A rare case of trapped fourth ventricle: an unique symptom appeared after fourth ventriculo-peritoneal shunting]

We experienced a unique case of trapped fourth ventricle after shunting for post-meningitic hydrocephalus. A five-year-old infant was diagnosed as meningitis shortly after his birth, and secondarily suffered from hydrocephalus. He underwent lateral-ventriculo-peritoneal shunting, fourth-ventriculo-cisterna-magna shunting and so on, but bilateral abducens palsy appeared. The following head CT and MRI revealed "trapped fourth ventricle". Though there are several case reports of trapped fourth ventricle with abducens palsy, most of them followed enlargement of the fourth ventricle; nevertheless in our case, abducens palsy appeared when the fourth ventricle reduced in size and the symptom vanished when it enlarged. We thought that a traction force to the abducens nerve had occurred also in the condition of reduced fourth ventricle size, because there would have been a dense adhesion after meningitis in his subarachnoidal space. We tried to improve his symptom in one way or another by keeping the fourth ventricle in appropriate volume. His abducens palsy was controlled by switching the on-off valve between forth ventricle and peritoneum. We expect that a higher-pressure programmable shunt valve or a lower-flow-regulating shunt system be invented in order to cope with the cases like ours.     

周围神经缩窄术在治疗脑瘫痉挛肢体中的应用

目的探讨周围神经缩窄术在治疗脑瘫痉挛性肢体的应用及手术效果.方法对31例脑瘫患儿的53肢痉挛肢体共行周围神经缩窄术82例.一次手术单神经干缩窄术31例,多神经干组合神经缩窄术22例.共行肌皮神经缩窄11例,正中神经缩窄15例,胫神经缩窄37例,闭孔神经缩窄6例,坐骨神经缩窄11例,腓深神经缩窄2例.结果所有病例随访6个月-1年4个月,平均10.8个月.以Ashworth分值、ROM关节活动度、随意功能改善和患儿家长满意度进行评价.术后肌痉挛缓解率97.6%,随访期间随意运动功能改善88.7%,不同程度痉挛复发4.9%.结论周围神经缩窄术对治疗脑瘫痉挛肢体具有较高的实用价值.     

An autopsied case of dementia with Lewy bodies with supranuclear gaze palsy

A 66-year-old man had suffered from a slow and steady decline in both physical and cognitive function for four years. He showed bradykinesia and small step gait with supranuclear vertical gaze palsy, especially upward gaze palsy. He was started on levodopa therapy but without response. A diagnosis of progressive supranuclear palsy was clinically suspected. He died at age 69. Pathologically, many alpha-synuclein positive inclusions were detected both in the brain stem and cerebral cortices, and the diagnosis of dementia with Lewy bodies was made. Scattered alpha-synuclein-positive inclusions and threads, which may be a pathological substrate for supranuclear gaze palsy, were identified in the rostal midbrain. From a review of five cases of dementia with Lewy bodies with supranuclear gaze palsy including this case, the absence of falls in the early stage of the disease, fluctuation of cognition, hallucination and vertical gaze palsy with a more severe defect in the upward direction distinguished dementia with Lewy bodies with vertical gaze palsy from progressive supranuclear palsy. In the differential diagnosis of parkinsonism with gaze palsy, clinicians should consider dementia with Lewy bodies with gaze palsy as well as progressive supranuclear palsy.     

The changing role of dynamic psychotherapy in psychiatric practice

The author reviews the range of accepted indications for dynamic psychotherapy when he first began practice after World War II and describes factors that have played a role in the current undervaluing of this treatment approach. He attributes much of the change to research that has produced a different understanding of many of the conditions treated by psychiatrists and has placed greater emphasis on their medical and biological aspects than on their psychological aspects. He also attributes many alterations in current practice to the change from a two-party to a three-party reimbursement system for psychiatric services. On the basis of his practice, the author illustrates his belief that dynamic therapy continues to have a role in today's psychiatric practice. He describes one category of patients in particular-those who seek treatment for "problems of living"-who can be helped by dynamic psychotherapy. The author makes a case for freeing dynamic therapy from the need to rely exclusively on the criteria of the medical model for its legitimacy.     

