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Chronic epilepsy with complex partial seizures is not always medically intractable--a long-term observational study.

OBJECTIVE: To study the prognosis of patients with complex partial seizures (CPS) with or without simple partial (SPS) and secondarily generalized tonic-clonic seizures (GTCS) and to analyze the factors related to the degree of medical responsiveness. MATERIAL AND METHODS: A total of 266 adult patients with CPS were included in a hospital based observational survey with a follow-up of 2 to 25 years. Clinical characteristics, seizure frequency, electroencephalography (EEG), cerebral computed tomography (CCT) and magnetic resonance imaging (MRI) findings were analyzed. Patients were categorized according to their degree of medical responsiveness into one of three groups: seizure free, improved control (>50% seizure reduction) and poor control. RESULTS: Mean age at follow-up was 44.7 years (SD 14.7, range 19-93). Mean age at seizure onset was 18.1 years (SD 14.7, median 15, range 1-79). Complete seizure control was achieved in 40%, improved seizure control in 36% and poor seizure control in 24%. Patients entered remission after a mean period of 15.7 years (SD 12.6, median 13, range 1-54) of active epilepsy. A third of all seizure-free patients were still in remission 6.1 years (SD 5.3, median 3.5, range 1-18) after discontinuation of antiepileptic drugs (AED). Patients with poor seizure control had a significantly younger age at onset (P<0.01), a higher initial seizure frequency (more than 3 per month) (P<0.01), abnormal neurological examination (P<0.01), and were more often mentally handicapped (P<0.01). Multiple logistic regression analysis revealed a high initial seizure frequency, mental handicap and an abnormal neurological examination as independent risk factors for poor seizure control. A positive family history, a history of febrile convulsions and/or psychosis, an abnormal EEG or MRI was not predictive of poor outcome. CONCLUSIONS: Not all patients with CPS were medically intractable. Seizure remission can be achieved after a long time of active epilepsy. Poor seizure control was associated with a high initial seizure frequency, mental handicap and abnormal neurological examination.

Adult↗

[Two siblings with congenital adrenocortical unresponsiveness to ACTH showing peripheral neuropathy--morphometric evaluation of the sural nerve].

A 13-year-old boy, with the diagnosis of congenital adrenocortical unresponsiveness to ACTH (ACTH insensitivity) at age 7, developed a steppage gait, when under glucocorticoid replacement therapy at age 13. The parents were healthy and not consanguinous. On general physical examination, a mild diffuse skin hyperpigmentation was noted. Neurological examinations revealed that all the muscle stretch reflexes of both limbs were absent without pathologic reflexes. Pes cavus was found bilaterally. A slight decrease of tactile sensation was noted distal to the ankle joints. Pain sensation was slightly decreased in the toes. On laboratory examination, the conduction velocities of the left ulnar and median motor nerves were 51 and 45 m/sec, respectively, which are normal. No M-wave responses were obtained by electrical stimulation of the tibial and peroneal nerves. The coefficient of the variation of the R-R interval from ECG recordings was normal. Orthostatic hypotension was not observed. Achalasia was negative on the barium swallow esophagram. Therefore, it was concluded that he had motor and sensory polyneuropathy, and a right sural nerve biopsy was performed. A 12-year-old girl, a sister of the boy described above, with the diagnosis of ACTH insensitivity at age 5, noted a pain on the medial aspect of the left sole after skating. On general physical examination, a mild diffuse skin hyperpigmentation was discovered. On neurological examination, a spontaneous pain with dysesthesia was noted on the plantar aspect of the 1st, 2nd and 3rd left toes and on the anterior and medial aspect of the sole. Otherwise she was normal. A diagnosis of the left tarsal tunnel syndrome was made.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Benign familial infantile seizures: further delineation of the syndrome.

