Frontal Ataxia in Childhood

Abstract

Frontal ataxia may be the result of a unilateral frontal lesion. In this report three cases are presented with ataxia due to right frontal lesions. One case concerns a boy presenting with an unsteady gait and titubation of the trunk, mimicking developmental disequilibrium and with complex partial seizures. It proved to be caused by a small right-sided cavernoma in the middle frontal gyrus. After surgical intervention the symptoms and the seizures disappeared. Two subsequent cases concern teenage patients presenting with headache after an ENT infection and on physical examination mild dysmetric function of the upper limbs and slight disequilibrium, due to right-sided frontal lobe abscesses. After neurosurgical and antibiotic therapy the symptoms were relieved. The frontal origin of ataxia should be considered in children presenting with a “cerebellar syndrome”. Frontal gait disorders consist of a clinical pattern of different gait disorders. The syndrome has been mentioned in the literature under different names. Our patients show signs compatible with the term frontal disequilibrium, a clinical pattern of frontal gait disorder. This assumes walking problems characterized by loss of control of motor planning, leading to imbalance. Remarkably, frontal ataxia may mimic developmental delay as demonstrated in the first case and may be the leading mild symptom in extensive frontal lobe damage as demonstrated by the two other cases. We suppose that frontal ataxia is the result of a disturbance in the cerebellar-frontal circuitries and an impairment of executive and planning functions of the basal ganglia-frontal lobe circuitry.

Key words

Childhood - frontal lobe - ataxia

References

• 1 Bennett H S, Selman J E, Rapin I, Rose A. Nonconvulsive epileptiform activity appearing as ataxia.  Am J Dis Child. 1982;  136 30-32
• 2 Della Sala S, Francescani A, Spinnler H. Gait apraxia after bilateral supplementary motor area lesion.  J Neurol Neurosurg Psychiatry. 2002;  72 77-85
• 3 Gentilucci M, Bertolani L, Benuzzi F, Negrotti A, Pavesi G, Gangitano M. Impaired control of action after supplementary motor area lesion: a case study.  Neuropsychologia. 2000;  38 1398-1404
• 4 Herrero M T, Barcia C, Navarro J. Functional anatomy of thalamus and basal ganglia.  Child's Nervous System. 2002;  18 386-404
• 5 Leiner H C, Leiner A L, Dow R S. The human cerebro-cerebellar system: its computing, cognitive, and language skills.  Behavioural Brain Research. 1991;  44 113-128
• 6 Nutt J G, Marsden C D, Thompson P D. Human walking and higher-level gait disorders, particularly in the elderly.  Neurology. 1993;  43 268-279
• 7 Rubio F A. Gait disorders.  Neurologist. 2002;  8 254-262
• 8 Stolze H, Kuhtz-Buschbeck J P, Drücke H, Jöhnk K, Deuschl G. Comparative analysis of gait disorder of normal pressure hydrocephalus and Parkinson's disease.  J Neurol Neurosurg Psychiatry. 2001;  70 289-297
• 9 Sibon I, Fenelon G, Quinn N P, Tison F. Vascular parkinsonism.  J Neurol. 2004;  251 513-524
• 10 Sunohara N, Mukoyama M, Mano Y, Toyoshima E, Satayoshi E. [Frontal pseudoataxia, discussion on its mechanism] (in Japanese).  Rinsho Shinkeigaku. 1981;  21 671-681
• 11 Takakusaki K, Habaguchi T, Ohtinata-Sugimoto J, Saitoh K, Sakamoto T. Basal ganglia efferents to the brainstem centers controlling postural muscle tone and locomotion: a new concept for understanding motor disorders in basal ganglia dysfunction.  Neuroscience. 2003;  119 293-308
• 12 Vergani M I, Reimao R, Silva A M, Muskat M, Esposito S, Diament A. Multiple sclerosis with early childhood onset. A case report.  Arq Neuropsiquiatri. 1988;  46 195-197