A case of subacute sclerosing panencephalitis developing 8 years after immunosuppressive treatment for acute lymphocytic leukemia]

A 13-year-old girl developed subacute sclerosing panencephalitis (SSPE) with atypical absence attacks as an initial symptom. Eight years earlier she had been treated for acute lymphocytic leukemia with cytotoxic treatment and radiotherapy, which had resulted in complete remission. She was first treated with an anticonvulsant because the atypical absence attacks and the presence of epileptic discharges on an EEG suggested epilepsy. However, with this mode of treatment the epileptic discharges did not disappear, but periodic high-voltage slow-wave complex discharges were revealed on subsequent EEGs. The antibody titer for measles virus in the cerebrospinal fluid and serum was elevated, confirming the diagnosis of SSPE. SSPE may arise, though rarely, in an individual in an immunosuppressive state due to congenital immunodeficiency or various kinds of malignancies, and also may arise several years after the contraction of measles infection. Our patient, however, lacked a past history of measles infection or immunization, suggesting the possibility that she had contracted measles during or shortly after the course of treatment for ALL.     

A case of familial essential myoclonus--electrophysiological study]

A case of familial essential myoclonus was reported with electrophysiological studies. A 58-year-old female presented with involuntary movement (myoclonic jerk) since age 6 years. At the age over 50, she developed difficulty in writing and fine finger movements. Two years ago, the diagnosis of hyperthyroidism was made, but she did not take any anti-thyroid medication. She was admitted to our hospital for further evaluation of myoclonus. On family history, her grandson (4-years-old) had myoclonus. On general physical examination, she showed a diffuse goiter. On neurological examination, she showed spontaneous myoclonus in the face, neck, trunk and the proximal limb muscles, especially in the left upper and right lower extremities. The myoclonus was exacerbated with auditory stimulation and emotional tension, but there was no temporal relationship between each stimulus and myoclonus. There was no myoclonus during sleep. Finger-to-nose test showed coarse intention myoclonus. Laboratory examinations including lysosomal enzyme assay revealed no abnormalities except for the decrease in total cholesterol (118 mg/dl) and the increase in serum lactate (19.7 mg/dl). The serum concentration of thyroxin was 18.4 micrograms/dl, that of triiodothyronine 3.0 ng/ml, and that of thyroid stimulating hormone (TSH) 0.027 microIU/ml. Anti-thyroglobulin antibody and anti-microsomal antibody were positive (X100, X6400). Neurological laboratory tests were normal including cerebrospinal fluid, peripheral nerve conduction study, electromyogram (EMG), muscle biopsy, cranial computed tomography and magnetic resonance imaging, and positron emission tomography. Electron transport system of the biopsied muscle was normal. So, we made a diagnosis of familial essential myoclonus and hyperthyroidism. In the electrophysiological studies, the electroencephalogram (EEG) was within normal limits.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hypnic headache syndrome: excessive periodic limb movements in polysomnography

We report a patient with hypnic headache syndrome associated with excessive periodic limb movements in sleep, which is a unique finding for this syndrome recorded in polysomnography. She had had daily hypnic headache attacks history for 10 years. Her headache attacks ceased immediately after lithium carbonate therapy and she has been headache-free for 5 months.     

Focal dystonia after removal of a parietal meningioma.

A patient presented at the age of 50 years with a right-sided, writing-specific dystonia which settled without treatment. Ten years later she developed focal seizures affecting the right leg and occasionally spreading to the right arm. A left parietal meningioma was removed and 2 years later she developed dystonic movements of her right hand. Gliotic changes were seen on magnetic resonance imaging in the superficial left parietal lobe. Mechanisms involved in the generation of dystonia associated with cortical injury are discussed.     

Unilateral loss of facial flushing and sweating with contralateral anhidrosis: Harlequin syndrome or Adie's syndrome?

A 45-Year-Old woman presented with a 10 year history of asymmetrical facial flushing and sweating after exertion or in hot weather. During these episodes the right side of her face remained dry and white, while the left side normally flushed. Sweating was impaired on the left side in the limbs and trunk. She also had areflexia in the lower limbs and slow pupillary reactions to light and darkness, as seen in Adie's syndrome. The topography of the sweating disorder suggested that the lesion involved the sympathetic pathways at the level of spinal cord. The relationship with the harlequin syndrome and related disorders is discussed.     