Benign familial infantile seizures are an autosomal dominant epilepsy disorder that is characterized by convulsions, with onset at age 3 to 12 months and a favorable outcome. Benign familial infantile seizures have been linked to chromosome 19q whereas infantile convulsions and choreoathetosis syndrome, in which benign familial infantile seizure is associated with paroxysmal choreoathetosis, has been linked to chromosome 16p 12-q12. Many additional families from diverse ethnic backgrounds have similar syndromes that have been linked to the chromosome 16 infantile convulsions and choreoathetosis syndrome region. Moreover, in one large pedigree with paroxysmal kinesiogenic dyskinesias only, the syndrome has also been linked to the same genomic area. Families with pure benign familial infantile seizures may be linked to chromosome 16 as well. In this study, we present a series of 19 families and 24 otherwise healthy infants with benign familial infantile seizures. Two of these families include members affected with benign familial infantile seizures and paroxysmal choreoathetosis. We included patients with normal neurologic examinations, who started having simple partial seizures, complex partial seizures, or apparently generalized seizures without recognized etiology between 2 months and 2 years of age. Neurologic studies were normal, but in all patients, there was a history of similar seizures and age at onset in either the father or the mother. Twenty-four patients (14 girls and 10 boys) were evaluated at our hospital between February 1990 and February 2001. Age at onset, sex, family history of epilepsy and/or paroxysmal dyskinesias, neurologic examination, semiology, distribution, and frequency and duration of seizures were evaluated. Electroencephalographic (EEG) and neuroradiologic studies were also performed. Seizures began between 3 and 22 months of life, with a median age of 5 1/2 months. Nine patients (37.5%) had only apparently generalized seizures, 5 patients (20.8%) had only partial seizures, and 10 patients had both partial and apparently generalized seizures (41.6%). Seizures were invariably brief, occurred during the waking state (100%), and presented mainly in clusters in 12 patients (50%). Interictal EEG was normal in 23 patients (95.8%). Sixteen patients (66.6%) had a confirmed history of convulsions in family members other than parents. Twenty-two patients became seizure free after 30 months of life. Two brothers in the same family had brief paroxysmal episodes of choreoathetosis in the hemibody triggered by stress while awake at 15 and 17 years old, respectively. One of them had paroxysmal choreoathetosis only, and the other was associated with benign familial infantile seizures. One father had brief spontaneous episodes of paroxysmal choreoathetosis when awake at age 18 years. All of them had a good response to antiepilepsy drugs, and neurologic examination and EEG and neuroradiologic studies were normal. Benign familial infantile seizure is a genetic epilepsy syndrome with autosomal dominant inheritance. It may be associated with paroxysmal choreoathetosis (infantile convulsions and choreoathetosis syndrome), which has been linked to the chromosome 16 infantile convulsions and choreoathetosis syndrome region. Patients in families with infantile convulsions and choreoathetosis syndrome could display either benign familial infantile seizures or paroxysmal choreoathetosis or both. It is likely that the disease in families with pure benign familial infantile seizures may be linked to the infantile convulsions and choreoathetosis region as well. We cannot exclude the possibility that the youngest patients may develop choreoathetosis or other dyskinesias later in life.

Athetosis↗

Neurological observations.

Family physicians generally find the neurological examination a problem because of its perceived length and complexity. But the neurological examination can be made both shorter and more informative by seeing it as commencing from the moment of first meeting the patient and continuing through the history taking and combining this with a focusing of the formal examination of relevant areas.

Family Practice↗

A rare case of diplopia: medial inferior pontine syndrome or Foville's syndrome.

A case of medial inferior pontine syndrome or Foville's syndrome is described. The patient presented to the emergency department with an acute history of slurred speech, vertigo and diplopia as major complaints. He also mentioned the appearance of weakness and numbness in his left leg. The physical examination revealed a crossed neurological deficit (ipsilateral cranial nerve deficit with contralateral motor weakness) which is typical for posterior circulation stroke in the brainstem territory. In our patient the lesion was located in the right medial inferior pontine region. All the symptoms and signs disappeared within 24 hours confirming the importance of a detailed physical and neurological examination of each patient presenting at the emergency department with a neurological deficit.

Aged↗

ERP P300 and brain magnetic resonance imaging in patients with vibroacoustic disease.

