A case of Cogan's syndrome associated with multiple cranial neuropathy

A 52-year-old woman was admitted to our hospital because of disturbance of right visual acuity and double vision. At 38-year-old she became deaf bilaterally and experienced many vertigo attacks. She was diagnosed as Ménière disease. At 45-year-old vertigo attacks disappeared. At 47-year-old right peripheral facial nerve palsy developed transiently with interstitial keratitis and episcleritis of the both eyes. Oral adrenocorticosteroid therapy produced an improvement of interstitial keratitis and episcleritis. On admission, ophthalmological examination revealed bilateral interstitial keratitis and episcleritis, right retrobulbar optic neuritis and she was proven to have bilateral sensorineural deafness by otologist. Neurological examination revealed right abducens nerve paresis. Laboratory examinations revealed slightly increased erythrocyte sedimentation rate. CRP was positive. Serological tests for syphilis were negative. CSF showed mildly elevated protein level. Orbital CT scans revealed the swelling of right optic nerve. Cerebral MRI showed multiple high spotty areas in left thalamus, bilateral basal ganglia and deep white matter in T2 weighted images. After treatment with adrenocorticosteroid, right optic neuritis and abducens nerve paresis improved together with bilateral interstitial keratitis and episcleritis. Multiple cranial neuropathy may develop with Cogan's syndrome.     

Chronic inflammatory demyelinating polyradiculoneuropathy presenting localized radiculopathy as an initial symptom

We reported a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), presenting localized radiculopathy as an initial symptom. The patient, a 38-year-old man, developed muscle atrophy of right shin and calf. In April 1988, neurological examination revealed decreased superficial sensation and muscle atrophy in right L-4 distribution. MRI of the lumbar spinal cord and myelography were negative. In April 1989, he was admitted to Chiba University Hospital. There were slight weakness of right ankle extension and flexion, muscle atrophy of bilateral shin and calf (that of the right leg was more marked), decreased sensation of polyneuropathy type in all modalities and generalized hypo- or a-reflexia. Nerve conduction study revealed marked prolongation of F response and focal conduction block in various site. The CSF was acellular and total protein was 177 mg/dl. Sural nerve biopsy disclosed decreased number of large myelinated fibers and scattered thin myelinated fibers. This case indicates that CIDP may present localized radiculopathy in early phase and is important to point out various mode of onset among CIDP.     

A Japanese case of Poland-M?bius syndrome]

We herein report a Japanese case of Poland-M?bius syndrome. The patient was a 19-year-old female. She was the product of a full-term forceps delivery. Birth weight was 2500 g. She had a defect of the right pectoral muscle, and syndactyly of the right hand. When she was 10 days old, facial diplegia, bilateral abducens nerve palsy, and bilateral ptosis were also noted. She was admitted to our hospital at 19 years of age. On physical examination, she had microsyndactyly of the right hand, and her right pectoralis major muscle was absent. Neurological examination revealed bilateral abducens nerve paresis, mild impairement of the upward and adducting movement of both eyes and bilateral facial weakness and atrophy of the left side of her tongue. Her karyotype was normal. Neither R 1 nor R 2 response was evoked in the blink reflex on either side. Brain MRI disclosed thin facial nerves and atrophy of the pons and medulla. Therefore, she was diagnosed as a case of Poland-M?bius syndrome. In this case, the facial nerves were considered to be hypoplastic.     

Two cases of hypertrophic cranial pachymeningitis associated with infection in the external auditory canal and paranasal sinus]

We here present two cases of hypertrophic cranial pachymeningitis exhibiting unique multiple cranial neuropathies, both of which were associated with otic and paranasal infections. Case 1: A 76-year-old woman developed headache after undergoing surgical dilatation of the external auditory canal, with subsequent development of a bacterial infection. Neurological examination reveled only bilateral hearing disturbance. MRI and CT scans demonstrated thickening of the dura mater and inflammatory granulation around the left cerebellar tentorium. Based on a diagnosis of hypertrophic pachymeningitis associated with previous infection, antibiotics were administered, followed by oral prednisolone therapy. This treatment relieved the headache and improved the MRI findings. However, 2 months later, the headache became worse and impaired movement of the soft palate, atrophy of the left side of the tongue, and atrophy of the sternocleidomastoideus muscle were noted. MRI revealed aggravated inflammatory changes around the left cerebellar tentorium and their expansion into the jugular foramen. Occlusive changes in the transverse and sigmoid sinuses were also seen. Case 2: A 78-year-old man developed bilateral visual loss, right frontal headache, and bilateral restriction of eye movement. He had been treated for phemphigus with prednisolone and azathioprine. MRI showed hypertrophic dura mater spreading continuously from the frontal base and ethmoid and frontal sinuses to the falx and right frontal lobe. Since Pseudomonas aeruginosa was cultivated in biopsy specimens from the dura mater, antibiotic agents were administered. The clinical symptoms resolved and MRI findings gradually improved.     