Sudden onset odontoid fracture caused by cervical instability in hypotonic cerebral palsy

Fractures of the upper cervical spine rarely occur but carry a high rate of mortality and neurological disabilities in children. Although odontoid fractures are commonly caused by high-impact injuries, cerebral palsy children with cervical instability have a risk of developing spinal fractures even from mild trauma. We herein present the first case of an odontoid fracture in a 4-year-old boy with cerebral palsy. He exhibited prominent cervical instability due to hypotonic cerebral palsy from infancy. He suddenly developed acute respiratory failure, which subsequently required mechanical ventilation. Neuroimaging clearly revealed a type-III odontoid fracture accompanied by anterior displacement with compression of the cervical spinal cord. Bone mineral density was prominently decreased probably due to his long-term bedridden status and poor nutritional condition. We subsequently performed posterior internal fixation surgically using an onlay bone graft, resulting in a dramatic improvement in his respiratory failure. To our knowledge, this is the first report of an odontoid fracture caused by cervical instability in hypotonic cerebral palsy. Since cervical instability and decreased bone mineral density are frequently associated with cerebral palsy, odontoid fractures should be cautiously examined in cases of sudden onset respiratory failure and aggravated weakness, especially in hypotonic cerebral palsy patients.     

Clinical analysis and the results of surgical treatment of aneurysms of the vertebrobasilar system]

The authors present a clinical analysis of 42 patients with vertebro-basilar aneurysms considering symptoms, course and complications of subarachnoid haemorrhage and localization of aneurysms: 88% were admitted after SAH, 47.6% had basilar bifurcation aneurysm. The ischaemic deficits due to vasospasm were observed in 5.4% of cases. Rebleeding occurred in 29.7% of patients, most of them died. 23 patients were operated on. In 69.9% of cases the operation was successful. The operative mortality was 17.4%. Temporary third nerve palsy was the most often postoperative complication.     

Use of transpedicular fixation in treatment of thoraco-lumbar spinal injuries

In Orthopaedic University Department in Lublin in years 1993-1998 39 patients with thoraco-lumbar spine injuries were treated including 34 fractures and 5 luxations. All of them were treated by surgery. Complete neurological palsy (Frankel A) occurred in 16 patients. Grade E on Frankel scale e.g. no neurological symptoms was in 2 cases. The operations consisted of wide laminectomy with interpedicular joints resection. It gave chance to mobilise the dural sac with its contents. In this situation it was possible to remodel the anterior wall of spinal canal by removal of fractured bony pieces from vertebral body or fibroid remnants of damaged disc. Injured segments of the spine were stabilized by Kluger's fixateur. In 20 cases reduction of vertebral body fracture and filting of bone loss by autogenic spongiosa grafts from iliac crest as proposed by Daniaux was done. In 6 patients posterior interbody spondylodesis was done also with iliac crest grafts. In 9 patients who had only reduction of injured segments, without vertebral body reconstruction and spondylodesis loss of correction occurred after fixateur removal. In 3 patients fatigue break of screws or stabilizing rods developed. It happened 12-16 months after surgical procedure. In patients with primary complete neurological palsy from the level of injury (Frankel A) no symptoms of function improvement appeared higher than one grade in Frankel's scale.     

Ventriculovenous shunt to the proximal segment of a ligated neck vein. A new surgical technique for shunting the cerebrospinal fluid to the venous circulation (preliminary report).

The author proposes ventriculovenous shunts to a ligated neck vein (external jugular or facial) for the treatment of hydrocephalus. He postulates that the ligated neck vein is filled with CSF and becomes an extension of the shunt tube, which prevents venous thrombosis. The pressure of the shunt at the venous tube was shown to be about 90 mm H2O avoiding excessive ventricular decompression. Reflux of blood to the ventricle can occur if the infant's cranium is compressible and must be prevented by protecting the cranium from external pressure with a bandage or helmet or by incorporating a one-way valve into the shunt. 31 cases of advanced hydrocephalus were thus treated during a 4-year period. Ages ranged from 15 days to 55 years, the cranium was rigid in 13 and compressible in 18. A valve was used in only two cases. Shunts were patent 7-10 days after surgery, as shown at autopsies in two early postoperative deaths. In two cases meningitis occurred, one of them died. The shunt was removed in one case on account of meningitis and in 4 cases due to cervical CSF leakage. The former and three of the latter were reoperated with success. Three cases had distal shunt obstruction, two due to kinking of the tube, and one due to reflux of blood. One of the former died. 23 patients benefited from the operation, 5 of which were operated on twice. The follow-up period was from 0.5-44 months (average 11.5 months). The last two cases were operated on using one-way valve and a spiral wire in the venous segment of the tube. They are well 2 and 3 months, respectively, following surgery.     

Isolated superior division oculomotor palsy in a child with spontaneous recovery.