INTRODUCTION: Subjects occupationally exposed to large pressure amplitude (> or = 90 dB SPL) and low frequency (< or = 500 Hz) (LPALF) noise for long term periods (>10 yr) can develop vibroacoustic disease (VAD). One of the earliest complaints of VAD patients deals with memory and attention disturbances. On the other hand, in over 50% of these patients, magnetic resonance imaging (MRI) of the CNS revealed cortical atrophy and dilation of the perivascular spaces, and small hyperintense lesions in T2 of the cerebral white matter, basal ganglia, and brainstem. The purpose of this study is to investigate whether there is any relationship between these morphological changes and the event-related potentials (ERP) P300 parameters. METHODS: Sixty aeronautical workers with more than 10 yr of LPALF noise exposure were studied using brain MRI and ERP P300. A careful neurological examination had been previously performed on all of them. RESULTS: The neurological examination revealed abnormalities in 80%, the most common finding being the archaic palmo-mental reflex. Some 37 subjects had hyperintense foci in T2 of the subcortical and periventricular white matter, the basal ganglia and the brainstem. Comparing the ERP P300 amplitude and latency values, morphology and topography, obtained in these 37 subjects with the data from the 23 who did not have any morphological changes in CNS, we found a strong correlation between the brain MRI lesions and the neurophysiological changes (p < 0.01). CONCLUSIONS: These results are important for understanding the pathophysiology of the cognitive impairment found in VAD patients.

Adult↗

[Neuropsychomotor development of infants born of mothers with gestational hypertension].

INTRODUCTION: Gestational hypertension is a major cause of maternal death in our country and may be associated with neonatal hypoxic-ischemic encephalopathy with serious neurological complications. OBJECTIVE: To correlate gestational hypertension with risk factors of neuropsychomotor development in infants. METHOD: This was a prospective study. We evaluated 30 consecutive infants born of mothers with gestational hypertension. The following risk factors were considered: small for gestational age; fetal asphyxia; age of onset of gestational hypertension; term/preterm newborn; Apgar scores; central cyanosis; O2 mask; meconium. The study followed two steps. In the first step, newborns underwent neurological examination soon after birth (48-72 hours of life). In the second step, children underwent another neurological assessment between 7 and 15 months of life. RESULTS: Six newborns presented neurological signs on the first evaluation. The only risk factor that showed a significant correlation with the neurologic examination was the Apgar score. Other risk factors did not show any correlation. All children evaluated on the second step of the study showed normal neurological development and examination, which did not allow any correlation with risk factors. CONCLUSION: Our data suggest that gestational hypertension per se is not sufficient to cause fetal neurological impairment.

Adult↗

Neurological findings in neonates with low Apgar in Zimbabwe.

OBJECTIVES: Document neurological condition of African neonates with a low apgar score. SETTING: Mpilo Hospital, Bulawayo, Zimbabwe. SUBJECTS: 165 babies with an Apgar score of 5 or less at 5 min. METHODS: Neurological examination at term age according to Prechtl. Babies were classified as normal, suspect or abnormal and compared with two reference groups, one from Groningen, the Netherlands and one from Grenada in the Caribbean. RESULTS: A higher number of Zimbabwean babies were delivered by Caesarean section compared to the Groningen group (P < 0.001). Babies delivered by vacuum extraction scored significantly lower compared to babies delivered by Caesarean section (P < 0.003). Twenty abnormal signs derived from the neonatal neurological examination proved to be predictive on the total optimality score (P < 0.001). The number of infants who were classified as abnormal was higher in the Zimbabwean population (P < 0.01). CONCLUSION: The selected abnormal signs derived from the neonatal neurological examination proved to be highly predictive on the neurological condition. The neonatal morbidity in Zimbabwean neonates with a low Apgar score was higher when compared with two reference groups from Groningen and Grenada.

Adolescent↗

Influence of nonbiologic implants on laminectomy membrane formation in dogs.