A case of motor neuron disease with presenile dementia showing bilateral degeneration of the pyramidal tract on cranial MRI]

A 58-year-old man developed dysarthria followed by a personality change. Subsequently, he developed muscle weakness and atrophy of the left upper and lower limbs, leading to repeated falls when he tried to walk. Neurological examination showed mild dementia, dysarthria, dysphagia, atrophy and fasciculation of the tongue, and muscle weakness and atrophy of all four extremities, particularly on the left side. Deep tendon reflexes were slightly diminished in the upper limbs and slightly exaggerated in the lower limbs without Babinski's sign. Cranial MRI revealed marked atrophy of the medial portions of the temporal lobes, more striking on the right, and T2-weighted imaging revealed symmetrical high-intensity signals from the posterior limbs of the internal capsules to the cerebral peduncles in the midbrain, extending to the pons on the left. 125I-IMP SPECT showed diffuse reduction of RI uptake in the frontal and temporal lobes, which was more marked on the right. We diagnosed this is a case of motor neuron disease with presenile dementia, which Mitsuyama et al. proposed as a new clinical entity, as well as a rare example of bilateral degeneration of the pyramidal tract on cranial MRI.     

A case of pseudo Tolosa-Hunt syndrome with bacterial infection and literature review]

We report a case with recurrent orbital pain and unilateral cranial nerve paresis mimicking Tolosa-Hunt syndrome. However, these features were most likely caused by bacterial infection because of beneficial response to antibiotics therapy. A 32-year-old man developed severe right orbital pain and diplopia. Neurological examination revealed right oculomotor paresis and 1st division of the right trigeminal nerve dysfunction. MR imaging revealed thickness of right cavernous sinus region with marked gadolinium enhancement. Cerebrospinal fluid (CSF) examination was initially normal. Treatment with steroid showed marked improvement. However soon after tapering of steroid dosage, his symptoms recurred and deteriorated. He was referred to our hospital because of second opinion. Neurological examination still showed right oculomotor paresis and 1st division of the right trigeminal nerve dysfunction with orbital and retro-orbital pain. Re-examination of CSF showed pleocytosis with neutrocytes dominancy and elevated protein concentration. Intravenous treatment with penicillin was initiated with marked improvement. There have been reported cases with bacterial infection resulting pseudo Tolosa-Hunt syndrome, which have good response to antibiotics treatment with excellent prognosis. It is speculated that bacterial infection might cause clinical features mimicking Tolosa-Hunt syndrome in our case.     

A case of brainstem and cerebellar infarctions related to basilar impression]

We report a 30-year-old man presenting with medial longitudinal fasciculus (MLF) syndrome after an afternoon nap. Magnetic resonance imaging revealed a right medial pontine tegmental infarction and right cerebellar infarctions. This patient was complicated with basilar impression detected on cervical X-ray and MRI. Three-dimensional CT angiography disclosed that the odontoid process migrated into the posterior fossa, thrusting the bilateral vertebral arteries postero-laterally. The mechanical stress on the bilateral vertebral arteries may have caused infarctions in the territories of the posterior circulation of this patient with basilar impression.     

Scleroderma,stroke, optic neuropathy: a rare association

A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature.     

