Correlation between EEG and Clinicopathological Change in Neuro-Behçet's Syndrome

Abstract: The serial EEGs on 10 cases of neuro-Behcet's syndrome have been investigated and were compared with the clinical symptomatology and autopsy findings in 4 cases. In 8 cases, the EEGs showed a mild to moderate increase in slowed alpha and theta waves and in 5 cases diffuse alpha patterns. In 2 cases, large slow wave patterns were observed and in Case 10, large slow waves changed into low voltage fast dominant patterns following the status epilepticus. In all the cases, the EEG changes seemed to be well correlated with the clinical symptoms. Our present study suggested that the diffuse alpha patterns were only seen in cases with the least affected brain stem diffuse projection system. From the investigation of the sequential alterations of large slow waves in Case 10, it was suggested that a synchronization mechanism would lie in the medulla oblongata.     

Progressive Dementia Associated with Sheehan's Syndrome –An Autopsied Case–

Abstract: This is a report of the study of progressive dementia associated with Sheehan's syndrome in a 61-year-old woman who had a 13-year history of insidious progressive decline in g'obal cognitive abilities. At the onset, there were only slight difficulties with abstract thoughts, and a shortened attention span, but the symptoms gradually increased in severity until death at the age of 61. The clinical deficits fluctuated in severity. During the last years of life she was at all times confused, apathetic, and incapable of self-care. A necropsy revealed a moderately severe neuronal loss in the central nervous system with marked destruction of the anterior pituitary lobe, and severe atrophy of the thyroid and adrenal glands. No senile plaques or neurofibrillary tangles were observed in the cerebral cortex. Possible pathophysiological mechanisms, which may have been present in this case, could be the hypometabolic effects on the central nervous system, mediated through a multiendocrine system malfunction, caused by postpartum uterine hemorrhage and a marked disappearance of the pituitary anterior lobe cells.     

Idiopathic Spinal Cord Herniation as a Treatable Cause of Progressive Brown-Sequard Syndrome

Idiopathic spinal cord herniation is a rare spinal cord disorder caused by spinal cord prolapse through a adural defect. It is a curable disease, so early detection is of particular importance. We report a 38-year-old woman with Brown-Sequard syndrome which was caused by the thoracic spinal cord herniation. Her weakness was almost completely resolved after surgical management, which emphasizes the importance of early diagnosis and surgical management in this rare disease entity.     

Evolutional Changes and Outcome of West Syndrome: Correlation with Magnetic Resonance Imaging Findings

Summary: The prognosis and evolutional changes of 77 patients with West syndrome (WS) were studied after patients were classified into four groups on the basis of their magnetic resonance imaging (MRI) findings: anomaly, perinatal injury, normal, and the other groups. The average age at onset of spasms was earliest in the patients with anomalies and latest in patients with normal MRI findings. Patients with normal MRI findings had the shortest duration of spasms, and patients with anomalies had the longest duration of spasms. Antecedent seizures were observed in 6 patients (3 patients with anomalies, 1 patient with normal MRI findings, and 2 patients with other abnormalities). Thirty-five patients had subsequent seizures. Patients with anomalies often had partial seizures and patients with perinatal injuries often had generalized seizures. Seizures were infrequent in patients with normal MRI findings. Developmental outcome was best in the patients with normal MRI findings and worst in patients with perinatal injuries. Various types of epileptic syndromes occurred subsequent to WS in patients with anomalies, although nonspecific symptomatic generalized epilepsy was common in patients with perinatal injuries. These results suggest that seizure prognosis, evolutional changes in seizures, and developmental outcome are different among the types of brain lesions.     

Pressure Measurement in Carpal Tunnel Syndrome : Correlation with Electrodiagnostic and Ultrasonographic Findings

Objective

This study was done to evaluate the correlation between carpal tunnel pressure (CTP), electrodiagnostic and ultrasonographic findings in patients with carpal tunnel syndrome (CTS).

Methods

CTP was measured during endoscopic carpal tunnel release (ECTR) for CTS using Spiegelberg ICP monitoring device with parenchymal type catheter. Neurophysiologic severity and nerve cross sectional area were evaluated using nerve conductive study and ultrasonography (USG) before ECTR in all patients.

