Acta Neurol Belg DOI 10.1007/s13760-015-0469-1
NEURO-IMAGES
Tethered spinal cord: a rare cause of foot deformities Evangelia Gavanozi1 • Tavitha Vlachou1 • Petros Zampakis2 • Elisabeth Chroni1
Received: 6 March 2015 / Accepted: 25 March 2015 Ó Belgian Neurological Society 2015
A 22-year-old man was presented with gait instability developed over the last 6 months, accompanying by painful distal dysesthesias in the legs and neurogenic bladder. He reported a 10-year history of progressive intrinsic foot muscle weakness and atrophy bilaterally, which was arbitrary attributed to Charcot–Marie–Tooth hereditary neuropathy, despite the absence of similar deformities in close family members, because this type of neuropathy is the commonest cause of such foot morphology in the region of the patient’s geographic origin. Neurologic examination showed normal motor and sensory function in the upper
limbs, asymmetric, more on the right, distal leg muscle weakness (MRC score right gastrocnemius 1/5, left gastrocnemius 2/5; right and left tibialis anterior 4/5; right abductor hallucis 0/5, left abductor hallucis 2/5) (Figs. 1, 2), suppressed tendon reflexes in the legs and hypoesthesia in the anterior and lateral aspects of right leg. The patient needed 4–5 intermittent self-catheterizations per day.
Fig. 1 The right foot has overly high arch and claw toes leading to the erroneous diagnosis of Charcot–Marie–Tooth hereditary neuropathy & Elisabeth Chroni [email protected] 1
Department of Neurology, School of Medicine, University Hospital of Patras, University of Patras, 26504 Rio Patras, Greece
2
Department of Radiology, School of Medicine, University of Patras, Rio Patras, Greece
Fig. 2 Atrophy of the posterior leg muscles and the presence of hindfoot varus (heels point to the midline)
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Acta Neurol Belg
Initially, neuropathy was excluded by the normal motor and sensory nerve conduction findings except for the absent response from right abductor hallucis muscle. Consecutively, the existence of several cafe´-au-lait spots on the patient’s back, lower abdomen and thighs in association with family history of neurofibromatosis type I raised the suspicion of neurogenic tumor originated from the lumbosacral roots. MRI of the lower spine revealed a tethered spinal cord (Fig. 3), setting the correct diagnosis after many years delay from the first appearance of clinical symptoms in childhood. Inspection of lumbosacral area did not reveal midline cutaneous stigmata such as localized
hypertrichosis, dermal sinus or dimple, which would have led to an early investigation for occult spinal dysraphism [1]. Surgical release of spine cord traction by sectioning the tight filum terminale stabilized the patient’s manifestations, including improvement of bladder function. Tethered cord syndrome is a well-described entity manifested during childhood or adolescent with predominately neurological symptoms and signs in the lower limbs [2]. Pain in the back and legs is the commonest sensory symptom, whereas foot muscles atrophy prior to skeletal maturation is also a frequent feature resulting in pes cavus and claw toes, which could be misinterpreted as characteristics of hereditary peripheral neuropathy. In cases such as the presenting one, low-lying of conus medullar without overt congenital stigma could go on unnoticed from childhood to adulthood minimizing the possibility of recovery after surgical intervention [3]. The progressive nature of this condition became apparent in our patient when the clinical picture supplemented with micturition deficit that pointed to a sacral cord lesion. A previous review study showed that surgical untethering offers a good chance to restore bladder function even when performing in adults [4]. It is suggested that tethered cord syndrome should be considered in differential diagnosis of foot deformity in young patients to prevent further neurological deterioration [5]. Conflict of interest cerning this paper.
The authors report no conflict of interest con-
Ethical standard This article does not contain any studies with animals performed by any of the authors. Informed consent Informed consent was obtained by the individual participant included in the study.
References
Fig. 3 Saggital T1 post gadolinium injection MRI scan. White arrow indicates the presence of tethered cord extending up to the level of S2. Black arrow shows a homogeneously enhancing mass within the posterior lower aspect of spinal canal, which has been widened
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1. Frainey BT, Yerkes EB, Menon VS, Gong EM, Meyer TA, Bowman RM, McLone DG, Cheng EY (2014) Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism. J Pediatr Urol 10:627–633 2. Cornips EMJ, Vereijken IMP, Beuls EAM, Weber JW, Soudant DLHM, van Rhijn LW, Callewaert PRH, Vles JSH (2012) Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome. Eur J Paediatr Neurol 16:103–117 3. Gharedaghi M, Samini F, Mashhadinejad H, Khajavi M, Samini M (2014) Orthopedic lesions in the tethered cord syndrome: the importance of early diagnosis and treatment on patient outcome. Arch Bone Jt Surg 2(2):93–97 4. Aufschnaiter K, Fellner F, Wurm G (2008) Surgery in adult onset tethered cord syndrome (ATCS): review of literature on occasion of an exceptional case. Neurosurg Rev 31(4):371–383 5. Garce´s-Ambrossi GL, McGirt ML, Samuels R, Sciubba DM, Bydon A, Gokaslan ZL, Jallo GI (2009) Neurological outcome after surgical management of adult tethered cord syndrome. J Neurosurg Spine 11(3):304–309
NEURO-IMAGES
Tethered spinal cord: a rare cause of foot deformities Evangelia Gavanozi1 • Tavitha Vlachou1 • Petros Zampakis2 • Elisabeth Chroni1
Received: 6 March 2015 / Accepted: 25 March 2015 Ó Belgian Neurological Society 2015
A 22-year-old man was presented with gait instability developed over the last 6 months, accompanying by painful distal dysesthesias in the legs and neurogenic bladder. He reported a 10-year history of progressive intrinsic foot muscle weakness and atrophy bilaterally, which was arbitrary attributed to Charcot–Marie–Tooth hereditary neuropathy, despite the absence of similar deformities in close family members, because this type of neuropathy is the commonest cause of such foot morphology in the region of the patient’s geographic origin. Neurologic examination showed normal motor and sensory function in the upper
limbs, asymmetric, more on the right, distal leg muscle weakness (MRC score right gastrocnemius 1/5, left gastrocnemius 2/5; right and left tibialis anterior 4/5; right abductor hallucis 0/5, left abductor hallucis 2/5) (Figs. 1, 2), suppressed tendon reflexes in the legs and hypoesthesia in the anterior and lateral aspects of right leg. The patient needed 4–5 intermittent self-catheterizations per day.