Bilateral high-frequency synchronous discharges: a new form of tremor in humans

OBJECTIVES: To report bilateral high-frequency synchronous discharges in a patient with a sporadic form of olivopontocerebellar atrophy; to investigate the electromyographic pattern, the coherence and cospectral density across limbs, and the resetting effects of electrical stimulation over the posterior fossa; and to highlight the clinical, electrophysiologic, and radiologic features of this new form of tremor associated with posterior fossa disorders. DESIGN: Case study of a patient clinically exhibiting a sporadic form of olivopontocerebellar atrophy associated with cerebellar and brainstem atrophy. SETTING: Research unit, university hospital. MAIN OUTCOME MEASURES: Electromyographic studies, coherence and cospectral density analysis, and calculation of a resetting index based on the timing of measured bursts and predicted bursts for an electrical stimulus given over the posterior fossa at increasing delays. RESULTS: Surface electromyographic recordings in forearm muscles revealed a low-frequency postural tremor in the upper limbs, with episodes of highly coherent tremor at a frequency of 14 Hz. Squared coherence and cospectral density was strong between agonist and antagonist muscles in the left and right upper limbs and across limbs for the high-frequency discharges. Electrical stimulation over the posterior fossa reset the explosive high-frequency bursts. The resetting index was 0.82 Hz. CONCLUSIONS: Our results show that bilateral high-frequency synchronous discharges may be associated with the sporadic form of olivopontocerebellar atrophy. Bilateral coherent bursting and resetting of this explosive postural tremor following electrical stimulation over the posterior fossa strongly suggest that the brainstem plays a key role in the network involved in the genesis of rhythmic bursts. We suggest that the high-frequency discharges are due to repetitive discharges in the reverberating cerebello-precerebellar circuits.     

Trigeminal neuralgia in a patient with Dandy-Walker malformation.

BACKGROUND: Trigeminal neuralgia may be idiopathic or secondary to a number of cranial pathologies. We report a novel case of trigeminal neuralgia associated with Dandy-Walker malformation, which may be an etiologic factor. CASE DESCRIPTION: A 32-year-old male presented with shock-like pain in the somatosensory distribution of the right trigeminal nerve, which was refractory to all medication. MRI revealed a cystic lesion in the posterior fossa and a hypoplastic vermis. The pain was diagnosed as trigeminal neuralgia and was thought to be secondary to the Dandy-Walker malformation. The trigeminal neuralgia was treated successfully with radiofrequency thermocoagulation rhizotomy (RF-TR). CONCLUSION: Trigeminal neuralgia may be associated with Dandy-Walker malformation, however an etiological relationship is not proven. We suggest that traction on the trigeminal nerve may be significant. The posterior fossa cyst of Dandy-Walker malformation may be a complicating factor when considering microvascular decompression to treat the trigeminal neuralgia. Collapse of the cyst at surgery may destabilize the posterior fossa and further deform the trigeminal nerve. We suggest that RF-TR, which is minimally invasive and reliable, may be preferable.     

A case of radiculomyelitis following chickenpox in adulthood]

A 34-year-old woman presented with numbness in both lower limbs and weakness of right lower limb twenty six days after a primary varicella infection (chickenpox) associated with fever and rash. Neurological examination revealed numbness of both lower limbs, more severe on the right side, mild paresis of the right lower limb, particularly in the tibialis anterior muscle, and absent ankle jerk on the right. After admission, hyperalgesia appeared at the thoracic 10-12 levels. The T2-weighted MRI of the spinal cord revealed a high signal intensity lesion at the Th 9 level and gadolinium enhancement was seen in that lesion as well as in the bilateral posterior radicles and the left anterior radix at the Th 9 level. On needle electromyography, fasciculation was found in the right tibialis anterior and gastrocnemius muscles. The temporal dispersion of F-wave was seen in the right peroneal nerve. We diagnosed the patient suffered from radiculomyelitis following the primary varicella infection. The secondary immunological mechanism rather than direct viral invasion is most likely in our patient, because (1) neither VZV DNA, nor anti-VZV antibody was positive in the CSF, and (2) the duration was relatively long between the development of skin rash and that of neurological symptoms.     

A case of neuro-Beh?et's disease presenting with chorea]

A 46-year-old man was admitted to our hospital because of emotional instability and involuntary movement of the right upper limb. Neurological examination revealed inability to concentrate, emotional incontinence, recent memory disturbance, chorea of bilateral upper limbs and neck, and bilateral pyramidal signs. Brain MRI showed atrophy of bilateral caudate nucleus and diffuse abnormal intensity area with low intensity on T1-weighted images and high intensity on T2-weighted images in cerebral white matter around the lateral ventricles. Huntington's disease was suspected at first, but it was ruled out by DNA analysis. After admission, oral and genital aphthae developed and the CSF examination showed pleocytosis (273 leukocytes/mm3; 39 polymorphonuclear leukocytes and 234 lymphocytes), so we diagnosed this case as neuro-Beh?et's disease. Although basal ganglia is occasionally involved in neuro-Beh?et's disease, chorea is rare. Neuro-Beh?et's disease should be considered as a cause of chorea.     