Results

Tests were performed in a total of 48 wrists in 39 patients (9 cases bilateral). Maximum CTP was 56.7 ± 19.3 mmHg (Mean ± SD) and 7.4 ± 3.3 mmHg before and after ECTR, respectively. No correlation was found between maximum CTP and either neurophysiologic severity or nerve cross sectional area, whereas we found a significant correlation between the latter two parameters.

Conclusion

CTP was not correlated with neurophysiologic severity and nerve cross sectional area. Dynamic, rather than static, pressure in carpal tunnel might account for the basic pathophysiology of CTS better.     

Progressive bulbar paralysis in childhood: a case report

The case of a progressive bulbar paresis in a nine and a half year old child is reported. The first symptoms were present at birth; however the subsequent evolution was very slow. Lesion of the motor nuclei of the V, VII, IX, XII, cranial nerves was evident on electromyographic investigation. Damage to the acoustic brain stem pathway was documented by the brain stem evoked potentials although audiometry was normal. No other neuronal systems or districts appeared to be damaged. The case suggests Fazio-Londe disease, although the involvement (albeit partial) of the auditory pathways recalls Van Laere syndrome. This supports the view that motor neuron disease in infancy is not an autonomous entity but a variant in a wide spectrum of progressive neuronal diseases.

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Sommario Viene presentato un caso di paralisi bulbare progressiva osservata in una bambina di 9 anni. I primi sintomi si sono manifestati già immediatamente dopo la nascita e la successiva evoluzione è stata molto lenta. La lesione dei nuclei motori del V, VII, IX, XII nervo cranico depone per una malattia di Fazio-Londe; tuttavia le alterazioni riscontrate a carico delle vie acustiche troncoencefaliche richiamano la sindrome di Van Laere. Non appaiono essere danneggiati altri sistemi neuronali. Tali reperti appaiono essere a sostegno delle tesi di quegli Autori che considerano la malattia del neurone di moto nell'infanzia non come entità autonoma ma come ulteriore variante nell'ampio spettro delle malattie progressive del neurone.

     

Psychosis following chloroquine ingestion: a 10-year comparative study from a malaria-hyperendemic district of India

Objectives

Serious adverse effects such as acute psychoses have been reported following treatment with chloroquine. Chloroquine can cause cell death, including neurons. We aimed to identify the most frequent type of psychiatric manifestation and symptomatological characteristics of psychosis following chloroquine ingestion (PFC).

Method

Out of a total of 4471 randomly selected recent-onset psychosis patients, 3610 consecutive patients who had responded to standard treatment were screened for entry in the study. We compared background clinicodemographic profile information and psychopathology of 51 PFC patients, who were either drug free or drug naive, to 51 brief psychotic disorder (BPD) patients who were matched in terms of age, sex and education. Only those patients who remitted within 8 weeks (PFC patients) or 4 weeks (BPD patients) were included. Cranial computed tomography, electroencephalography and lumbar puncture of the entire experimental group were normal, and none had Mini Mental Status Examination score < 22. Group difference and correlational statistics (parametric and nonparametric) have been used to test the hypotheses and explain the results.

Results

The most common (76.2%) type of psychiatric disturbance in PCF group was mood disorder (mixed episode) accompanied by predominant irritability with little blunting of affect. PFC patients characteristically had prominent positive symptoms with visual hallucination and derealization experiences. They were more restless, agitated and anxious and had more disturbed thought content and orientation, but better preserved insight. There was no linear relationship between the amount of chloroquine consumed and the severity of psychosis.

Conclusion

Considering the large number of patients still receiving chloroquine especially in developing countries, this study has been presented to draw attention of the psychiatrists and other health professionals to the hazardous effect of chloroquine on mental health.     

Value of Ultrasonography in the Diagnosis of Carpal Tunnel Syndrome: Correlation with Electrophysiological Abnormalities and Clinical Severity

Objective

To investigate a diagnostic value of ultrasonography in carpal tunnel syndrome (CTS) patients and to evaluate a correlation of sonographic measurements with the degree of electrodiagnostic abnormalities and clinical severity.