Unilateral choreic movements in idiopathic hypoparathyroidism]

We report a patient with idiopathic hypoparathyroidism associated with unilateral calcification of the basal ganglia and contralateral choreic movements. A 66-year-old woman was admitted to our hospital because of involuntary movements of the left limbs. Two years before the present admission, she had operations for bilateral cataracts. Eight months before admission, she had an onset of severe carpal spasms and pain in both arms and legs. These symptoms improved after one month's rest in a hospital. About a month before admission, she noted progressive difficulty in using her left hand, followed by difficulty in walking due to the abnormal involuntary movement of the left lower limb. Two weeks before admission her carpal spasms appeared again, bilaterally. On admission, she was alert. The general physical examination was normal. Neurological examination revealed choreic movements in the left hand, arm, leg and neck, and grimacing of the left face. She also complained of the spasms and pain in her hands, dysarthria and shortness of the breath. Chvostek and Trousseau signs were positive. Laboratory examination revealed marked decrease in serum calcium level (2.57 mEq/ml) and increase in inorganic phosphorus level (6.40 mEq/ml). Serum level of parathyroid hormone was less than 10 pg/ml. Ellthworth-Howard test was positive, in that the infusion of parathyroid hormone (100 u) elicited a marked increment of the urinary excretions of phosphorus and cAMP. X-ray examination of the spine revealed ossification of the posterior longitudinal ligament in the cervical region. CT-scans of the brain revealed calcification in the region of right globus pallidus and putamen.(ABSTRACT TRUNCATED AT 250 WORDS)     

Neuroepithelial cysts presenting with movement disorders: two cases

BACKGROUND: The authors present two cases of movement disorders caused by neuroepithelial cysts and highlight their management. Neuroepithelial cysts are ependymal or epithelial lined fluid collections of unknown etiology within the central nervous system parenchyma with no obvious ventricular or subarachnoid connection. Most cysts are asymptomatic, however, some present with seizures, mass effect, or rarely with movement disorders. CASE REPORTS: The first patient, a 27-year-old female, presented with progressive weakness, dystonic posturing, tremor, ballismus and choreoathetotic movements of her right upper extremity. Her symptoms improved after stereotactic drainage of a neuroepithelial cyst in her basal ganglia but recurred within a year. The second case, a 56-year-old female, presented with diplopia, nystagmus, gait imbalance and hemiparesis. Her symptoms improved after stereotactic drainage of a midbrain neuroepithelial cyst. The cyst reaccumulated over the next few years and she became symptomatic with left arm tremor and facial weakness. Aspiration was again performed with symptomatic improvement for nine months. Her tremor recurred and a cyst access device was placed stereotactically. She improved and has remained stable for over a year. CONCLUSION: Simple stereotactic drainage of neuroepithelial cysts has a high recurrence rate. The authors recommend considering placement of a drainage device to facilitate aspiration of the cyst fluid during follow-up, if needed.     

A case of chorea gravidarum with moyamoya disease]

A 16-year-old girl developed acute left choreic movements during her fourth week of pregnancy. She had sometimes had transient ischemic attacks since she was 10 years old. During the eighth week of pregnancy, a brain MRI showed old ischemic lesions deep in the right frontal white matter. Her angiograph revealed a complete obstruction of the terminal portion of the right internal carotid artery with a developed moyamoya net work. After her abortion, all involuntary movements completely subsided. The choreic movements might have been caused not only by ischemia, but also by enhanced dopaminergic sensitivity mediated by elevations in female sex hormones due to pregnancy.     

Postencephalitic chronic granulomatous disease

This report details the evolution of a case of herpes simplex encephalitis to chronic granuloma in a 13-year old female who, at the age of 8, suffered herpes simplex virus type 1 encephalitis. Eight months later, she developed an intracranial hypertension syndrome with the onset of a new lesion in the necrosed zone of her right temporal lobe, with no viral presence in the cerebrospinal fluid. The histologic characteristics were those of chronic granuloma with multinucleated giant cells and calcifications. Initially treated for neurosarcoidosis, the patient remained steroid-dependent for 4 years and the steroids could only be withdrawn by treatment with Infliximab. This type of evolutive pattern has probably been completely overlooked until now; it can only be accurately diagnosed by biopsy. It was initially mistaken for neurosarcoidosis.     

