Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

At the light of authors' present experience, radicletomy appears as an excellent antalgic operative procedure in the case of roots with high functional risk (brachial plexus and lumbar plexus). In the absence of any motor deficiency or ataxia, it appears that radicletomy is of help in the cure of severe hypertonies of the extremities (sequelae of cerebral stem contusions). Conversely, in the spastic sequelae of hemi- or paraparesias, lumbar-sacral posterior selective radicotomy is a sure procedure that procures results nearly super-imposable to radicletomy with an appreciable gain in time. At last, for what concerns the motor involvements of the upper extremity ending in spasticity, selective radicletomy recovers its rights and has to be preferred to S.P.R. The indications may be summarized as follows: -- At the level of the lower extremities: in the case of paraparetic sequelae or of sequelae due to spastic paraplegia, a S.P.R. has to be performed; for what concerns antalgic surgery, in the absence of motor deficiency, the best indication is radicletomy. -- At the level of the upper extremities: in the case of dystonic sequeale of the cerebral stem, spastic pain bound with hemiplegia or with carcinoma etc. (herpes zoster..), radicletomy constitutes the ideal surgical procedure.
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PMID:[Results of selective posterior radiculetomy at the lumbar and cervical level]. 5 51

A 46-year-old healthy man suffered from sore throat, fever and right otalgia. On the next day, he developed hoarseness and difficulty in swallowing. On the 6th day, he suffered from vertigo, nausea and vomiting associated with unsteady gait. He was admitted to the otorhinolaryngology department in our hospital and pointed out to have vesicles at his right ear. On the 13th day, he was referred to our service. On admission, no vesicles were noted at the right ear or pharynx. Neurological examination revealed mild nuchal rigidity and marked hoarseness, associated with poor elevation of soft palate and loss of pharyngeal reflex on the right side. He also had horizontal-clockwise rotatory nystagmus in primary gaze and ataxic gait. There was no hearing loss nor facial palsy. No other abnormal neurological findings were noted. The cerebrospinal fluid showed pleocytosis associated with increased protein. The viral antibody titre for herpes zoster was significantly elevated on 18th day in serum as well as in cerebrospinal fluid. Vertigo, nausea, vomiting, ataxia and difficulty in swallowing were all disappeared by the 25th day, whereas hoarseness was improved but still noted 6 months later. Among cranial nerves, trigeminal and facial nerves are the most commonly affected in patients with herpes zoster, but there have been a few reported cases of the 9th and 10th cranial nerve involvement in the literature. In these previously reported cases, all were written before the era of serological diagnosis, and herpes zoster was diagnosed by the vesicles at the ear or pharynx.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of unilateral VIIIth, IXth and Xth cranial nerve involvement with herpes zoster]. 216 88

The clinical course of herpes zoster associated encephalitis (HZAE) with special emphasis on the treatment with acyclovir is described from the experience in 14 own patients and 47 review cases. Immunosuppression and dissemination involved increased risk of HZAE, whereas cranial zoster implied no or only a slightly increased risk. The symptoms were mainly disturbances of mental function and ataxia. Nuchal rigidity was noted in approximately one third of cases. The median duration from dermatomal lesion to HZAE was 15 days in immunosuppressed patients versus 5 days in non-immunosuppressed patients. Abnormal spinal fluid findings included mononuclear pleocytosis, occasionally with low glucose concentration. Protein was elevated in half of the patients. Serum sodium levels were often low. Brain CAT scans were generally normal and EEGs always abnormal. Recurrence of HZAE was noted in 2 patients. Treatment with acyclovir seemed to have a beneficial effect. The results, however, need cautious interpretation due to the heterogenous patient material. Two patients developed signs of HZAE while on treatment with desciclovir but recovered during ongoing therapy.
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PMID:Herpes zoster associated encephalitis: clinical findings and acyclovir treatment. 322 75

