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Clinical consequences of hypomagnesaemia are manifold and include various neurological syndromes up to life-threatening conditions. Despite its importance, magnesium is generally not routinely determined leading to an under recognition of hypomagnesaemia related disorders. In the past years, there are growing numbers of reports of hypomagnesaemia induced cerebellar syndromes (HiCS) with corresponding cerebellar oedema, which might be a distinct disease entity. To provide further insights into HiCS, we describe a patient with HiCS and performed a literature review on cerebellar syndromes due to severe hypomagnesaemia with regard to the clinical, magnetic resonance imaging (MRI) and laboratory findings. We identified 17 cases, so including our case, 18 cases contribute to this review. Summarized, HiCS seems to be a distinct disease entity due to the remarkable similarities of clinical, MRI and laboratory features. It should be diagnosed and treated early to avoid recurrent disease courses, residual symptoms and potentially life-threatening conditions such as seizures. Physicians must be alert to HiCS as magnesium is usually not part of the routine electrolyte panel.

Background Abnormal restricted diffusion on magnetic resonance imaging is often associated with ischemic stroke or anoxic injury, but other conditions can present similarly. We present six cases of an unusual but consistent pattern of restricted diffusion in bilateral hippocampi and cerebellar cortices. This pattern of injury is distinct from typical imaging findings in ischemic, anoxic, or toxic injury, suggesting it may represent an under-recognized clinicoradiographic syndrome. Despite initial presentation with stupor or coma in the context of obstructive hydrocephalus, patients may have acceptable outcomes if offered early intervention. Methods We identified an ad hoc series of patients at our two institutions between years 2014 and 2017 who presented to the neurocritical care unit with severe, otherwise unexplained cerebellar edema and retrospectively identified several commonalities in history, presentation, and imaging. Results Between two institutions, we identified six patients—ages 33–59 years, four male—with similar presentations of decreased level of consciousness in the context of intoxicant exposure, with acute cytotoxic edema of the cerebellar cortex, hippocampi, and aspects of the basal nuclei. All patients presented with severe cerebellar edema which led to obstructive hydrocephalus requiring aggressive medical and/or surgical management. The five patients who survived to discharge demonstrated variable degrees of physical and memory impairment on discharge and at follow-up. Conclusions We present findings of a potentially novel syndrome involving a distinct pattern of cerebellar and hippocampal restricted diffusion, with imaging and clinical characteristics distinct from ischemic stroke, hypoxic injury, and known toxidromes and leukoencephalopathies. Given the potential for favorable outcome despite early obstructive hydrocephalus, early identification and treatment of this syndrome are critical.

Young-onset cerebellar syndromes are quite interesting and challenging for treating clinicians. While dealing with such cases, a clinician should be aware of rare possible causes too. We report a rare case of Gordon Holmes syndrome—an autosomal recessive cerebellar ataxia with endocrinal abnormalities.

The cerebellum is central to normal motor function and co-ordination, and can be frequently affected in a number of common disease processes. However, medical student teaching relating to cerebellar anatomy and pathology is lacking, leaving many graduates with a significant knowledge gap. Junior doctors need to be able to recognize ‘cerebellar syndromes’ on presentation to hospitals, and to identify and manage reversible causes rapidly and effectively. After review of relevant literature, a simple approach to the functional anatomy and practical classifications of common cerebellar pathology is presented here, with a focus on symptoms, signs and examination techniques essential to medical school final exams.

Motor planning is not a monolithic process, and distinct stages of motor planning are responsible for encoding different levels of abstractness. However, how these distinct components are mapped into different neural substrates remains an open question. We studied one of these high-level motor planning components, defined as second-order motor planning, in a patient (R.G.) with an extremely rare case of cerebellar agenesis but without any other cortical malformations. Second-order motor planning dictates that when two acts must be performed sequentially, planning of the second act can influence execution of the first. We used an optoelectronic system for kinematic analysis to compare R.G.’s performance with age-matched controls in a second-order motor planning task. The first act was to reach for an object, and the second was to place it into a small or large container. Our results showed that despite the expected difficulties in fine-motor skills, second-order motor planning (i.e., the ability to modulate the first act as a function of the nature of the second act) was preserved even in the patient with congenital absence of the cerebellum. These results open new intriguing speculations about the role of the cerebellum in motor planning abilities. Although prudence is imperative when suggesting conclusions made on the basis of single-case findings, this evidence suggests fascinating hypotheses about the neural circuits that support distinct stages of the motor planning hierarchy, and regarding the functional role of second-order motor planning in motor cognition and its potential dysfunction in autism. NEW & NOTEWORTHY Traditionally, the cerebellum was considered essential for motor planning. By studying an extremely rare patient with cerebellar agenesis and a group of neurotypical controls, we found that high stages of the motor planning hierarchy can be preserved even in this patient with congenital absence of the cerebellum. Our results provide interesting insights that shed light on the neural circuits supporting distinct levels of motor planning. Furthermore, the results are intriguing because of their potential clinical implications in autism.

