Progressive Paraparesis Research Articles

NIMG-06. GUILLAIN BARRE MIMICKING LEPTOMENINGEAL SPREAD IN CANCER PATIENTS: REPORT OF TWO CASES AN REVIEW OF LITERATURE

Abstract BACKGROUND Guillain-Barre syndrome (GBS) is an immune acute polyradiculoneuropathy usually presented as an ascending peripheral paresis with absent tendon reflex and albumino-cytologic dissociation on cerebrospinal fluid (CSF) exam.We describe two oncologic patients with GBS who presented with atypical symptoms. RESULTS The first patient was a 35 years old woman with newly diagnosed grade 3 invasive breast ductal carcinoma. During the sixth courses of neo-adjuvant chemotherapy she complained at back pain and within 24 hours developed progressive severe bilateral proximal paraparesis with a D10 sensory level. Spine MRI demonstrated diffuse leptomeningeal enhancement. Since leptomeningeal spread (LMD) is uncommon at this stage of breast cancer CSF was obtained. CSF sample revealed albumin-cytologic dissociation (4 cells, protein level 340 mg/dl). EMG showed acute demyelinating neuropathy consistent with GBS. Intravenous immunoglobulins (IVIG) therapy was initiated with clinical improvement. The second patient was a 45 years old woman with newly diagnosed T-Cell lymphoma. Before initiating chemotherapy she presented with a rapidly progressive tetraparesis, hyperreflexia and cervical sensory level. Spine MRI was intact. CSF showed 22 cells without atypia, FACS negative, protein level was 74 mg/dl. The patient worsened clinically. A second CSF sample was obtained and demonstrated an albumin-cytologic dissociation (8 cells without atypia, protein level 115 mg/dl) raising the possibility of GBS. EMG showed acute axonal polyneuropathy consistent with AMSAN variant of GBS. The patient was treated with IVIG with partial recovery. CONCLUSION Atypical presentation of GBS could be challenging among oncologic patients and could lead to misdiagnosis and delay in treatment.

A complex histopathological challenge: suspicion of an osteoblastoma-like osteosarcoma arising from the second thoracic vertebra in a cat

BackgroundReports of osteoblastic tumours are limited to a few case reports in veterinary medicine. Osteoblastoma-like osteosarcoma has been accepted by the World Health Organization as an intermediate form between an osteosarcoma and osteoblastoma. This type of tumour indicates an osteosarcoma, that may resemble osteoblastoma clinically, histologically, and radiologically and have the capability for metastasis. Osteoblastoma-like osteosarcoma has not been described in veterinary medicine so far.Case presentationAn eight-year old cat was presented due to progressive ataxia and paraparesis of the pelvic limbs. Imaging confirmed a well-defined, extradural mass originating from the spinous process of the second thoracic vertebra (T2) leading to severe compression of the spinal cord. Decompressive cytoreduction was achieved by removal of the mass after dorsal laminectomy of T1. After recovering from an acute worsening 3.5 weeks after surgery, the cat had an improved neurological status and the dorsal compression was resolved at follow-up 8 months later. A focal contrast enhancing lesion was still evident at the base of T2 spinous process and lung metastasis was additionally suspected. Based on histopathological, radiographic, and clinical features, an “osteoblastoma-like osteosarcoma” was suspected.ConclusionsTo the best of our knowledge, this is the first description of this tumour in veterinary medicine. In addition, this case report highlights the difficulty in the diagnosis and definition of osseous neoplasia in cats and provides a literature review.

Childhood Seeligmüller Strümpell Philip Disease: The First Case in Iraq and a Review of the Early Historic Documentation of the Disease in the Literature

Seeligmüller Strümpell Philip disease is characterized by familial progressive spastic paraplegia or paraparesis associated with a progressive deterioration of gait. The disorder is genetically heterogeneous and can be inherited in an autosomal dominant, autosomal recessive or X-linked recessive manner. The mode of inheritance involved has a direct impact on the chances of inheriting the disorder. The cornerstone of treatment is the use of various muscle relaxants. The disorder has not been reported or documented in Iraq. This paper aims to report the first case of childhood Seeligmüller Strümpell Philip disease in an Iraqi girl. Patients and Methods: A thirteen-year girl with childhood Seeligmüller Strümpell Philip disease who developed systemic lupus erythematosus is described and the early historic documentation of Seeligmüller Strümpell Philip disease in the medical literature is briefly updated. Results: The diagnosis of Seeligmüller Strümpell Philip disease in this girl was based on clinical findings and supported by electromyography and nerve conduction studies. Brain MRI showed normal findings. Few weeks before the patient was seen, they took her outside Iraq to Medipol Mega complex of Hospitals for treatment. They performed tenotomy and tenoplasty of the hip adductors, achilloplasty, and posterior capsule relaxation. When the girl was first seen at the Children Teaching Hospital of Baghdad Medical City, the parents regretted taking her outside Iraq for treatment as she didn’t show any improvement, and was still using the wheelchair. Conclusion: Unfortunately, the girl was seen too late, and despite consulting many doctors in Iraq and outside Iraq, she didn’t receive appropriate evidence based treatment and was treated unsatisfactorily surgically.