Methods

Two-hundred-forty-six symptomatic hands in 135 patients and 30 asymptomatic hands in 19 healthy individuals as control group were included. In ultrasonographic study, we measured the cross-sectional area (CSA) and flattening ratio (FR) of the median nerve at the pisiform as well as palmar bowing (PB) of the flexor retinaculum. Sensitivity and specificity of ultrasonographic measurements were evaluated and ultrasonographic data from the symptomatic and control hands were compared to the grade of electrodiagnostic and clinical severity.

Results

The mean CSA was 13.7±4.2 mm² in symptomatic hands and 7.9±1.3 mm² in asymptomatic hands. The mean FR was 4.2±1.0 in symptomatic hands and 3.4±0.4 in asymptomatic hands. The mean PB was 3.5±0.5 mm in symptomatic hands and 2.6±0.3 mm in asymptomatic hands. Statistical analysis showed differences of the mean CSA, FR and PB between groups were significant. A cut-off value of 10 mm² for the mean CSA was found to be the upper limit for normal value. Both the mean CSA and PB are correlated with the grade of electrophysiological abnormalities and clinical severity, respectively.

Conclusion

Ultrasographic measurement of the CSA and PB is helpful to diagnose CTS as a non-invasive and an alternative modality for the evaluation of CTS. In addition, ultrasonography also provides a reliable correlation with the grade of electrodiagnostic abnormalities and clinical severity.     

Persistent, severe hypoglycemia-induced organic brain syndrome with neurological sequelae: a case report

Hypoglycemia is a biochemical abnormality and often the rate-limiting step in the treatment of both type 1 and type 2 diabetes mellitus. Left uncorrected and prolonged, hypoglycemia can result in neuronal dysfunction and death, with deficits ranging from measurable cognitive impairments to aberrant behavior, seizures and coma. In this case report, hypoglycemia resulted in severe and persistent neurological (slurred speech and gait abnormalities), cognitive (inattention, disorientation and memory deficits) and behavioral manifestations (verbal hostility and irritability). It highlights the potentially severe neuropsychiatric sequelae following hypoglycemia and is timely for clinicians to be reminded that hypoglycemia prevention needs to be more of a focus of diabetes care in general.     

Kufs''Disease Presenting as Progressive Dementia with Late-Onset Generalized Seizures: A Clinicopathological and Electrophysiological Study

A 32-year-old woman presented with increasing motor difficulties and memory disturbances. Neurological examination only showed mild cerebellar and extrapyramidal symptoms, whereas neuropsychological evaluation disclosed severe cognitive changes consistent with dementia. Her motor and mental status progressively deteriorated until death, which occurred 5 years after the first admission. One year before death, while she was almost bedridden, symptoms of myoclonic epilepsy first appeared, with frequent generalized seizures and generalized myoclonus, occurring especially upon sensory stimulation or passive joint movements. Pathological examination showed neuronal inclusions typical of Kufs' disease. This case, with primary progressive dementia and late-onset myoclonic epilepsy, differs from previously reported cases. Three special electrophysiological features were abnormal, "giant", evoked potentials; unusually marked photosensitivity; and seizure induction by any sensory stimulation.     

Epilepsy and the Electroencephalogram in Progressive Encephalopathy with Edema, Hypsarrhythmia, and Optic Atrophy (the PEHO Syndrome)

Summary: Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome) is an apparently autosomal recessive disorder manifested by infantile spasms, severe hypotonia, and early arrest of psychomotor development. Subcutaneous edema in the limbs, typical facial features, and blindness with optic atrophy are also present. Neuropathologic and radiographic studies show progressive brain atrophy, which is accentuated infratentorially. We recorded 85 EEGs from 10 patients between the ages of 3 weeks and 12.7 years; follow-up ranged from 7 months to 12.1 years. The infantile spasms were preceded by other neurological symp- toms in all patients. Seven of nine patients showed focal or generalized epileptiform activity or abnormal EEG background. All patients developed hypsarrhythmia, first recorded between 3 and 11 months of age, that was resistant to therapy with ACTH and antiepileptic drugs. After the hypsarrhythmia disappeared, five patients showed slow spike-wave activity generally seen in the Lennox-Gastaut syndrome, and three patients showed background EEG abnormality with generalized or diffuse paroxysmal activity. There were no specific EEG features that could help in the diagnosis of PEHO. The PEHO syndrome should be borne in mind in the diagnostic work-up of patients with infantile spasms, so that potentially harmful treatment can be avoided, and the parents can be counseled about the inheritability of the disorder.     