The prevalence of paraneoplastic cerebellar degeneration (PCD) associated with gynecological cancer is rare. Here, we reported the first case of ovarian cancer revealed by PCD in our institute. we describe a 80- year –old Moroccan female presented with subacute vestibular and cerebellar syndromes, she had an inguinal lymphadenopathy,with high levels of Anti-YO. Rapid progression and absence of known etiologies point towards a probable paraneoplastic origin of the syndrome in this patient. The exact incidence of PNS among those diagnosed with cancer remains uncertain, it is important to report this cases in the literature to help early diagnosis and appropriate treatment, which are able to stabilize the neurological symptoms.Key words: Paraneoplastic cerebellar degeneration, Paraneoplastic syndromes, ovarian cancer

Esclerose múltipla é uma doença crônica, inflamatória e desmielinizante do sistema nervoso central. O tratamento preconizado para a forma surto-remissão é com imunomoduladores, fornecidos em Belo Horizonte pela Secretaria de Estado da Saúde de Minas Gerais (SES/MG). Analisamos os dados de 283 pacientes inscritos no Programa de Medicamentos Excepcionais (imunomoduladores) da SES/MG no período de maio a outubro de 2004. Houve predomínio do sexo feminino (3,7:1) e a idade média dos pacientes foi 38,4±10,7 anos, com as primeiras manifestações clínicas ocorridas aos 29,7±10,2 anos. Os sintomas iniciais foram: sensitivos (46,4%), visuais (33,1%), síndrome de tronco cerebral/cerebelo (30,1%) e motores (25,9%). Os sintomas observados em surtos subseqüentes foram motores (22,3%), síndromes de tronco cerebral/cerebelo (18,9%), visuais (18,9%) e sensitivos (9,6%). Em 234 RM de encéfalo e 109 RM da medula espinhal existiam lesões desmielinizantes em 97,4% e 85,3% respectivamente. Comparamos nossos achados com outras séries descritas na literatura brasileira.

Summary Among the hereditary ataxias, autosomal recessive spinocerebellar ataxias comprise a diverse group of neurodegenerative disorders. Clinical phenotypes vary from predominantly cerebellar syndromes to sensorimotor neuropathy, ophthalmological disturbances, involuntary movements, seizures, cognitive dysfunction, skeletal anomalies, and cutaneous disorders, among others. Molecular pathogenesis also ranges from disorders of mitochondrial or cellular metabolism to impairments of DNA repair or RNA processing functions. Diagnosis can be improved by a systematic approach to the categorisation of these disorders, which is used to direct further, more specific, biochemical and genetic investigations. In this Review, we discuss the clinical characteristics and molecular genetics of the more common autosomal recessive ataxias and provide a framework for assessment and differential diagnosis of patients with these disorders.

Left or right unilateral removal of a cerebellar hemisphere altered visuo-motor coordination as well as spatial representation of the environment. Moreover, the operated rats were unable to adapt their behavior to a novel situation, suggesting frontal dysfunctions similar to those demonstrated in human cerebellar syndromes.

Sri Lanka is hyperendemic for dengue viral infection. Dengue has a wide spectrum of neurological manifestations including previously reported Sri Lankan cases with a 6th nerve palsy and a cerebellar syndrome from a co-infection with dengue and Epstein-Barr virus. This series describes a spontaneously resolving cerebellar syndrome following a dengue viral infection. Dengue is potentially an important cause of cerebellar syndromes in countries hyperendemic for the disease; patients need further studies to identify the responsible serotypes.