Post-traumatic intradiploic leptomeningeal cyst of the posterior fossa in an adult

Intradiploic cysts in the posterior fossa are rare. We report a post-traumatic intradiploic leptomeningeal cyst in an adult and review the diagnosis and pathogenesis of this lesion.A 28-year-old woman presented with a headache and a hard mass in the occipital region. She had a history of head injury as she had fallen from a height at the age of 18 months. CT scans and MRI revealed an expanding intradiploic cyst with the density of cerebrospinal fluid (CSF) and thinning of the inner and outer tables. A bony defect of the inner table connecting to the cisterna magna was also visualized. At surgery, we detected free communication of the CSF between the cystic cavity and the subarachnoid space of the posterior fossa via a defect in the dura and inner table. The dural defect was sutured primarily and covered with the autogenous outer table.An intradiploic cyst of the occipital bone can be detected in adults and might be caused by remote head injuries during childhood. Prompt surgical repair of the dural and bony defect in an adult patient has an excellent prognosis.     

A case with posterior column ataxia associated with cerebellar ataxia and sensory neuropathy]

The patient was a 72-year-old man who had a history of subtotal gastrectomy for gastric ulcer at age of 37 years. He had no familial history of hereditary disorders. In 1980 he noticed mild ataxic gait which exaggerated while he closed eyes. The symptoms increased gradually, and four years later he noticed hypoesthesia of his soles. In 1983 he was admitted to the National Center Hospital for Mental, Nervous and Muscular Disorders for the first time. Neurological examination revealed dysarthria, ataxic gait, disturbance of coordination to a slight degree, and muscle strength of the upper and lower limbs were in normal range. Mild hypoesthesia of pain and temperature sensation, and marked decrease of deep sensation and vibration of the lower extremities were demonstrated. Romberg sign was positive. EMG studies revealed low amplitude of action potential and normal motor nerve conduction velocity. Biopsy of the sural nerve showed marked decrease of both large and small myelinated fibers. In 1998 he was admitted second time for the further examination. Laboratory examination including routine blood examination, blood chemistry including CRP, TPHA, vitamin B1, B2, B12, A, E, K, hexosaminidase A in leucocyte were in normal range. CSF was normal. Genetic studies including SCA 1, 2, 3, 6, DRPLA, CMT1A, CMTX 1 were all negative. MCV of lower limbs was in normal range, though SCV was not evoked in the upper and lower limbs. MRI studies showed mild atrophy of the bilateral lobulus of the cerebellum which was not so much changed in the last 5 years. The clinical symptoms revealed dominant posterior column disturbance, ataxia and sensory neuropathy. These combination was not described in the previous literature, and this case may be a new variant of the spinocerebellar degeneration.     

Two cases with chronic inflammatory demyelinating polyneuropathy showing high signal and enlargement of the brachial plexus in short TI inversion recovery of MRI]

We reported valuable MRI findings of the brachial plexus seen in two cases with chronic inflammatory demyelinating polyneuropathy (CIDP). Case 1 was a 44-year-old man who developed slowly progressive weakness and atrophy of the extremities with no sensory disturbances. Studies of CSF showed a normal level of protein and no increase of cell counts but nerve conduction studies demonstrated a significant conduction block between the axilla and the elbow in the right ulnar nerve. Case 2 was a 34-year-old male who had been suffering from distal limb weakness and sensory disturbance. Protein content in CSF was markedly elevated without pleocytosis, and nerve conduction studies revealed a conduction block between the elbow and the wrist in the right ulnar nerve. He received corticosteroid therapy, resulting in a good recovery. Brachial plexus in both cases showed enlargement with marked high signal on short TI inversion recovery (STIR) of MRI. STIR is a fat suppressed T2 weighted image and this technique is known to be useful to identify the morphology of peripheral nerve tissues. CIDP is one form of hypertrophic neuritis and the MRI findings seen in these two cases strongly support the diagnosis of CIDP.     

A patient with neuroborreliosis presenting gadolinium-enhanced MRI lesions in bilateral facial nerves]

We report a 33-year-old man with bilateral facial paralysis due to neuroborreliosis. About three weeks after rhinorrhea and fever lasting four days, he noticed fatigue in the legs and paresthesia in all four extremities. Another week later, he developed paresthesia in his tongue and bilateral facial muscle weakness, and was admitted to our hospital. On admission, neurological examination revealed moderate bilateral facial muscle weakness, mild paresthesia in the tongue and four extremities, and decreased Achilles tendon reflex bilaterally. Mild pleocytosis and increased protein were found in the cerebrospinal fluid (CSF). IgM antibodies that reacted with the antigens of Borrelia garinii and Borrelia afzelii were found in his serum. Clinically and serologically, he was thus diagnosed as having neuroborreliosis. Brain MRI revealed gadolinium-enhanced lesions of the bilateral facial nerves in the facial nerve canal portion. After three weeks of treatment with 100 mg/day doxycycline and 2 g/day ceftriaxone sodium, his symptoms and CSF abnormalities were rapidly improved. Although facial nerve paralysis is a major symptom of neuroborreliosis, the present report is the first to detect the inflammatory lesions of the facial nerves in the facial nerve canal portion by MRI.     