A contemporary case of encephalitis lethargica

We present a clinicopathological report of a recent fatal case of a 27-year-old woman whom we consider to have had encephalitis lethargica. Clinical features of note were a presentation with vertigo, persistent vomiting and sleep disturbance including marked daytime somnolence and vivid nightmares. On examination, she had impaired slow pursuit vertical eye movements, dysarthria, an expressionless face and slow tongue movements. She went on to develop gross supranuclear gaze palsy, neck rigidity, bradykinesia, blepharospasm, profound somnolence and anarthria but no tremor, weakness or impairment of cognition. She died after an illness lasting 12 months. On investigation, the cerebrospinal fluid was found to contain a very high level of IgG with oligoclonal bands but no cells. Post-mortem examination revealed an active encephalitis, mainly centered on the upper brainstem and diencephalon with extensive Purkinje cell loss and marked plasma cell infiltrates and morula cells. No virus was recovered.     

Unilateral palatal myoclonus with peculiar ocular movements--neurotological studies and MRI

This is the first report of unilateral palatal myoclonus with which two different ocular movements were synchronized. A 55-year-old woman was admitted to our hospital due to intubation and dysarthria of sudden onset after three similar attacks for these four years. On admission right ptosis, hypalgesia in the right face, right facial nerve palsy, dysarthria, bilaterally increased deep tendon reflexes and trunkal ataxia were noted. Continuous repetitive contractions at 110/min frequencies were observed in the left soft palate, pharynx and larynx. On electronystagmography and electro-magnetic search coil system (Skala system), two different ocular movements, i.e., rotary oscillation with torsion and nystagmus to the right, successively alternated each other at random during eyelids closure. They were synchronized with palatal myoclonus. True nystagmus synchronized with palatal myoclonus has not been reported. When she calculated, rotary oscillation disappeared. In sleep polygraphy, rotary oscillation reduced in amplitude in stage 1 and disappeared in stages 2, 3, 4 and REM. On the other hand, the nystagmus reduced in amplitude in stage 1 and 2 and disappeared in stages 3, 4 and REM. The direction of nystagmus was converted to the left in stages 1 and 2. Similarly, in a drowsy state induced by intravenous injection of 7 mg diazepam, the direction of the nystagmus was converted to the left. On brain magnetic resonance imaging (MRI) right inferior olive was identified as a well circumscribed, enlarged increased signal area on T2-weighted and proton density-weighted images in addition to the lesions of infarcts in left corona radiata, posterior limb of right internal capsule and tegmentum pontis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Rapid spontaneous postpartum remission in a case of chronic inflammatory demyelinating polyradiculoneuropathy associated with pregnancy]

A 28-year-old woman developed numbness and weakness of the hands and arms when she was 8 months pregnant, and weakness worsened gradually. However, weakness started to spontaneously subside immediately after delivery and she felt almost recovered several hours later. But weakness and numbness recurred one week after delivery and she was admitted to our hospital. Neurological examination revealed moderate weakness and disturbance of the deep and cutaneous sensations in the upper and lower extremities, and marked decrease of the deep tendon reflexes. The upper extremities were more severely affected. Nerve conduction study showed marked decrease in the motor conduction velocities. Cerebrospinal fluid showed increase of protein without pleocytosis. Teased preparation of the biopsied sural nerve showed occasional internodal segments with thin myelination, indicating demyelination and remyelination. A diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy was made. Treatment with prednisolone markedly improved the weakness and hyporeflexia as well as the cerebrospinal fluid protein. Postpartum rapid remission may have been produced by rapid increase of endogenous steroid hormone in the blood by its massive excretion during delivery.     

An autopsy case of primary central nervous system lymphoma with diffuse intracerebral lesion]

A case of primary central nervous system lymphoma (PCNSL) who initially showed clinical pictures like encephalitis and diffuse lesions on MRI was reported, including postmortem pathologic examinations. A 68-year-old woman was seen in March, 1990 with a 1-month history of the progressive gait disturbance. She was very unstable and could barely stand by herself, though she did not show any focal neurologic deficits. She showed no evidence of systemic diseases. The cerebrospinal fluid analysis was normal. T2-weighted image of MRI demonstrated the diffuse symmetric hyperintense lesions mainly in the periventricular white matter. The progressive intellectual decline and the spasticity of four limbs developed as the diffuse lesions on MRI gradually extended. Despite the administration of corticosteroids under the presumptive diagnosis of PCNSL, she rapidly fell into the apallic syndrome within two months. Her EEG showed periodic synchronous discharges. Three months later, she suddenly developed signs of right uncal herniation. CT showed a large mass lesion in the right hemisphere. After the anti-edema therapy, signs of herniation regressed. The serial CT scans demonstrated a gradual decrease in the mass effect, while another multi-nodular lesions appeared and then disappeared one after another bilaterally. Eventually, the diffuse low densities in the cerebral white matter and the ventricular enlargement had remained. She died of bronchopneumonia eight month after the onset of symptoms. The clinical importance of the diagnosis of PCNSL which initially shows diffuse symmetric lesions without a mass is stressed. Postmortem examinations revealed PCNSL, diffuse, large cell type according to the Lymphoma Study Group classification. The lymphoma cells were proved to be B cell origin from the immunohistochemical study of frozen tissue.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of spontaneous intracranial hypotension without any history of positional headache]