The effects of various surgical implants, spinal cord hypothermia, and glucocorticoid administration on formation of the laminectomy membrane were evaluated in 32 preconditioned chondrodystrophoid dogs. Modified dorsal laminectomies and full-length durotomies, from T12 to L1, were performed on all dogs. Dogs were allotted to 2 groups. Group-1 dogs (n = 20) were further allocated to 4 subgroups (a, b, c, and d) consisting of 5 dogs each. Group-1a dogs received no implant, group-1b dogs had absorbable gelatin sponges implanted, group-1c dogs had absorbable gelatin films implanted, and group-1d dogs had absorbable gelatin sponges and absorbable gelatin films implanted. Daily neurologic examinations permitted correlation of neurologic dysfunction with secondary spinal cord compression in those dogs in which it developed. The influence of these implants on laminectomy membrane formation and dural healing was assessed by gross and microscopic evaluation of transverse sections of the vertebrae and spinal cord after euthanasia of one member of each subgroup at 1, 2, 4, 8, and 16 weeks after surgery. Group-2 dogs (n = 12) were further allotted to 3 subgroups (a, b, and c) consisting of 4 dogs each. One dog in each group-2 subgroup underwent the same surgical procedures described for the group-1 subgroups (ie, 4 procedures/group-2 subgroup). The additional effects of 3 conventional supportive techniques (selective regional spinal cord hypothermia, glucocorticoid administration, or spinal cord hypothermia and glucocorticoid administration) on laminectomy membrane formation and on immediate postoperative recovery were examined in groups 2a, 2b, and 2c, respectively. Neurologic examinations were performed daily until this time. All dogs in group 2 were euthanatized 1 week after surgery for gross and microscopic examination of transverse sections of the vertebrae and spinal cord. Qualitative histopathologic effects of the different implants and supportive techniques on formation of the laminectomy membrane were determined. Statistical analysis of the degrees of secondary spinal cord compression was performed in group-1 dogs by measuring and comparing ratios of the vertical to the horizontal diameters of the transverse spinal cord sections from locations within (T12 to L1) and out of (T11, T11-12, L1-2, and L2) the region of surgical intervention. The vertical/horizontal diameter ratios measured from transverse sections from T11 to L2 in size-matched, untreated control dogs formed the standards for a mean roundness index of the spinal cord in the various anatomic locations of the vertebral column.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

Mild head injury: revisited.

OBJECTIVE: 1. To define the incidence of positive CT scans in patients with different grades of mild head injury (MHI), 2. To identify clinical predictors of positive CT scans, 3. To evaluate the usefulness of plain radiographs in the triage of patients with MHI, 4. To evaluate frequency and nature of surgical intervention in MHI, 5. To evaluate the risk of deterioration in MHI, and 6. To find out whether patients with normal neurological examination and normal CT can be safely discharged. METHODS: In a prospective study conducted over a period of four months in our center, all patients with mild head injury (defined as Glasgow Coma Scores (GCS) 13-15) were admitted to the head injury unit. Patients underwent standard clinical examination, skull radiography and cranial CT. No clinical criteria were used to select patients for CT scanning and all the patients were subjected to CT. Patients with negative findings on CT and a normal neurological examination were discharged after 24 hours of observation. Patients with positive findings on cranial CT were treated either medically or surgically as deemed necessary. Outcome measures included safe discharge, clinical deterioration, need for surgical intervention or death. The following factors were analyzed statistically to find out whether they can be used as predictive factors for positive cranial CT. They were: age, sex, mode of injury, loss of consciousness, post-traumatic seizures, ear/ nose/throat bleeding, vomiting, admission GCS score, scalp injury, polytrauma, focal neurological deficit, fractures visualized on skull radiography. RESULTS: Three hundred and eighty one patients were included in the study. Of these males constituted 63%, females 17% and children 20%. RTA was the most common mode of injury. Seventy five percent of the patients had GCS of 15, 15% had GCS of 14 and 10% had GCS of 13. Thirty eight percent of the patients had positive findings on the CT. Age, mode of injury, loss of consciousness, post-traumatic seizures, ENT bleeding, vomiting, scalp injury and polytrauma were not found to be predictors of positive CT. Admission GCS score, focal neurological deficits, and fractures detected by skull radiography were found to be statistically significant predictors of positive findings on CT. Seven percent of patients required surgical intervention. Six percent of patients showed neurological deterioration and there was one death in this series of MHI patients. Patients with multiple lesions on CT had a higher chance of deterioration than those with a single lesion. No patient who had a GCS of 15 and normal CT developed any complication during the hospital stay or after discharge. CONCLUSIONS: The incidence of positive CT scans in this prospective, consecutive, unselected cohort of MHI patients was 38%. Lower admission GCS scores, focal neurological deficits, and fractures detected by skull x-rays were found to be significant predictors of positive CT. Other clinical parameters were not found to be predictors of positive CT. CT was found to be superior to plain x-rays in detecting skull fractures. Seven percent of this cohort required neurosurgical intervention. Six percent showed neurological deterioration and there was one death in this series. Patients with multiple lesions on CT had a higher chance of deterioration than those with single lesions. The duration of hospital stay was prolonged in patients with positive CT. As no patient with a normal neurological examination and a normal CT deteriorated, we believe these patients can be safely discharged without need for admission and observation.