Defining diagnostic cutoffs in neurological patients for serum very long chain fatty acids (VLCFA) in genetically confirmed X-Adrenoleukodystrophy

X-linked Adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene resulting in the accumulation of very long chain fatty acids (VLCFA). X-ALD is the most common peroxisomal disorder with adult patients (male and female) presenting with progressive spastic paraparesis with bladder disturbance, sensory ataxia with impaired vibration sense, and leg pain. 80% of male X-ALD patients have an adrenal failure, while adrenal dysfunction is rare in women with X-ALD. The objective of this study was to define optimal serum VLCFA cutoff values in patients with X-ALD-like phenotypes for the differentiation of genetically confirmed X-ALD and Non-X-ALD individuals. Three groups were included into this study: a) X-ALD cases with confirmed ABCD1 mutations (n = 34) and two Non-X-ALD cohorts: b) Patients with abnormal serum VCLFA levels despite negative testing for ABCD1 mutations (n = 15) resulting from a total of 1,953 VLCFA tests c) Phenotypically matching patients as Non-X-ALD controls (n = 104). Receiver operating curve analysis was used to optimize VLCFA cutoff values, which differentiate patients with genetically confirmed X-ALD and Non-X-ALD individuals. The serum concentration of C26:0 was superior to C24:0 for the detection of X-ALD. The best differentiation of Non-X-ALD and X-ALD individuals was obtained with a cutoff value of < 1.0 for the C24:0/C22:0 ratio resulting in a sensitivity of 97%, a specificity of 94.1% and a positive predictive value (PPV) of 83.8% for true X-ALD. Our findings further suggested a cutoff of < 0.02 for the ratio C26:0/C22:0 leading to a sensitivity of 90.9%, a specificity of 95.0%, and a PPV of 80.6%. Pearson correlation indicated a significant positive association between total blood cholesterol and VLCFA values. Usage of serum VLCFA are economical and established biomarkers suitable for the guidance of genetic testing matching the X-ALD phenotype. We suggest using our new optimized cutoff values, especially the two ratios (C24:0/C22:0 and C26:0/C22:0), in combination with standard lipid profiles.

Compressive myelopathy presenting with paraparesis in pediatric age

Compressive myelopathy is a common presentation of the adult age group secondary to degenerating etiologies. Trauma is a rare cause of compressive myelopathy, especially in the pediatric age group as the pediatric spine is more elastic than that of adults, especially below 8 years of age. Boys are more commonly injured as compared to girls. The present study reports a case of a six-year-old female patient of compressive myelopathy presenting with jerks with progressive paraparesis. MRI shows posterior subluxation of dens (C2) attached to C2 vertebral effacing anterior – subarachnoid space and indenting spinal cord.

A Case of Foramen Magnum and C1–C2 Meningiomas: Double Challenge, One Approach

Objectives We present a challenging case of a 61-year-old male patient with a double meningioma. The first one was located on the right side of foramen magnum and the second one was located on left anterolateral side of C1–C2. Background Patient referred at our institution for a progressive spastic paraparesis from approximately 1 year with a recent onset of numbness in right hand. Neuroradiological imaging showed an extra-axial enhancing tumor with a dural tail on the right border of foramen magnum and another contralateral extra-axial enhancing lesion at C1–C2 level ( Fig. 1 ). Results We have chosen a park bench position. Neurophysiological monitoring was used during both positioning and surgery. A linear skin incision in the occipital region extended to C7 was performed and a suboccipital craniotomy with the laminectomy of C1–C3 was performed ( Fig. 2 ). Videofluroangiography was performed to the best visualization of the vertebral artery position during microsurgical dissection and exposure of craniovertebral junction region. An Y -shape dural opening was executed and two meningiomas was resected with the classical four D steps (devascularize, detach, debulking, and dissect). All anatomical structures were respected and there were no changes at neuromonitoring. Patient was discharged after 10 days in good neurological condition. Conclusion a single approach for multiple meningiomas of the craniovertebral junction and upper cervical spine region is feasible and effective. Videofluoroangiography as a way to achieve better visualization and control of the vertebral artery, and neurophysiological monitoring are crucial tools to reduce the morbidity of this surgical challenge. The link to the video can be found at: https://youtu.be/4w9HCfQZkgg .