Fig. 1 The right foot has overly high arch and claw toes leading to the erroneous diagnosis of Charcot–Marie–Tooth hereditary neuropathy & Elisabeth Chroni [email protected] 1
Department of Neurology, School of Medicine, University Hospital of Patras, University of Patras, 26504 Rio Patras, Greece
2
Department of Radiology, School of Medicine, University of Patras, Rio Patras, Greece
Fig. 2 Atrophy of the posterior leg muscles and the presence of hindfoot varus (heels point to the midline)
123
Acta Neurol Belg
Initially, neuropathy was excluded by the normal motor and sensory nerve conduction findings except for the absent response from right abductor hallucis muscle. Consecutively, the existence of several cafe´-au-lait spots on the patient’s back, lower abdomen and thighs in association with family history of neurofibromatosis type I raised the suspicion of neurogenic tumor originated from the lumbosacral roots. MRI of the lower spine revealed a tethered spinal cord (Fig. 3), setting the correct diagnosis after many years delay from the first appearance of clinical symptoms in childhood. Inspection of lumbosacral area did not reveal midline cutaneous stigmata such as localized
hypertrichosis, dermal sinus or dimple, which would have led to an early investigation for occult spinal dysraphism [1]. Surgical release of spine cord traction by sectioning the tight filum terminale stabilized the patient’s manifestations, including improvement of bladder function. Tethered cord syndrome is a well-described entity manifested during childhood or adolescent with predominately neurological symptoms and signs in the lower limbs [2]. Pain in the back and legs is the commonest sensory symptom, whereas foot muscles atrophy prior to skeletal maturation is also a frequent feature resulting in pes cavus and claw toes, which could be misinterpreted as characteristics of hereditary peripheral neuropathy. In cases such as the presenting one, low-lying of conus medullar without overt congenital stigma could go on unnoticed from childhood to adulthood minimizing the possibility of recovery after surgical intervention [3]. The progressive nature of this condition became apparent in our patient when the clinical picture supplemented with micturition deficit that pointed to a sacral cord lesion. A previous review study showed that surgical untethering offers a good chance to restore bladder function even when performing in adults [4]. It is suggested that tethered cord syndrome should be considered in differential diagnosis of foot deformity in young patients to prevent further neurological deterioration [5]. Conflict of interest cerning this paper.
The authors report no conflict of interest con-
Ethical standard This article does not contain any studies with animals performed by any of the authors. Informed consent Informed consent was obtained by the individual participant included in the study.
References
Fig. 3 Saggital T1 post gadolinium injection MRI scan. White arrow indicates the presence of tethered cord extending up to the level of S2. Black arrow shows a homogeneously enhancing mass within the posterior lower aspect of spinal canal, which has been widened
123
1. Frainey BT, Yerkes EB, Menon VS, Gong EM, Meyer TA, Bowman RM, McLone DG, Cheng EY (2014) Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism. J Pediatr Urol 10:627–633 2. Cornips EMJ, Vereijken IMP, Beuls EAM, Weber JW, Soudant DLHM, van Rhijn LW, Callewaert PRH, Vles JSH (2012) Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome. Eur J Paediatr Neurol 16:103–117 3. Gharedaghi M, Samini F, Mashhadinejad H, Khajavi M, Samini M (2014) Orthopedic lesions in the tethered cord syndrome: the importance of early diagnosis and treatment on patient outcome. Arch Bone Jt Surg 2(2):93–97 4. Aufschnaiter K, Fellner F, Wurm G (2008) Surgery in adult onset tethered cord syndrome (ATCS): review of literature on occasion of an exceptional case. Neurosurg Rev 31(4):371–383 5. Garce´s-Ambrossi GL, McGirt ML, Samuels R, Sciubba DM, Bydon A, Gokaslan ZL, Jallo GI (2009) Neurological outcome after surgical management of adult tethered cord syndrome. J Neurosurg Spine 11(3):304–309