干燥综合征并发帕金森综合征1例及文献复习

目的：探讨干燥综合征和帕金森综合征之间的关系。方法：经临床症状和右侧唇腺病理确诊的1例干燥综合征患者合并出现帕金森综合征样表现,结合国内外文献报道对其进行分析。结果：干燥综合征患者并发中枢神经系统病变并不少见,但合并出现帕金森综合征的不多见,国内尚未见报道。予以甲泼尼龙0.5g冲击治疗3d后,患者眼干、口干症状改善,且帕金森综合征表现也较前好转。结论：干燥综合征并发帕金森综合征样临床表现,文献报道均为女性,其发病机制有待进一步研究。治疗方法与其他帕金森综合征不尽相同,目前主张激素治疗,但疗效不一。     

Progressive Manifestations of Reversible Cerebral Vasoconstriction Syndrome Presenting with Subarachnoid Hemorrhage,Intracerebral Hemorrhage,and Cerebral Infarction

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset headache with focal neurologic deficit and prolonged but reversible multifocal narrowing of the distal cerebral arteries. Stroke, either hemorrhagic or ischemic, is a relatively frequent presentation in RCVS, but progressive manifestations of subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction in a patient is seldom described. We report a rare case of a 56-year-old woman with reversible cerebral vasoconstriction syndrome consecutively presenting as cortical subarachnoid hemorrhage, intracerebral hemorrhage, and cerebral infarction. When she complained of severe headache with subtle cortical subarachnoid hemorrhage, her angiography was non-specific. But, computed tomographic angiography showed typical angiographic features of this syndrome after four days. Day 12, she suffered mental deterioration and hemiplegia due to contralateral intracerebral hematoma, and she was surgically treated. For recurrent attacks of headache, medical management with calcium channel blockers has been instituted. Normalized angiographic features were documented after 8 weeks. Reversible cerebral vasoconstriction syndrome should be considered as differential diagnosis of non-aneurysmal subarachnoid hemorrhage, and repeated angiography is recommended for the diagnosis of this under-recognized syndrome.     

Diagnostic Significance of Ultrasonographic Measurements and Median-Ulnar Ratio in Carpal Tunnel Syndrome: Correlation with Nerve Conduction Studies

MethodsThe pisiform CSA (CSA_pisiform), swelling ratio (SR), palmar bowing, and CSA_pisiform/ulnar CSA (CSA_ulnar) measurements made in two subgroups of CTS patients (having sensory affection alone or having both sensory and motor affection) were compared with controls. CSA_ulnar was measured in Guyon''s canal at the level of most-protuberant portion of the pisiform bone.ResultsThe values of all of the measured US parameters were higher in patients with CTS (n=50) than in controls (n=62). CSA_pisiform could be used to diagnose CTS of mild severity. All of the parameters were positively correlated with the distal latency of the compound muscle action potential, and all of them except for SR were negatively correlated with the sensory nerve conduction velocity. A CSA_pisiform/CSA_ulnar ratio of ≥1.79 had a sensitivity of 70% and a specificity of 76% for diagnosing CTS.ConclusionsOnly CSA_pisiform measurements were reliable for diagnosing early stages of CTS, and CSA_pisiform/CSA_ulnar had a lower diagnostic value for diagnosing CTS.     