Behr syndrome and hypertrophic cardiomyopathy in a family with a novel UCHL1 deletion

BackgroundBehr syndrome is a clinically distinct, but genetically heterogeneous disorder characterized by optic atrophy, progressive spastic paraparesis, and motor neuropathy often associated with ataxia. The molecular diagnosis is based on gene panel testing or whole-exome/genome sequencing.MethodsHere, we report the clinical presentation of two siblings with a novel genetic form of Behr syndrome. We performed whole-exome sequencing in the two patients and their mother.ResultsBoth patients had a childhood-onset, slowly progressive disease resembling Behr syndrome, starting with visual impairment, followed by progressive spasticity, weakness, and atrophy of the lower legs and ataxia. They also developed scoliosis, leading to respiratory problems. In their late 30’s, both siblings developed a hypertrophic cardiomyopathy and died of sudden cardiac death at age 43 and 40, respectively. Whole-exome sequencing identified the novel homozygous c.627_629del; p.(Gly210del) deletion in UCHL1.ConclusionsThe presentation of our patients raises the possibility that hypertrophic cardiomyopathy may be an additional feature of the clinical syndrome associated with UCHL1 mutations, and highlights the importance of cardiac follow-up and treatment in neurodegenerative disease associated with UCHL1 mutations.

Acute Paraparesis after Epidural Corticosteroid Injection Revealing Spinal Dural Arteriovenous Fistula in a HIV Patient.

Consider an SDAVF in cases of slowly progressive paraparesis and hypoaesthesia, especially if symptoms worsen after an epidural injection.The need for an in-depth work-up including repeated angiographic explorations in cases where no malformation is found in a straightforward manner.In a patient infected with HIV, even though a broad range of neuromuscular diseases can be suspected, non-related aetiologies should not be forgotten.

Mental disorders in neurological diseases. Can symptoms of bipolar disorder be the first manifestation of X-linked adenoleukodystrophy? A case report

Abstract Background: X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disease which causes demyelination of the white matter of the brain. The symptoms include mental impairment, progressive paresis, impaired motor coordination, and epileptic seizures. Diagnosis is established mainly by genetic testing. Currently, the recommended treatment is haematopoietic stem cell transplantation (HSCT). Goal: The aim of the study was to present the case of a patient suffering from X-ALD, who developed symptoms of bipolar disorder in the initial phase of the disease prior to the onset of characteristic neurological symptoms. Case presentation: In 2015, a 33-year-old patient was admitted to a psychiatric department due to aggressive behaviour he showed towards his wife and other family members. He had been treated for a depressive episode in 2005, and for a manic episode without psychotic symptoms earlier in 2015. During the successive psychiatric hospitalizations, in addition to psychopathological symptoms, the patient had been observed to have neurological symptoms, which included progressive paraparesis and ataxia. In 2018, based on imaging and genetic tests, the patient was diagnosed with X-ALD. The patient’s condition gradually deteriorated; with time, he was unable to move on his own. During a hospital stay in 2019, he was transferred to an internal medicine department due to a progressive urinary tract infection, which, however, could not be controlled, and the patient died. Conclusions: 1. X-ALD is a rare metabolic illness. In the early stages of the disease, various psychopathological symptoms, including affective disorders, are observed. 2. Early initiation of adequate treatment increases the chances of extending the patient’s life. 3. In the present case, the patient did not die due to the underlying disease, but due to causes typical of bed-bound patients, i.e. complications of progressing infection.

Expression of TSLC1 in patients with HAM/TSP.

Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is chronic myelopathy characterized by slowly progressive spastic paraparesis and urinary dysfunction. A few biomarkers in the cerebrospinal fluid are known to be related to disease activity, but no biomarker has been reported in peripheral blood. This study aims to explore the expression level of the adhesion molecule during the expression level of the adhesion molecule among HAM/TSP disease activity. In lymphocyte function-associated antigen 1 and DNAX accessory molecule 1, no variation in expression levels specific to HTLV-1 infection was observed in CD4-positive T cells; however, TSLC1 expression was higher in HAM patients than in asymptomatic carriers and non-infected persons. TSLC1 tended to be higher in patients whose symptoms were worsening. On the contrary, the expression level of TSLC1 in CD8-positive T cells was lower in HAM patients than in asymptomatic carriers, and this tendency was stronger in patients whose symptoms had deteriorated. No significant correlation was found between TSLC1 and either of the transcription factors Tax or HBZ in any T cell group. Therefore, TSLC1 expression in CD4-positive T cells might be a useful biomarker of HAM/TSP disease activity.