Cerebellar atrophy in epileptic patients

High-resolution CT scans of the brain and posterior fossa were performed on 106 phenytoin (PHT)-treated epileptics, 28 de novo epileptics and 43 control subjects. A higher incidence of cerebellar and brainstem (CBS) atrophy was observed in chronic PHT- or PHT+ phenobarbital-treated epileptics compared to the two other groups. Some control subjects and de novo epileptics presented mild CBS atrophy, whereas moderate to severe atrophy was noted exclusively in chronically-treated patients. In attempting to delineate the etiology of CBS atrophy, epileptic patients were divided in three groups: 55 subjects with normal CT scans, 30 with both cerebral and CBS atrophy, and 49 with pure CBS atrophy. Their ages, length of illness, number of generalized seizures, number of other seizures, and amount of PHT received during their lifetime were assessed. Statistical analysis revealed that posterior fossa atrophy in epileptics was significantly correlated with both the length of the illness and the amount of PHT ingested during the patient's lifetime. The number of seizures appears to not be related to CBS atrophy.     

Neuroradiological findings in classical late infantile neuronal ceroid-lipofuscinosis

We describe a girl aged 5 years, 6 months who began to have seizures at the age of 3 years, 9 months. A cranial CT scan revealed mild, generalized cerebral atrophy. During the next year, she gradually developed ataxia, myoclonic jerks, and bilateral optic nerve atrophy and lost motor skills. A second CT scan performed 12 months after the onset of first symptoms revealed marked progression of cerebral atrophy, especially in the infratentorial area. MRI demonstrated bilateral, periventricular hyperintensities in the T₂-weighted images but no changes in the basal ganglia. Electron microscopic investigations of skin biopsies demonstrated curvilinear bodies, confirming the suspected diagnosis of late infantile neuronal ceroid-lipofuscinosis (LINCL). Predominance of cerebral atrophy in the infratentorial area is typical of LINCL. Periventricular white matter lesions may be evident on MRI scans of patients with classical and LINCL-variant disease. In contrast to neuroradiological findings in patients with LINCL-variant disease, findings in patients with classical LINCL revealed no changes in the basal ganglia.     

Putaminal signal hypointensity on T2 weighted MR image in a patient with corticobasal degeneration]

In the course of a 74-year-old woman with corticobasal degeneration (CBD), bilateral putaminal signal hypointensity appeared on T2 weighted MR image. This right-handed woman noticed progressive difficulty in writing and using chopsticks during two years. Neurological examination in February 1998 showed apraxia, rigidity, akinesia and myoclonus, which were more marked in the right hand side. MRI revealed atrophy of the left frontotemporal lobes. These findings were compatible to CBD. Her symptoms progressed slowly. On re-admission due to aspiration pneumonia in December 1998, she showed difficulty in swallowing and walking in addition to the signs and symptoms described above. MRI revealed bilateral putaminal signal hypointensity on T2 weighted image. In the literature, totally 9 cases in 2 reports showed similar putaminal signal hypointensity. Although this finding is unusual in CBD, advanced degenerative process in the putamen might have induced this change due possibly to the accumulation of iron secondary to the nerve cell loss.     

Transcranial approach for spontaneous CSF rhinorrhea due to Sternberg's canal intrasphenoidal meningoencephalocele: case report and review of the literature

One type of congenital intrasphenoidal meningoencephalocele is remnant of lateral craniopharyngeal (Sternberg's) canal. We present a case of a 23-year girl with 10-month history of right side CSF rhinorrhea. CT scan, MRI revealed congenital meningoencephalocele and CSF leak from middle fossa to right side of sphenoid sinus, and there were bony defects at the floor of the anterior aspect of the right middle fossa. Transcranial repair was performed with right side pterional craniotomy. Careful preoperative evaluation and localization of the sphenoid defect are essential for selection of the best possible surgical approach and skull base reconstruction for repair of sphenoid sinus CSF leaks and meningoencephaloceles. In this case, an endoscopic technique was not successful so, transcranial repair was performed with right side pterional craniotomy.