A 52-year-old woman was admitted to our hospital with sudden diplopia, right ophthalmalgia, and occipital pain. The nature of her headache was continuous, and changing her position provided no relief. Three weeks before her admission, she experienced continuous cough and rhinorrhea during a few days. On admission, her neurological examination revealed right abducens paralysis, and right lateral gaze aggravated diplopia. Brain magnetic resonance imaging scan demonstrated diffuse pachymeningeal enhancement by gadolinium. Radioisotope cisternography showed a leak of cerebrospinal fluid from near the third lumbar vertebra. The first lumbar puncture revealed an initial pressure of 0 mm H2O. She was given a diagnosis of spontaneous intracranial hypotension due to a leak of the cerebrospinal fluid. During her hospitalization, she complained of vertigo and nausea. We found horizontal gaze nystagmus to left side and sensorineural deafness in her left ear, which improved in a few days. After treatment by a lumbar epidural continuous infusion of saline and a lumbar epidural blood patch, her headache and abnormal MRI findings had improved. The continuous cough before her admission may be the cause of the leak of cerebrospinal fluid. We had difficulty in diagnosis only by the history and clinical examination, because she had no history of orthostatic headache.     

Hemicrania with massive autonomic manifestations and circumscribed eyelid erythema

OBJECTIVES: To describe a unilateral headache that in addition to the typical shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating and rhinorrhea (SUNCT) syndrome - traits with excessive and ipsilateral autonomic phenomena - had circumscribed eyelid erythema and adjacent ocular redness. OBSERVATIONS: A 60-year-old female had excessive, right-sided lacrimation and local pain at 15 years of age. Due to steadily increasing discomfort, with lacrimation and swelling over the outer part of the upper eyelid, the right lacrimal gland was removed at 20 years of age, with a suspicion of lacrimal gland adenoma. Preoperatively, symptomatic side mild-degree eyelid erythema/rhinorrhea were integral parts of the attack. After years with minor complaints, she, in the mid-twenties, experienced more long-lasting pain attacks, and pain soon became the main problem. A marked, distinct erythema on the lateral part of the right-sided eyelids and marked, localized 'eye redness' in the adjacent area were main ingredients of the attacks together with eyelid edema and viscous rhinorrhea. There were visible vessels below the eye, and telangiectasia of the upper eyelid. CONCLUSIONS: This headache has many similarities with SUNCT - but has several, grossly deviating traits: the temporal aspects, excruciatingly intense pain attacks, and above all marked, lateral eye-lid erythema, and adjacent, massive ocular reddening. This constellation probably alienates it from SUNCT.     

Chorea associated with antiphospholipid antibodies]

We report a 21-year-old woman in whom chorea was associated with antiphospholipid antibodies. In August 1986, she developed involuntary movement which started in the right hand but subsided spontaneously. In September 1988, she again developed right-sided involuntary movements which started in the right hand but rapidly progressed to involve the whole of the right side. In September 1990, she was admitted to our hospital for investigation of choreiform movements, because her involuntary movements had progressed to involve all four extremities. She had no family or past history of chorea, psychiatric, rheumatological or vascular disease. On admission, she had difficulty in speaking and swallowing due to choreiform movements of her mouth and tongue. Her gait was unsteady. On walking she had wild gyrations of the arms. Choreiform movements of all four extremities, neck, face, mouth and tongue were present at rest, more marked on the right side. There was no other neurological deficits. She had none of the classical features of SLE. She had none of the complications commonly associated with antiphospholipid antibody syndrome (APS) (i.e., recurrent spontaneous abortion, thrombosis and thrombocytopenia). Laboratory tests revealed that antinuclear antibody was present. Cardiolipin antibody (VDRL) was positive but specific tests for syphilis were negative. Anticardiolipin antibodies were present. All coagulation studies have failed to reveal lupus anticoagulant. Brain CT, MRI, 123IMP-SPECT and cerebral angiography were normal. Associated with her chorea, she had the serological but not the clinical features of APS. We suggest that antiphospholipid antibodies should be looked for in all unexplained cases of chorea, even when the associated clinical signs of APS are absent.     

