball paradigm, and cerebral metabolism (18F-FDG-PET) occurring in two different kinds of dementia in order to better clarify the specific role of these two techniques. We studied 10 patients affected by probable Alzheimer’s disease (AD) and 30 patients affected by multiple sclerosis (MS). in the AD group P300 was recognizable only in 4 patients, while PET showed a significant hypometabolism over parietotemporal regions in all patients and frequently a frontal involvement also; the patients with P300 had lower degree of hypomethabolism. In the MS group, P300 was abnormal in 57% of the cases and PET in 26%. A positive relationship was present between glucose consumption and P300 amplitude. Moreover patients with an abnormal P300 had lower rates of metabolic consumption, particularly in thalamic and temporo-parietal regions. Differently from what happens in AD, in subcortical dementia P300 is more sensitive than PET in the detection of functional abnormalities and the involvement of specific relays, as a thalamic structure, plays an important role in the correct genesis of this component.
Proceedings
group. Some of these patients were categorized together by Welander and Miyoshi because of their shared clinical features and geographic distribution. We present a 28-year-old Italian woman without a family history of neuromuscular disease with similar clinical and pathological features as seen in Welander’s cases. Her neuroEogica1 examination revealed: bilateral pes cavus, distal symmetrical leg muscular atrophy without fasciculations and marked extensor and mild flexor weakness of legs. All deep tendon reflexes were elicited only with facilitatory maneuvers. The sensory exam was normal. Investigation revealed: slightly elevated serum CK levels; normal cerebrospinal fluid analysis; ECG: incomplete A-V conduction defect; echocardiography: slight mitral insufficiency; ECG Holter: diurnal sinoatrial tachycardia. The EMG showed: (1) abundant spontaneous activities (fibrillations, PSWs,); (2) low amplitude and polyphasic motor units in the affected muscles; (3) myopathic interference pattern; (4) normal motor and sensory conductions. The sural nerve biopsy was normal. Muscle biopsy of the right tibial anterior revealed myopathic features with rimmed vacuoles. Our case resembles Welander’s cases based on similar clinical features, mild CK elevation, and the presence of rimmed vacuoles on muscle biopsy. However our case has a sporadic ptesentation and an unusual geographic distribution. 46.
Distal myophatby with rimmed vacuoles: a sporadic case. (M. Santangelo, V. Miele, V. Lolli, A.P. Strafellaa, A. Tropeanf’, D. Lucchi”, F. Valzanlaa (Divisione di Neurologia, Ospedale Civile, Carpi. aDivisione di Neurologia, Ospedale Bellaria, Bologna) Myopathic
disorders
with
distal
distribution
am a heterogeneous
Modulation of postural tremor with traitscranial magnetic stimulation in Parkinson’s disease. - F. Valzania, R. Quatralea, A.P. Strafella, M. Santangelob, A. Tropeani, D. Lucchi, R. Bombardia, P. Passadorea, C.A. Tassinari, D. De GraudisC @ivisione di Neurologia, Ospedale Bellaria, Bologna. aClinica Neurologica, Ospedale S. Anna, Ferrara. bDivisione di Neurologia, Ospedale Civile, Carpi. ‘Divisione di Neurologia, Ospedale S. Anna, Ferrara)
In 10 patients with Parkinson’s disease (PD) (6M, 4F), mean age 62.8, we have studied the effect of transcranial magnetic stimulation (SMT) on postural tremor of the wrist, and its relation with the silent period (SP). In 4 of these patients, we also studied the response to paired transcranial magnetic stimuli during the tremor at intervals of interstimulus (ISI) 50, 100, 150 ms. The intensity of stimulation was of 12&140% of threshold and the tremor response was recorded from ECR and FCR muscles. Results: mean period of postural tremor: 156.2 ms (range 109-243); mean latency of first bust EMG post-MEP: 160.5 ms (118-255); mean duration of SP: 163 ms (I 16-262). A significant linear correlation was shown between the period of the tremor and the latency of first burst EMG post-MEP (r 0.8 I ; P < 0.001) as well as between the period of the tremor and the duration of SP (r 0.75; P < 0.001). With the paired stimulus test the conditioning MEP amplitude changed in relation to phase of tremor while the MEP test, at the same ISI, had a constant amplitude. The significant relationship between the period of tremor and the EMG rebound post-SMT suggests that the magnetic stimulus produces a ‘reset’ of the oscillatory mechanism which is responsible for the postural tremor. This reset occurs repeatedly at the same point at the cycle, and is fallowed by a resumption of the oscillatory mechanism. This phenomenon was confirmed by paired stimulus test. The relationship between tremor and SP suggests two considerations: (1) probably the SMT might produce a reset of the postural tremor acting on the cortical interneuron population, which is responsible for the late component of SP; (2) the SP could be strongly influenced by pallido-thalamo-cortical projections which produce the postural tremor. 47.
45.
63P
Sleep-wake transition spinal myoclonus, - F. Provini, G. Plazzi, R. Liguori, S. Macri, R. Corsini, K. Donati, E. Lugaresi, P. Montagna (Clinica Neurologica, Univ. Bologna)
We describe 3 healthy males of 41, 50 and 7 1 years presenting from 5, 20, and 31 years, respectively, with massive muscle jerks upon relaxation and drowsiness. Involuntary movements typically arise when patients lie down, causing insomnia. Two patients responded to