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PubMed1984

[Baló's concentric sclerosis].

A clinico-pathological case of concentric sclerosis (Baló type) is reported. A 30-year-old man experienced dizziness, nausea and vomiting. Twelve days later he developed gait disturbances. Neurological examination showed broad based gait, brisk tendon reflexes, bilateral extensor plantar responses, right hemihypoesthesia, cerebellar dysmetria, and a left lateral gaze palsy. CSF examination showed, 520 mg p. 100 ml protein, 7500 red blood cells, 31 lymphocytes and 9 polymorphonuclear leukocytes/mm3, 18 p. 100 gammaglobulin. Three CT scans were performed and showed a round hypodensity in the parieto-occipital white matter with contrast enhancement on one occasion, and several other hypodensities in the contralateral parieto-occipital white matter and in both frontal lobes. 23 days after the onset of the disease, the patient became comatose. A cerebral biopsy was obtained from one of the frontal lesions. He died from aspiration bronchopneumonia 2 months after the first signs. Neuropathological examination showed numerous concentric zones of demyelination which involved the white matter of both hemispheres, brain stem, and cerebellum. On light microscopy sudanophilic myelin breakdown products were numerous in the bands of demyelinisation. Astrocytic proliferation was marked, with frequent Rosenthal fibers. Edema was noted in some lesions. Myelin-axonal dissociation was obvious, but some axonal swelling were observed. Electron microscopy demonstrated the integrity of oligodendrocytes and of blood vessels and confirmed the prominent alterations of the astrocytes. Fifteen similar cases of the literature have been reviewed. The present case seems to be the first one with CT scan examination and electron microscopic study of a brain biopsy. The nosological situation of Baló's disease among the inflammatory demyelinating diseases of the group of MS is discussed.

Adult↗
PubMed1984

[A case of spontaneous spinal epidural hematoma presenting Brown-Séquard syndrome].

Although there have been a few reports of spontaneous spinal epidural hematoma, it may be difficult to make a correct diagnosis of the lesion because of atypical clinical symptoms. The authors reported a case of spontaneous spinal epidural hematoma presenting Brown-Séquard syndrome, with a review of literature. This 75-year-old male experienced a sudden onset of severe dorsal neck pain during sleep associated with right hemiparesis which deteriorated quickly to hemiplegia, without loss of consciousness, nausea, vomiting and vertigo. The patient has a history of hypertension and has been treated for chronic hepatitis for one year. On admission, neurological examination revealed right hemiplegia with normotensive deep tendon reflexes, and loss of pain and temperature sensations below the level of C5 on his left side. Position sense of fingers and toes was diminished on his right side, and hyperesthesia was recognized at the area of C4 level. Consciousness disturbance, cranial nerve signs, and urinary incontinence were not seen. Right retrograde vertebral angiograms showed no abnormal vascular shadow, even though anterior spinal artery was visible at the level of C8 through C5. Cervical CT scan revealed a left-side dominant extradural high density mass which situated dorsally at around the upper part of C2 body changing it's position to dorsolaterally as it descended to the lower part of C5. On contrast enhancement study, only the margin of the lesion was partially enhanced, which might be the hematoma membrane. On the third day after the onset, laminectomy from C2 to C5 was performed and the epidural hematoma was evacuated.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1984

[A case report of post-traumatic syringomyelia].

A case of posttraumatic syringomyelia which appeared 26 years after the injury was presented. A patient was 61 year old female, who sustained thoraco-lumbar spine injuries rendering her to paraplegic in 1954. Eleven months later she had an operation of T6-T9 and L1-L2 laminectomies and regained motor and sensory functions of the both lower extremities. She was ambulatory with crutches till 1979. In 1980, burning pain was noticed in the left scapular region, and thereafter, extended to the ulnar side of the left forearm. The pain became progressively worse and intractable. Analgesics were ineffective. Two years later muscle atrophies and weakness in the left finger intrinsic muscles appeared. Absent deep tendon reflexes in the left upper extremity, dissociated sensory loss (in the left C2-S1 and right T5-T12 dermatomes) and paraparesis were also documented. Metrizamide CT scan performed 24 hours after the intrathecal injection disclosed an intramedullary syrinx between C2 and L1 vertebral levels. No communication with the fourth ventricle was seen. A syringoperitoneal shunt with low pressure valve was placed. The pain subsided immediately after this procedure. However, no improvement in motor and sensory functions were observed. Pathophysiological mechanisms involved in post-traumatic syrinx formation and its development were discussed. We prefer hypothesis proposed by Ball and Dayan to Gardner's hydrodynamic theory regarding to development of the syrinx secondary to spinal cord injury.