A 59-y-old with a history of chronic renal failure on hemodialysis was diagnosed with herpes zoster and begun on 800 mg acyclovir 5 times daily. Two days later the patient developed visual hallucinations, ataxia, confusion and memory loss along with focal myoclonus, nausea and vomiting. No fever, elevated WBC count or significant electrolyte imbalance was found. CT scan of the brain was unremarkable. The patient was then dialyzed for presumed acyclovir toxicity. Her acyclovir level was later found to have been 3.4 micrograms/ml (normal peak range 0.4-2 micrograms/ml) prior to dialysis. After 3 h of hemodialysis, her post-dialysis acyclovir level was 1.9 micrograms/ml. After a second course of hemodialysis the next day the patient's mental status improved, and she was discharged 5 d later. Due to its low volume of distribution (0.6 L/kg), low protein binding (about 15%) and water solubility, acyclovir is an example of the ideal drug that can be removed by hemodialysis. About 45% of the total body amount can be extracted through a 3-h course of hemodialysis with resultant improvement in symptoms.
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PMID:Hemodialysis removal of acyclovir. 757 52

Focal lesions of central nervous system are extremely rare following cutaneous herpes zoster. A 55 year old male developed cerebellar speech, right sided ataxia and intention tremor, three weeks after herpes zoster of right spinal C4 segment. Clinical examination and investigations confirmed a focal vascular lesion in the midbrain suggestive of granulomatous angiitis which can cause focal neurological defect after herpes zoster.
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PMID:Unilateral ataxia following herpes zoster of spinal C4 segment. 822 7

Deep brain stimulation of the thalamus (thalamic DBS) is an established therapy for medically intractable essential tremor and tremor caused by multiple sclerosis. In both disorders, motor disability results from complex interaction between kinetic tremor and accompanying ataxia with voluntary movements. In clinical studies, the efficacy of thalamic DBS has been thoroughly assessed. However, the optimal anatomical target structure for neurostimulation is still debated and has never been analysed in conjunction with objective measurements of the different aspects of motor impairment. In 10 essential tremor and 11 multiple sclerosis patients, we analysed the effect of thalamic DBS through each contact of the quadripolar electrode on the contralateral tremor rating scale, accelerometry and kinematic measures of reach-to-grasp-movements. These measures were correlated with the anatomical position of the stimulating electrode in stereotactic space and in relation to nuclear boundaries derived from intraoperative microrecording. We found a significant impact of the stereotactic z-coordinate of stimulation contacts on the TRS, accelerometry total power and spatial deviation in the deceleration and target period of reach-to-grasp-movements. Most effective contacts clustered within the subthalamic area (STA) covering the posterior Zona incerta and prelemniscal radiation. Stimulation within this region led to a mean reduction of the lateralized tremor rating scale by 15.8 points which was significantly superior to stimulation within the thalamus (P < 0.05, student's t-test). STA stimulation resulted in reduction of the accelerometry total power by 99%, whereas stimulation at the ventral thalamic border (68%) or within the thalamus proper (2.5%) was significantly less effective (P < 0.01). Concomitantly, STA stimulation led to a significantly higher increase of tremor frequency and decrease in EMG synchronization compared to stimulation within the thalamus proper (P < 0.001). In reach-to-grasp movements, STA stimulation reduced the spatial variability of the movement path in the deceleration period by 28.9% and in the target period by 58.4%, whereas stimulation within the thalamus was again significantly less effective (P < 0.05), with a reduction in the deceleration period between 6.5 and 21.8% and in the target period between 1.2 and 11.3%. An analysis of the nuclear boundaries from intraoperative microrecording confirmed the anatomical impression that most effective electrodes were located within the STA. Our data demonstrate a profound effect of deep brain stimulation of the thalamic region on tremor and ataxia in essential tremor and tremor caused by multiple sclerosis. The better efficacy of stimulation within the STA compared to thalamus proper favours the concept of a modulation of cerebello-thalamic projections underlying the improvement of these symptoms.
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PMID:Kinematic analysis of thalamic versus subthalamic neurostimulation in postural and intention tremor. 1743 79