Adolescent↗

Psychomotor development and general movements in offspring of women with epilepsy and anticonvulsant therapy.

UNLABELLED: While the role of antiepileptic drug (AED) therapy in teratogenesis has widely been investigated, there are few prospective studies on later postnatal development in offspring of epileptic women in utero exposed. The aim of this study was a prospective investigation of the psychomotor development in a selected population of infant born to women with epilepsy on AED therapy during pregnancy. PATIENTS AND METHODS: Children were assessed at various times until 30 months of age by general movement (GMs) observation (at 7 days and 4 and 13 weeks), traditional neurologic examination (at 7 days and 4 and 13 weeks, 6, 9 and 12 months) and Brunet-Lezine (B-L) administration (at 30 months). We present the preliminary results of our study conducted on 11 children. RESULTS: Psychomotor delay in children was confirmed by traditional neurological examinations scores at 7 days, 4 weeks, 13 weeks and 6 months and by B-L score at 30 months. Between 9 and 12 months of age, traditional neurologic examination became "silent". GM assessment was found to be a better predictor of psychomotor development. In fact, GM analysis, particularly at 4 weeks, was strongly correlated with the Brunet-Lezine score at 30 months. In conclusion, on the basis of these data we suggest a psychomotor delay in the offspring of epileptic women and that GMs and neurologic evaluation provide complementary information concerning psychomotor development and later outcome of these children.

Adult↗

[A case of recurrent cranial neuropathy presenting as recurrent Tolosa-Hunt syndrome].

A 64-year-old woman, with history of hypertension and arteriosclerosis, developed left painful ophthalmoplegia in July, 1988. Neurological examination proved abnormality of the third cranial nerve innervation, otherwise normal. No systemic illness was present. With corticosteroid therapy, the symptoms regressed and completely disappeared in 3 months. In January, 1990, right painful ophthalmoplegia appeared. Neurological examination revealed involvement of right sixth nerve and first branch of the right fifth nerve. With corticosteroid therapy, the symptoms completely regressed in several weeks. In April, 1990, she developed severe pain in the right side of the face. The facial pain disappeared rapidly with corticosteroid therapy, but reappeared following quit of steroid. She complained of severe pain of the right face, the territory of first and second branch of the right fifth nerve, but neurological examination was negative. With corticosteroid therapy, the pain disappeared remaining with mild tingling sensation on the right face, but during the tapering of corticosteroid in August, a severe peripheral type right facial palsy developed. Corticosteroid therapy resumed and the facial palsy regressed almost completely in ten days. Our case suggests that THS might be a variant of so-called recurrent cranial neuropathy.

Aged↗

Metabolic and cognitive changes in hereditary ataxia.

Fourteen subjects (affected, n = 7; at risk, n = 7) from one well-known kindred with adult onset autosomal dominant olivopontocerebellar atrophy (OPCA), were studied with [18F]-2-deoxy-D-glucose (FDG) positron emission tomography (PET), magnetic resonance imaging (MRI), cognitive testing and scored neurological examination, and compared with normal controls. The neurological examination, MRI and cognitive tests showed no significant differences between at risk and normal control subjects. Mild cognitive deficits were seen in affected subjects; the degree of cognitive change appeared to relate to the severity of the illness. MRI demonstrated cerebellar and brainstem atrophy in all affected subjects. PET studies showed higher global metabolic rates (mean [SD]) in at risk subjects (10.5 [1.5] mg per min per 100 g) as compared to affected (9.0 [0.8] mg per min per 100 g) and normal control subjects (9.1 [1.5] mg per min per 100 g). Normalized (region/global average) regional metabolic rates were reduced in cerebellar hemispheres and vermis, and in frontal and prefrontal areas of affected subjects in comparison to at risk and normal control subjects. These findings indicate that functional changes in some forms of autosomal dominant hereditary cerebellar ataxia may extend beyond the cerebellum and brainstem to involve other parts of the neuraxis.