Female↗
PubMed1984

Functional paralysis mimicking spinal cord injury resulting in admission to a spinal cord injury center.

From 1978 through 1982, five patients with final diagnoses of nonorganic functional paraplegia or quadriplegia were seen and treated at the Northern California Regional Spinal Cord Injury Center. The patients ranged in age from 13 to 26 years and four were males. All had normal bowel and bladder function with otherwise complete motor and sensory paralysis, shifting sensory findings, and normal deep tendon reflexes early after injury. In each patient, a retrospective analysis demonstrated that structural anatomic lesion could be ruled out by a neurologic examination. Clinical and investigatory studies are discussed with respect to the diagnostic process.

Adolescent↗
PubMed1984

[Intramedullary holocord tumor. Report of an autopsy case and review of literature].

This report describes a case of cystic astrocytoma that extended from C4 to the conus, and reviews intramedullary holocord tumors. A two-year-old body was admitted because of gait disturbance. He had suffered from rigidity of his left upper and lower limbs and thoracic scoliosis for six months prior to admission. On admission, neurologic examination revealed spastic paraparesis with muscle atrophy of the left lower extremity, sphincter disturbances and neck rigidity. All deep tendon reflexes were bilaterally hyperactive. Spine roentgenography demonstrated widening of the interpedicular distance from the midthoracic to the upper lumbar segments. A lumbar myelography disclosed a block at the L2 level, and a suboccipital myelography revealed a block with tapering of contrast medium at the level of the C2 vertebra. A laminectomy was performed from C1 to L3, and a myelotomy was performed from C4 to L2. Grossly total removal of solid tumor which extended from Th3 to Th8 was carried out. There were large intramedullary cysts extending from C4 to Th3, and from Th9 to L2. Eight hours after surgery, high fever, tachycardia and anuria developed, and he died next day. Cross section of the spinal cord revealed the tumor cells infiltrated diffusely in the midthoracic region, and histologically the tumor was diagnosed to be astrocytoma. The cystic portion was separate from the central canal, and the lining of the cyst was a band of fibroglial tissue without infiltration of tumor cells. A tumor of this kind extending throughout the almost whole spinal cord is extremely rate. For such a holocord tumor complete removal is the ideal treatment, but the possibilities for removal must be evaluated in terms of the tumor's relationship to the spinal cord, the histological nature and the general condition of the patient.

Astrocytoma↗
PubMed1980

Endocrine studies with mazindol in obese patients.

Detailed endocrine studies were carried out in 95 hospitalized obese patients during their treatment with diet and the tricyclic anoretic mazindol. The results obtained after 1 week or more of mazindol (2 mg or occasionally 4 mg/day) administration were compared with the results after placebo and with the initial pre-treatment values. There were no significant changes in the following parameters: FSH, LH, testosterone, renin, angiotensin II, growth hormone (GH) levels during insulin tolerance tests (ITT), 131I uptake, basal metabolic rate, Achilles tendon reflexes, T3 RIA, rT3 RIA, 17-ketosteroids and 17-ketogenic steroids in urine, both basal and after stimulation with ACTH and metyrapone. Blood glucose and plasma immunoreactive insulin (IRI) levels during oral glucose tolerance tests decreased during mazindol administration, IRI levels were significantly lower during ITTs after mazindol. T4 RIA serum levels increased significantly after mazindol. When mazindol was administered, GH levels increased somewhat in some obese patients during ITTs, while T3 RIA and rT3 RIA decreased in some patients. Mazindol has not only hunger (appetite) suppressing properties, but it probably affects the metabolism of energy substrates as well. The drug was well tolerated and there were no pathological findings in routine laboratory examinations during a long-term study with mazindol (non-stop treatment for 6 months).

Adolescent↗
PubMed1977

Polyneuropathy in hypothyroidism. Clinical and nerve biopsy study of 4 cases.