Homocystinuria with generalized chorea and other movement disorders: a case report]

We report a 50-year-old right-handed woman who, at age 20, was diagnosed to have homocystinuria presumably due to cystathionine beta-synthase deficiency. At age 40 years, involuntary movements developed insidiously, affecting her face, neck and upper limbs. During the next 10 years, involuntary movements progressed and she could no longer walk. Examination disclosed bilateral lens dislocation and marfanoid skeletal deformity. Muscle strength was mildly decreased in the right arm and bilateral legs. There was hyperreflexia in the right upper and bilateral lower extremities without Babinski sign. Muscle tone was mildly increased in the neck and bilateral upper extremities. She had persistent generalized choreic movements, dystonia affecting the face and neck, and resting and postural tremor in the upper limbs and tongue. A cranial CT scan showed an old low density lesion in the left frontal area. Involuntary movements in homocystinuria is rare and, to our knowledge, only 9 cases have been reported. This case was unique in that involuntary movements developed in the adulthood.     

A case of multiple sclerosis with paroxysmal attacks of facial paresthesia, unilateral hand tremor, epigastric pain and urinary incontinence]

A Japanese woman, aged 42, was admitted because of paroxysmal attacks consisting of paresthesia of the left face, tremor in the right hand, epigastric pain and urinary incontinence. A year prior to the admission, she noticed some difficulty in writing, dysarthria and unsteadiness of walking. These symptoms had been persistent since then. At the end of March, 1991, these symptoms rapidly worsened, and she fell down frequently. She also experienced pain behind both eyes, numbness in her left fingers and toe, urinary frequency and the above-mentioned attacks. Neurological examination disclosed bilateral internuclear ophthalmoplegia and upbeating nystagmus on upward gaze, titubation in the head, scanning speech, dysmetria in all limbs, exaggerated reflexes in jaw and both legs, bilateral extensor plantar reflexes and ankle clonus. SEP showed delayed cortical response with stimulation of the median nerves bilaterally and of the right posterior tibial nerve. P40 was absent with the left posterior tibial nerve stimulation. VEP was normal. T2-weighted image of MRI showed multiple high intensity areas located around the third ventricle, crus cerebri and the right upper part of the pons. The diagnosis of multiple sclerosis was made. Each paroxysmal attack started with numbness in the left face and burning sensation in the neck. Almost simultaneously tremor in the right hand began. The surface EMG showed the rhythmic contractions in the dorsal hand muscles and wrist extensors at a frequency of 6-7 Hz, and sometimes it revealed synchronized contractions of finger flexors and the dorsal hand muscles. A few seconds later she felt painful sensation in the epigastric region, and the tremor gradually increased in its intensity.(ABSTRACT TRUNCATED AT 250 WORDS)     

Sustained visual recovery after treatment with intrathecal methotrexate in a case of optic neuropathy caused by chronic lymphocytic leukemia.

A 68-year-old woman with chronic lymphocytic leukemia (CLL) had acute optic neuropathy associated with cerebrospinal fluid evidence of meningeal spread of CLL. There was no evidence of a hematologic relapse. After undergoing four weekly doses of intrathecal methotrexate, vision improved dramatically and spinal fluid became normal. Four years later, she has near normal vision in the affected eye and remains in hematologic remission. This is the first reported case of successful treatment of optic neuropathy in CLL with intrathecal methotrexate alone.     

Spinal drop metastasis of choroid plexus papilloma.

A rare case of choroid plexus papilloma (CPP) with spinal drop metastasis is presented. A 51-year-old woman was operated on for a tumor of the fourth ventricle with histopathological diagnosis of CPP. Seven years later, she was re-admitted with low back pain. MRI showed multilobulated tumor at the lumbosacral subarachnoidal space with histopathological diagnosis of CPP. Thus, CPP can spread via the cerebrospinal fluid pathways and cause drop metastasis.