Cerebellitis associated with herpes zoster has rarely been observed. We report here a 76-year-old man who had a history of anterior resection for sigmoid colon cancer and presented during chemotherapy with vesicular rash of the left ear, neuralgic pain in the postauricular area, and ataxic gait. After a while, he developed left peripheral facial palsy, fever, aggravated gait ataxia, and prolonged nausea and vomiting. The left facial nerve was enhanced on gadolinium-enhanced brain magnetic resonance imaging. We suspected that the patient had Ramsay Hunt syndrome accompanied by cerebellitis, which has not been reported previously. Over the course of several months, during which he was treated with acyclovir and corticosteroid, his symptoms improved significantly.
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PMID:A case of ramsay hunt syndrome complicated by cerebellitis. 2039 7

We report on a white Afrikaans family from eastern South Africa with three members affected with North Sea progressive myoclonus epilepsy, resulting from a homozygous founder GOSR2 mutation (c.430G>T, p.Gly144Trp). The mutation was identified by exomic sequencing in a research study investigating childhood onset ataxias. All three subjects presented with ataxia, tremor, early gait difficulties, and myoclonic and generalized tonic clonic (GTC) epilepsy. Each patient underwent deep brain stimulation of the caudal Zona Incerta before coming to the attention of the authors. In each case there was a reduction in GTC seizures, and two patients exhibited a reduction in involuntary movements, as evaluated during long-term follow-up. In one case there was an improvement in gait and stance when assessed while the stimulation was on.
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PMID:Deep Brain Stimulation in Three Related Cases of North Sea Progressive Myoclonic Epilepsy from South Africa. 3083 61

Acute cerebellitis is a well recognized complication of varicella zoster virus (VZV) infection in children. It has been described in adults in the setting of virus reactivation with a preceding herpes zoster rash, but it is exceedingly rare in adults who are not elderly or immunocompromised, particularly in the absence of a rash. To our knowledge, there has been only one reported case of acute cerebellitis in an immunocompetent adult less than age 65 with virological confirmation of acute VZV infection. We describe a 59-year-old immunocompetent man who presented with acute truncal ataxia without rash and was diagnosed with VZV cerebellitis, supported by anti-VZV IgM and anti-VZV IgG antibodies in the serum and a positive VZV polymerase chain reaction in cerebrospinal fluid. He had robust improvement with intravenous acyclovir treatment and was free of neurologic disability at two month follow-up. This case highlights the importance of virological evaluation in patients with acute ataxia, even in the absence of typical features of infection.
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PMID:Acute truncal ataxia in a healthy adult with varicella zoster virus cerebellitis: A case report and literature review. 3098 Nov 22

We present a case of a 62-year-old Caucasian male with laryngeal cancer and Ramsay Hunt Syndrome otherwise known as herpes zoster oticus due to reactivation of the varicella zoster virus. Classic findings include the triad of ipsilateral facial paralysis, otic pain, and herpetic lesions in the sensory supply of the facial nerve. The common pathogenesis is associated with anterograde axonal reactivation of the varicella zoster virus in the geniculate ganglion. Unique features of our case include retrograde transaxonal spread of the varicella-zoster virus from the geniculate ganglion into the brainstem and cerebellum including involvement of the abducens nucleus, facial nucleus, middle cerebral peduncle, and inferior cerebellar peduncle. This presented as left facial paralysis, left sixth nerve palsy, horizontal diplopia to the left lateral gaze, profound truncal ataxia, and left-sided dysmetria. Clinical awareness that Ramsay Hunt syndrome may also involve the brainstem and cerebellum is critical in evaluating the clinical neurologic findings and expanding the diagnostic workup to include brain magnetic resonance imaging and cerebrospinal fluid analysis, including varicella zoster polymerase chain reaction. Encephalitis requires longer duration administration of high-dose intravenous acyclovir in conjunction with steroids. Delays in treatment are often associated with unsatisfactory outcomes with extensive residual deficits.
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PMID:Brainstem and Cerebellar Involvement in Ramsay Hunt Syndrome. 3191 61


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