Clinical and sural nerve biopsy findings are described in four hypothyroid patients with manifestations of a diffuse peripheral neuropathy. Clinical examination revealed distal sensory inpairment, complaints of burning and lancinating extremity pains, ataxia and a decrease of deep tendon reflexes with total ankle jerk loss. In one patient, signs of the peripheral neuropathy comprised the leading symptomatology of the underlying metabolic disorder. Light and electron microscopic investigations of the sural nerve biopsies revealed a marked reduction of myelinated fibers, affecting mainly the large myelinated axons. The histograms showed a pathological unimodal spectrum of myelinated fiber diameters. Dystrophic and degenerative changes in axons of all fiber classes and the presence of clusters of thinly myelinated small fibers suggest that neuroaxonal degeneration and incomplete regeneration may represent the major pathogenetic mechanism of the neuropathy in these hypothyroid patients.

Aged↗
PubMed1983

[The clinical significance of the F-wave of upper limb on cervical myelopathy].

The physiological characteristics of F-wave on the evoked EMG has been investigated and the utility of the objective index on cervical myelopathy was discussed. The wave pattern of F-wave varied each time, but the resembling wave shapes could be recognized while series of recording was done. The latency and amplitude of F-wave in resembling wave-shapes were equal. The F-wave could therefore be differentiated into several wave patterns. The wave patterns of the pathologic cases were almost the same as normal patterns. There is no any meaningful difference in conduction velocity between the normal and abnormal cases. Concerning the amplitude and the frequency of occurrence, the utilities are recognized. There has been a tendency in parallel with the severity of myelopathy, the more severe was the involvement, the more frequently occurred the big F-waves. It was also related to the motor function of the extremities and the degree of the acceleration of tendon reflex. Moreover, along with the postoperative improvement of the clinical symptoms, the occurrences of big F-wave approached that in normal cases. Apparently, the F-waves well reflected the clinically abnormal state. In other word, under the discussion of the amplitude and frequency, the F-wave of upper limb is believed to be useful as an objective index in cervical myelopathy.

Adolescent↗
PubMed1983

[2 Cases of cystic meningioma].

Although meningiomas are usually a solid and firm tumor, some are associated with diagnostically confusing large cysts. The authors experienced two cases of meningioma associated with large cyst (cystic meningioma). The first case was a 20-year-old female. She was admitted because of blurred vision. On admission she was slightly apathetic and showed bilateral papilledema. Computed tomography showed a large area of low density in both frontal regions. CT scan after intravenous contrast enhancement revealed an enhancing mural nodule attached to the falx. At operation, dark-green fluid in an amount of 160 ml from the left cyst and 50 ml from the right cyst was aspirated. The nodular tumor was found attached to the falx. The pathological examination revealed meningotheliomatous meningioma. Neoplastic meningothelial cells were found also in the cyst wall. The second case was a 21-year-old female. She was admitted because of weakness of the left lower extremity. Neurological examination revealed left hemiparesis. Deep tendon reflexes were hyperactive on the left side and there were positive left Babinski and Chaddock reflex. The computed tomography with contrast enhancement showed a large area of low density in the right parietal region with markedly enhancing mural nodule. At operation, 70 ml of xanthochromic fluid was aspirated. The tumor was well-defined, hard and attached to the dura. Histologically the tumor was a typical meningotheliomatous meningioma. The diagnostic value of computed tomography, the pathogenesis of cyst formation and the surgical treatment were discussed. The authors emphasized the important role of computed tomography in the diagnosis of cystic meningioma. Theories were reviewed for the pathogenesis of cyst formation. At operation, not only the mural nodule but also the cyst wall should be removed, because the neoplastic cells are presented in the cyst wall.

Adult↗
PubMedDate not supplied

Osteoid osteoma of the proximal femur simulating spinal root compression.

In a 6 1/2-year-old boy and a 9 1/2-year-old girl, spinal root compression was suspected on the basis of muscle wasting and weakness, diminished deep tendon reflexes, and severe pain, all in a radicular distribution. However, extensive and invasive neuroradiologic evaluation failed to demonstrate compression of spinal roots. Only several months later was the correct diagnosis of osteoid osteoma reached. In one patient, the tumor was removed and on histologic examination groups of unmyelinated nerve fibers were found near the center of the tumor nidus, generally surrounding small arterioles. In the other patient parental consent was not given for the operation. The symptoms gradually subsided, although the typical radiologic features of the tumor went unchanged. The presence of unmyelinated autonomic nerve fibers in the nidus of osteoid osteoma may explain the severe pain commonly experienced by the patients. The spinal origin of such fibres can account for the radicular syndrome, while the localization of pain and sensory findings remote from the tumor site might be explained by a mechanism similar to that responsible for "referred pain."