Adult↗

A neurologic comparison of pre-term and full-term infants at term conceptional age.

There are currently no neurologic examinations designed for pre-term infants with sufficient data to determine neurologic deviance at various gestational or conceptional ages (gestational age plus age from birth). In this study a neurologic examination standardized for full-term newborn infants was administered to 97 full-term newborn infants was administered to 97 full-term newborn infants and 97 pre-term infants at their expected date of birth, 40 weeks' conceptional age. The pre-term infants had more weak responses than did the full-term infants despite being at a higher level of activity state throughout the examination. The pre-term infants also had more asymmetric responses. There was no correlation between gestational age and the incidence of weak responses in the pre-term infants, but the pre-term infants of lowest birth weight at all gestational ages had the greatest number of weak responses. The latter may be because pregnancy and neonatal problems contribute more to the neurologic findings than does the length of gestation.

Age Factors↗

Neonatal pattern vision: a predictor of future mental performance?

The visual fixation responses of 33 high-risk neonates to patterned stimuli were rated as normal, suspect, or abnormal in an attempt to predict future intellectual performance. For comparison purposes, a neurologic examination was aslo administered. Follow-up studies based on autopsies and neurologic and psychological testing agreed with the neonatal visual preference ratings in 27 cases, but with the neurologic examination ratings in only 22 cases. This preliminary study suggests that neonatal visual testing is a promising technique for evaluating brain integrity in the newborn period.

Child, Preschool↗

[Idiopathic pure sudomotor failure].

We describe three cases of acquired generalized anhidrosis without other autonomic and somatic nervous dysfunctions (idiopathic pure sudomotor failure; IPSF) and a review of the literature was made in regard to the clinical features of this disease. Patient 1. A 17-year-old man was found to be severely anhidrotic and intolerant to heart loading, which produced an immediate sharp pain over the entire body surface in April, 1989. Sudden and spontaneous remission occurred in July, 1990, but relapsed six months later. He was admitted to our hospital seeking for therapy in April, 1991. There were no abnormal findings on physical and neurological examinations except for anhidrosis. Patient 2. A 14-year-old boy was admitted to our hospital in March 1991 with complaints of intolerance to heart loading and sharp pain, which had begun ten months before admission. Physical and neurological examinations revealed no abnormal findings except for anhidrosis. Patient 3. A 21-year-old woman suddenly experienced a burning sensation in the whole body in 19th, February, 1991, which was followed by severe pain. She was admitted to our hospital in the next day. Apart from sensory impairment of glove and stocking type, neurological examination revealed no abnormal findings. Reflex sweating to pilocarpine was absent in all three cases, suggesting abnormality in the postganglionic sudomotor nerves or cholinergic receptors. Skin biopsy was performed in one patient (patient 1), and revealed no abnormalities of the sweat glands and ducts.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Value of neuroimaging in the evaluation of neurologically normal children with recurrent headache.

Headache is one of the most frequent physical complaints in children. Although headaches in children are generally benign, neuroimaging studies are frequently performed in clinical practice for the fear of missing a serious underlying disease. Despite this, limited data exist about the utility of neuroimaging in recurrent headache of children with a normal neurologic examination. This prospective study was planned to determine the value of neuroimaging in neurologically normal children with migraine and tension-type headache. Among 95 consecutive patients presenting with headache, 72 patients receiving a diagnosis of migraine or tension-type headache were included in the study. Neuroimaging procedures were performed in 83%. Magnetic resonance imaging (MRI) was abnormal in 11 of 49 cases. Abnormalities consisted of foci of gliosis in four, sinusitis in two, pineal cyst in one, periventricular leukomalacia in one, arachnoid cyst in one, old traumatic changes in one, and cervical syrinx in one. Two of the 11 computed tomographic (CT) scans revealed sinus disease. The percentage of findings causally related to headache was about 10. None of the patients had undergone surgery because of neuroimaging results. In conclusion, the yield of neuroimaging in recurrent headaches of children with a normal neurologic examination is low, and neuroimaging should not be part of a routine initial examination of these patients.

Adolescent↗