A 10 year old boy with a superior division palsy of the left oculomotor nerve is reported. He had a flu-like illness 1 week before the onset. The computed tomography and magnetic resonance imaging scans were normal. Laboratory data for evaluation of infection, diabetes mellitus and myasthenia gravis were normal. The symptoms spontaneously disappeared after 2 months. The course of the illness in conjunction with the negative laboratory findings made this case an example of partial paralysis of the third nerve related to viral infection. In the literature, only several cases with isolated divisional palsy of the oculomotor nerve were found after a viral infection. Inferior division palsy has been reported in five children. Superior division palsy has been published in only two cases. Divisional palsy is more common among children and resolves spontaneously. This rare but important clinical entity is one of the differential diagnoses in oculomotor nerve palsies, particularly in children, which are neuroradiologically undiagnosed. It occurs after a viral infection and may affect a superior or inferior division alone.     

Delayed Bilateral Abducens Nerve Palsy after Head Trauma

Although the incidence of unilateral abducens nerve palsy has been reported to be as high as 1% to 2.7% of head trauma cases, bilateral abducens nerve palsy following trauma is extremely rare. In this report, we present the case of a patient who developed a bilateral abducens nerve palsy and hypoglossal nerve palsy 3 days after suffering head trauma. He had a Glasgow Coma Score (GCS) of 15 points. Computed tomography (CT) images demonstrated clivus epidural hematoma and subarachnoid hemorrhage on the basal cistern. Herein, we discuss the possible mechanisms of these nerve palsies and its management.     

The Rett syndrome in males

Two young males in their thirties are reported with a clinical history and examination indistinguishable from typical females with the Rett syndrome. Both had normal early development. The first patient had a regression by the end of the second year. He was late in walking, had prominent hand-wringing from the age of 4 years, and non-progressive dystonia from the age of 14 years. He is still ambulatory. Seizures which started at the age of 18 months have been easily controlled. The second patient has had a severe seizure disorder since the age of 7 months. In his early teens, he lost ambulation and his height and weight fell below the 2nd percentile. He has severe foot dystonia without spasticity. Both patients have a normal head size and no evidence of atrophy on a CT scan of the brain. Both had kyphoscoliosis in their teens. It is difficult to evaluate the incidence of such cases. Little attention being paid to the normal early development, they hide behind vague diagnoses such as cerebral palsy, static encephalopathy, and behavior disorder. Dystonia is often confused with spasticity, the lack of paralysis is not appreciated, apraxia and hand wringing are assumed to be self-stimulatory behaviors.     

Joubert syndrome labeled as hypotonic cerebral palsy

Joubert syndrome (JS) is a rare autosomal recessive disorder with cerebellar vermis hypoplasia and complex brainstem malformation. The diagnosis of cases can be difficult as the presentation can be similar to cases of cerebral palsy. We present a case of JS in an 18-month-old girl who presented to pediatric rehabilitation with a diagnosis of hypotonic cerebral palsy and abnormal eye movements. The brain MRI confirmed the typical brain malformations.Cerebral palsy (CP) is a group of disorders of posture and movement development due to a non-progressive insult to the developing brain.1 The timing of the insult could be in the prenatal, perinatal, or postnatal period.2 Most of the clinical classifications of CP depend primarily on the tone and distribution of the motor abnormality; notwithstanding, interest in the functional classification has become popular among clinicians and therapists.3 Cerebral palsy can be classified according to the tone into spastic, dyskinetic, hypotonic, or mixed type. Spastic CP is the most common type of CP; in which, the muscle tone is increased whereas hypotonic CP is usually rare and present in children with varying degrees of reduced tone and delayed motor milestones.4 The brain MRI was found to have a strong correlation with clinical findings in most cases of CP and has helped in identifying the various etiologies.5 Our objective in presenting this particular case is to highlight the importance of thorough investigations in those labeled as hypotonic CP.     

One-and-a-half syndrome in ischaemic locked-in state: a clinico-pathological study.

Five patients with "locked-in" syndrome and dysconjugate palsy of horizontal gaze were studied. In all cases internuclear ophthalmoplegia due to dysfunction or destruction of the median longitudinal fasciculus was combined with an ipsilateral gaze palsy, producing the "one-and-a-half" syndrome. Clinical and electro-oculographic examination suggested involvement of the paramedian pontine reticular formation when all ipsilateral saccades were abolished, when exotropia of the contralateral eye was present, and when vestibular stimulation showed full conjugate deviation to the damaged side. Involvement of the abducens nucleus was suggested when the palsy of ipsilateral gaze was not dissociated on vestibular stimulation. In three cases these clinical deductions were confirmed by the pathological study, which showed a corresponding destruction of the median longitudinal fasciculus, paramedian pontine reticular formation and abducens nucleus. In one case the one-and-a-half syndrome evolved into a total horizontal gaze palsy, which corresponded to involvement of the abducens nucleus contralateral to the initially destroyed paramedian pontine reticular formation. Vertical oculocephalic response disappeared, because of destruction of the median longitudinal fasciculus on both sides (bilateral internuclear ophthalmoplegia). Patients with the locked-in syndrome provide a unique situation in which complex pontine oculomotor disturbances may be studied, because consciousness is preserved. In these patients, dissociated and dysconjugate oculomotor palsy may have been underestimated.     