Cerebellar syndromes are one of the commonest neurological diseases. Objectives : To study the patterns of clinical presentations of cerebellar syndromes and to identify the possible causes. Methods : This is a prospective hospital based, cross-sectional study. One hundred adult Sudanese patients with cerebellar syndromes were included in the study during the period from January 2006– January 2007. Results : The most common age group affected was 18 – 25 years. Male to female ratio was 1.5: 1 unsteadiness on walking was the most common symptom (83%). Gait-ataxia was the most common sign (83%). Cerebrovascular disease was the most common aetiology (25%). Conclusion : Cerebellar syndromes are not rare in Sudan. However, they were diagnosed more commonly at the central regions of the country probably because of more awareness of patients and better facilities for diagnosis. The age of onset, the male predominance, the presentation and clinical findings were not different from reported literature. This also goes for the common causes apart from alcohol which is a strikingly rare as a cause in this study and could be accounted for the implementation of Elshariya (Islamic laws) Laws in Sudan. Keywords : ataxia, dysmetria, disdiadochokenesis, decomposition, nystagmus, dysarthria.

The effect of temporarily suppressing the visual display of either the target (desired) trajectory or the actually movement trajectory upon the accuracy of visuo-motor tracking was studied in 6 patients with cerebellar syndromes and 6 healthy subjects. Subjects made extension and flexion movements of the wrist to superimpose a cursor displaying their movement (movement cursor) upon one indicating target (target cursor) on a VDU screen. The target trajectory consisted of a sawtooth pattern of slow (4 deg/sec) ramp extension and instantaneous flexion return phases. Following practice, the tracking of cerebellar patients was significantly less accurate than that of healthy subjects for each phase (P = 0.02). Temporary suppression of the movement cursor during both the mid-section of the ramp phase (P = 0.05) and around the reversal phase (P = 0.04) caused a significant increase in tracking errors in the patients whereas suppression of the target cursor did not alter their performance. Suppression of neither cursor altered the tracking accuracy of healthy subjects during the ramp extensions whilst suppression of either caused reduced (P = 0.02) performance for the reversal phase. We interpret the increased dependence of patients upon visual information of their movements during slow trajectories as indicating an impairment of proprioceptive guidance.

In Reply. —I thank Botez for his comments. In attempting to establish a causal relationship between lesions of the cerebellum and disturbances of higher function, it is important to know the nature and extent of the cerebellar lesion and also the integrity of the cerebral hemispheres in any given individual. This is particularly true in patients with degenerative cerebellar syndromes, because their cerebral hemispheres are frequently abnormal when studied post mortem. Similarly, it would seem that the study of intoxications of the nervous system is suboptimal because other regions of the cerebral hemispheres are likely to be affected as well. The neuropsychologic performance of patients with chronic unilateral cerebellar damage appears to be unimpaired, according to a preliminary report by Botez and Botez.1It would be helpful to know the lesion site in these patients, because behavioral manifestations in animals seem to vary depending on the part of the

A system is described which, by means of a digital computer, enables the collection of cerebellar symptoms gained by conventional examination, and computes, in a completely automatic way, all present cerebellar syndromes.

The presenting symptoms of neuroblastoma in children are variable. Cerebellar ataxia, opsoclonus and myoclonus can be the only signs of an occult neuroblastoma. Extirpation of the primary lesion should prevent further complications, but may not necessarily reverse the cerebellar symptomatology.

In 1917 Gordon Holmes1published his classic dissertation on cerebellar symptomatology. Despite this work, the localization of cerebellar lesions has continued to be difficult. In recent years greater understanding of the cerebellum has become available through studies of cerebellar morphology and physiology.2These advances have made it possible to recognize localizing syndromes which are of pure cerebellar origin and additional symptom complexes which result from combined involvement of the cerebellum and adjacent structures.3As a preliminary step to the understanding of these syndromes, it is important to review briefly the recent advances in the clinical aspects of cerebellar anatomy. No attempt will be made to go into the finer details of cerebellar anatomy, physiology, histology and comparative morphology. Generalizations made for clinical purposes leave inaccuracies as to finer details but are valuable when dealing with patients. CEREBELLAR ANATOMY The Purkinje cells of the cerebellum are arranged in