Child↗
PubMedDate not supplied

Cervical spondylolisthesis associated with the multiple nevoid basal cell carcinoma syndrome.

Cervical spondylolisthesis is a rate vertebral anomaly that has not been associated with other major congenital abnormalities. In a 16-year-old girl, lower cervical spondylolisthesis was associated with a multiple nevoid basal cell carcinoma syndrome. The salient features of cervical spondylolisthesis include occipital headache, nuchal rigidity, torticollis, painful range of limited motion, hypesthesia, and depressed deep tendon reflexes. The treatment in the patient was anterior vertebral body fusion. Awareness of this associated problem and prompt treatment are essential for prevention of neurologic loss.

Adolescent↗
PubMed1982

[A case of nodular calcification of the ligamentum flavum with ossification of the posterior longitudinal ligament in the cervical spine].

A 72-year-old male noted weakness in the right lower extremity in August 1979, and clumsiness of the finger movement in the right hand in April 1980. In June 1980, he became tetraplegic and was unable to stand or walk. He also developed hypesthesia in the four extremities and difficulty in voiding. On admission to this hospital (July 12, 1980), neurological examination showed spastic tetraparesis, more pronounced on the right side. Deep tendon reflexes were markedly increased in the four extremities. Chaddock reflex in the right lower extremity and Trömner reflex in the both upper extremities were elicited. Hypesthesia below the level of C4 was present with anesthesia in the Th9-11 region. Plain film and tomogram of the cervical spine showed ossification of the posterior longitudinal ligament from C3 to C6 and a lobulated nodular calcification in the posterior part of the spinal canal at the level of C4,5 intervertebral space. CT scan revealed nodular calcifications in the ligamentum flavum not only at the level of C4 but also at the level of C6. Laminectomy from C3 to C6 was performed on August 21. The ligamentum flavum with nodular calcifications was removed. His postoperative course was uneventful. Seven weeks postoperatively the patient was able to walk without assistance and had no difficulty in voiding. He was discharged on October 14 with excellent activities of daily life. The content of the nodular calcification was identified as a kind of calcium phosphate by the physico-chemical analysis. Ossification and/or calcification of the ligamentum flavum in the cervical spine is rare, while ossification of the posterior longitudinal ligament is frequently encountered in the cervical spine. Only two cases with nodular calcification of ligamentum flavum in the cervical spine have been reported. To our knowledge, this is the first case of the nodular calcification of the ligamentum flavum in the cervical spine associated with ossification of the posterior longitudinal ligament. Two dimensional analysis by CT scan was very useful in locating the lesion and deciding the operative procedure.

Aged↗
PubMed1981

Central nervous system and facial defects associated with maternal hyperthermia at four to 14 weeks' gestation.

An analysis of 28 dysmorphic offspring with a retrospectively ascertained history of maternal hyperthermia during the first trimester of pregnancy showed a similarity in their pattern of CNS dysfunction and facial dysmorphogenesis. All survivors had mental deficiency and most of them demonstrated altered muscle tone, including hypotonia with increased deep tendon reflexes. Those exposed at four to seven weeks' gestation showed an increased prevalence of facial dysmorphogenesis. The duration of the high fever was usually one or more days, an unusual occurrence during the first trimester of pregnancy. The nature of these defects in relation to the relative timing of hyperthermia exposure is similar to that previously noted in animal studies. The morphogenetic implications of these findings are explored and the need for larger, controlled studies is suggested.

Adolescent↗
PubMed1981

[Dynamics of the hypertensive-hydrocephalic syndrome in infants].