Self-induced depersonalization syndrome.

The author reports two cases in which depersonalization occurred during the waking consciousness of individuals who had engaged in meditative techniques designed to alter consciousness. Psychiatrists should be aware of this phenomenon, as the number organizations in the "consciousness movement" is increasing, and should ask people manifesting depersonalization about any involvement in activities leading to altered states of consciousness. In some cases it might be appropriate to refer such patients to responsible groups that teach altered consciousness by meditation as an egosyntonic desirable state. The author cautions against the use of phenothiazines in cases where depersonalization is a prominent feature.     

An autopsy case of pericallosal lipoma with mental retardation]

The patient was a 69-year-old male who had visited our psychological department due to mental retardation. Pericallosal lipoma was indicated by MRI taken during treatment. On December 30, 1998, the patient fell from steps and struck his forehead hard, and then limb palsy occurred. He was emergently brought to our hospital. Under the suspected diagnosis of traumatic cervical spinal cord injury, preventive therapy was performed to observe the clinical course in which paralysis gradually improved. On January 22, 1999, however, he died of cardiopulmonary arrest due to sudden suffocation. In general, the connection of choroid plexus lipoma and pericallosal lipoma is bilateral in most cases. In our patients, MRI suggested that a connection with cerebral ventricles through choroidal fissure existed with no right and left difference, however, autopsy findings revealed that the lipoma existed along the left cerebral arch with obvious connection only with the left choroid plexus. The present case seemed to be a very rare case.     

The early markers for later dyskinetic cerebral palsy are different from those for spastic cerebral palsy

Qualitative abnormalities of spontaneous motor activity in newborns and young infants are early predictive markers for later spastic cerebral palsy. Aim of this research was to identify which motor patterns may be specific for later dyskinetic cerebral palsy. In a large, prospectively performed longitudinal study involving four European hospitals we identified twelve cases with the relatively rare condition of dyskinetic cerebral palsy and compared their early motor development with twelve spastic cerebral palsy cases and twelve controls. From birth to the fifth month post-term, all infants were repeatedly videoed and their spontaneous motor patterns, including general movements, were assessed. Until the second month post-term, the infants that later became dyskinetic displayed a poor repertoire of general movements, "arm movements in circles" and finger spreading. Abnormal arm and finger movements remained until at least five months and were then concurrent with a lack of arm and leg movements towards the midline. Later dyskinetic infants share with later spastic infants the absence of fidgety movements, a spontaneous movement pattern that is normally present from three to five months. Qualitative assessment of spontaneous motor patterns enabled us to identify infants at high risk for dyskinetic cerebral palsy early in life. Additionally, we were able to discriminate them from those infants at high risk for later spastic cerebral palsy. This is a matter of significant clinical relevance because the two types of cerebral palsy ask for different management and early intervention.     

Facial palsy in multiple sclerosis

Facial palsy occurred in 21 (19.6%) of 107 Japanese patients with multiple sclerosis (MS) during a mean follow-up period of 4.3 years. We observed residual signs of facial palsy in five other patients in whom acute onset was confirmed from medical records. Facial palsy began on average 7.6 years after the onset of MS but in five patients (4.7%) was the first symptom of MS, preceding the next MS symptom by 0.5–3 years. Facial palsy was usually associated with other brainstem signs, while two patients showed only facial palsy 1 and 3 years after the onset of MS. Twenty-one (84.0%) of the 25 patients who underwent brain magnetic resonance imaging (MRI) showed brainstem lesions in the pontine tegmentum ipsilateral to the facial palsy. However, the two patients without other symptoms or signs had no apparent causal lesion on MRI, which suggests difficulty in differentiating idiopathic Bell’s palsy from MS- associated facial palsy by MRI, although it has an excellent capacity to detect causal lesions of facial palsy associated with MS. Received: 6 March 1997 Received in revised form: 25 July 1997 Accepted: 12 August 1997