A Progressive Neurologic Disorder with Multiple CNS Lesions: A Neuroimaging Clinicopathologic Correlation

A 51‐year‐old man with a diagnosis of myelodysplasia and non‐Hodgkin's lymphoma underwent an unmatched allogenic bone marrow transplantation and was treated posttransplant with chronic immunosuppressive medication. Eight months following transplantation, he presented with progressive dysarthria, cognitive and visual decline. Evaluation included brain magnetic resonance (MR) imaging demonstrating multifocal areas of increased T2 and FLAIR (fluid attenuated inversion recovery) signals involving the left frontal, parietal, and occipital lobes. The MR lesions demonstrated diffuse increased signal on DWI (diffusion‐weighted images) and normal to low signal on ADC (apparent diffusion coefficients). Contrast‐enhanced T1 images were unremarkable. Lumbar puncture revealed a mild elevation in cerebrospinal fluid (CSF) protein. CSF PCR assay for viral DNA fragments were negative on two occasions. Serum serology for HIV was negative as well. A brain biopsy was subsequently performed. The clinical and neuroimaging differential diagnoses as well as neuropathologic correlation are presented.     

Progressive motor syndrome in a welder with pallidal T1 hyperintensity on MRI: A two-year follow-up.

Chronic exposure to manganese (Mn) fume during welding may lead to mainly extrapyramidal syndrome that is resistant to treatment. We present a 32-year-old patient who developed severe postural instability, Parkinsonism, dystonia, and pyramidal signs in the 10th year of welding. The neurological condition of the patient worsened markedly in the following 3 years, resulting in severe disability rendering him to be assisted in all his daily activities and he did not benefit from any dopaminergic agent. T1 sequences of the MRI of the brain showed pallidal hyperintensity symmetrically. Welders in our country often protect their eyes but ignore to use tools that protect them from inhalation of the fume. Since chronic Mn toxicity may cause serious disability and irreversible neurological disturbances, we strongly believe that it is necessary to inform welders and their employers about this potential hazard.     

Correlation between Ultrasonography Findings and Electrodiagnostic Severity in Carpal Tunnel Syndrome: 3D Ultrasonography

Background and Purpose

To determine the correlation between the cross-sectional area (CSA) of the median nerve measured at the wrist using three-dimensional (3D) ultrasonography (US) and the electrophysiological severity of carpal tunnel syndrome (CTS).

Methods

We prospectively examined 102 wrists of 51 patients with clinical CTS, which were classified into 3 groups according to the electrodiagnostic (EDX) findings. Median nerve CSAs were measured using 3D US at the carpal tunnel inlet and at the level of maximal swelling.

Results

Ten wrists were negative for CTS. Of the 92 CTS-positive wrists, 23, 30, and 39 were classified as having mild, moderate, and severe CTS, respectively. The median nerve CSA differed significantly between the severe- and moderate-CTS groups (p=0.0007 at the carpal tunnel inlet and p<0.0001 at the maximal swelling site). There was a correlation between median nerve CSA and EDX parameters among those wrists with severe and mild CTS (p<0.0001 at both sites).

Conclusions

The median nerve CSA as measured by 3D US could provide additional information about the severity of CTS, as indicated by the strong correlation with standard EDX findings.     

4p- Syndrome: A Chromosomal Disorder Associated with a Particular EEG Pattern

Summary: We report an electroclinical and cytogeneticstudy of 4 patients with Wolf-Hirschhorn syndrome (WHS). In all cases, we observed a stereotyped EEG and clinical picture characterized by generalized or unilateral myoclonic seizures followed later by brief atypical absences. Electrographically, these were accompanied by a sequence of centroparietal or parietotemporal sharp waves; high-voltage wave with a superimposed spike becoming unusual spike-wave complexes, often elicited byeye closure; bursts of diffuse spikes and waves; and frequent jerks. This electroclinical pattern is very similar to the one described in Angelman syndrome (AS) in which a defect in GABA_A receptor function has been suggested. Moreover, the genes encoding the GABA_A receptor subunit have been mapped to the p12–pl3 bands of chromosome 4. Even though the deletion in these cases does not encompass the 4p12–p13 region, we suggest that the electroclinical picture common to WHS and AS might represent a characteristic type of epilepsy linked to a common genetic abnormality.