Results of a 3-year-long observation of 1,158 children in whom the hypertensive-hydrocephalic syndrome was diagnosed in the first months of life are presented. With regard to the size of the head, the gravity of the hypertensive-hydrocephalic symptoms, and the changes on the part of the nervous system by the third year of the life the children were divided into three groups. The first group included 51 children with progressing hypertensive syndrome and congenital hydrocephalus because of anomalies of the cerebrospinal fluid system and the brain. In 821 children of the second group, the head remained enlarged in size by 3 to 10 cm. In half of them changes of the neurological status in the form of pathologies of the craniocerebral nerves, high tendon reflexes, pareses, and the atactic syndrome were revealed. In 102 children there remained a retardation of the psychic and speech development. 286 children of the third group were found to be healthy at the age of 3 years. Thus, in 1/3 of the children who have had a perinatal pathology the hypertensive-hydrocephalic syndrome appeared to be of a transitory character, and regressed completely by the age of two years.

Cephalometry↗
PubMed1981

[Use of an adenyl preparation for treating neuralgic amyotrophy].

The authors suggest that adenyl should be included into the therapeutic combination used for treating patients suffering from neural amyotrophy. The drug is to be injected intramuscularly twice a day for a month. Observations of 24 patients showed that in 23 of them positive changes could be noted. These changes manifested not only in a subjective, but also in a distinct objective increase of the muscular force and motor potentialities, and even in appearance of weak tendon reflexes in some cases. No side effects were observed.

Adenosine Monophosphate↗
PubMedDate not supplied

[Isaacs and Mertens syndrome: report of one case (author's transl)].

The author reports a clinical case of Isaacs-Mertens syndrome (syndrome of continuous muscle fibre activity, neuromyotonia, pseudomyotonia). A 55 years old man had increasing stiffness, painful spasms and myokymias of the peripheral muscle, associated with hyperhidrosis and absent tendon reflexes. Electromyography showed continuous electrical activity while at rest, general anaesthesia, after intravenous injection of diazepam and after peripheral nerve block. The activity disappeared after muscle infiltration of procaine and after intravenous injection of succinylcholine. After two years treatment with carbamazepine, 600 mg daily, the patient showed a normal clinical and neurophysiological picture. The etiology of the disease is unknown. The defect probably lies in the terminal motor network. A review of previous literature shows a typical uniformity of the electromyographic findings and of improvement on phenytoin and carbamazepine therapy, while there is a relative polymorphism of the clinical features.

Carbamazepine↗
PubMed1993

Marden-Walker syndrome: a case report and a critical review of the literature.

We present a patient with blepharophimosis, joint contractures, immobile facies, decreased muscular bulk, postnatal growth retardation, developmental delay, micrognathia, cleft palate, camptodactyly, arachnodactyly, pectus, kyphoscoliosis, hypospadias, and absent deep tendon reflexes. These findings are consistent with Marden-Walker syndrome (MWS). Twenty-two additional cases in the literature are reviewed. Diagnostic criteria are proposed, and the spectrum of variability is discussed. Evidence for autosomal recessive inheritance is reviewed as is the differential diagnosis. Possible pathogenetic mechanisms are considered.

Abnormalities, Multiple↗
PubMed1994

[A familial Charcot-Marie-Tooth disease type 1B (CMTD1B) manifesting a new mutation of myelin P0 gene].

A 15-year-old girl (case 1) was admitted to our hospital because of progressive muscle weakness of the lower limbs and numbness of the upper limbs. She noted these symptoms beginning at 13 years of age. Neurological examination revealed that deep tendon reflexes were absent and hypesthesia of touch and pain sensation were distributed in a glove-and-stocking pattern. Muscle weakness and atrophy were predominantly present in the distal portions of the extremities. There were obvious pes cavus and champagne-bottle shape deformities. The motor conduction velocity of the median nerve was markedly delayed and sensory potentials were not evoked in any nerves examined. Lumbar MRI showed thickening of the nerve radices. Cranial MRI showed thickening of the acoustic nerves, as well. Histological studies of a biopsied sural nerve revealed a marked decrease in the number of myelinated and unmyelinated fibers and remarkable onion bulb formation. The patient's clinical manifestations and histological findings were more severe than that seen in the usual case of CMT1A. Her mother (case 2, 39 years old) had similar neurological and electrophysiological findings. DNA duplication encoding peripheral myelin protein 22, was not detected in either case 1 or 2. Sequencing of DNA from these patients revealed the presence of a mutant allele containing an A- to G-substitution of nucleotide 245, which replaced tyrosine with cysteine in the extracellular Ig-domain of the P0 protein.

Adolescent↗