AGGRESSIVE VERTEBRAL HEMANGIOMA PRESENTING IN POSTPARTUM PERIOD: A CASE REPORT

Pregnancy is a known risk factor for hemangiomas to become aggressive and symptomatic. This is mainly because of the hormonal and hemodynamic changes and also due to increased intra-abdominal pressure. Vertebral hemangiomas usually become symptomatic in the third trimester. We present a rare case in which a young 20-year-old woman who remained asymptomatic during her pregnancy and presented with progressive paraparesis 5 days after vaginal delivery. Magnetic resonance imaging was done which revealed a vertebral hemangioma also involving the posterior elements, with soft-tissue extension in posterior epidural space. Surgery was done with removal of the soft tissue which was purely extradural in location. Histopathology revealed cavernous hemangioma.

Clinical characteristics of Taiwanese patients with Hereditary spastic paraplegia type 5.

ObjectivesTo investigate the clinical, electrophysiological, neuroimaging characteristics and genetic features of SPG5 in Taiwan.MethodsMutational analysis of the coding regions of CYP7B1 was performed by utilizing targeted resequencing analysis of the 187 unrelated Taiwanese HSP patients. The diagnosis of SPG5 was ascertained by the presence of biallelic CYP7B1 mutations. The SPG5 patients received clinical, electrophysiological, and neuroimaging evaluations. Disease severity was assessed by using the Spastic Paraplegia Rating Scale (SPRS) and the disability score. Two microsatellite markers as well as 18 single‐nucleotide polymorphism (SNP) markers flanking CYP7B1 were genotyped to assess the founder effect of the CYP7B1 p.R112* mutation.ResultsNineteen SPG5 patients from 17 families were identified. They typically presented an insidious onset progressive spastic paraparesis with proprioception involvement beginning at age 8 to 40 years. Their MRIs often showed white matter abnormalities in bilateral occipito‐parietal regions, spinal cord atrophy, and mild cerebellar atrophy. Six different mutations in CYP7B1 were recognized, including three novel ones (p.N131Ifs*4, p.A295V, and p.L439R). CYP7B1 p.R112* was the most common mutation and present in 88.2% of the 17 SPG5 pedigrees. The patients with homozygous CYP7B1 p.R112* mutations had a milder clinical severity. Detailed haplotype analyses demonstrated a shared haplotype in the 25 individuals carrying at least one single allele of CYP7B1 p.R112*, suggesting a founder effect.InterpretationThis study delineates the distinct clinical and genetic features of SPG5 in Taiwan and provides useful information for the diagnosis and management of SPG5, especially in patients of Chinese descent.

Neurorehatilitation and complex therapeutical approaches in a patient with spinal multiple sclerosis

The purely spinal form of multiple sclerosis, presenting as a progressive spastic paraparesis, hemiparesis, or, spastic monoparesis of a leg with varying degrees of posterior column involvement, is a special source of diagnostic difficulty. We present the case of a 47 years old patient, with no personal pathological antecedents, admitted in our department through the emergency ward for motor deficit of the lower limbs, sympthomatology that had an acute onset the day before admittance. The neurologic examination revealed: orthostatism and gait not possible, spastic paraparesis – 3/5 MRC (medical research council), deep tendon reflexes were found to be hyperactive on both lower extremities, bilateral plantar extension, a dermatomic level of sensory disturbance at T10 – T11 vertebral segment and urinary retention. The cerebral MRI revealed no pathological findings. The lumbar punction revealed oligoclonal bands and the spine MRI outlined multiple focal images with demyelination aspect reaching the cervical and dorsal level of the medullary cord. The patient had two more relapses each at approximately 6 months, and this allowed us to establish the final diagnostic: Pure spinal multiple sclerosis. Key words: spinal multiple sclerosis, paraparesis, neurorehabilitation,

Spinal intramedullary epidermoid cysts: Three case presentations and literature review.

Background:True intramedullary epidermoid cysts (IECs) not associated with congenital anomalies or previous spinal procedures are extremely rare. In a review of the literature since 1992, only 29 such cases have been reported. Here, we add three new cases in this category.Case Description:Three adults presented with spastic paraparesis attributed to thoracic IECs. Gross total microsurgical removal was achieved in two cases, while one case was a partial resection due to capsular adherence to the cord. In all three cases, patients sustained complete recoveries of neurological function and remained symptom free for an average of 5 years follow-up.Conclusion:IECs are rare lesions; here, the three located in the thoracic spine, contributed to slow, progressive spastic paraparesis with/without incontinence, and resolved following total (2 patients) and partial (1 patient) resection.

Combined partial lateral corpectomy and pediculectomy in a Dachshund

A 5-year-old 8. 2-kg Dachshund was presented with progressive paraparesis and ataxia of several weeks’ duration and signs of pain persisting despite conservative treatment consisting of (cage) rest and analgesic...

Spinal dural arteriovenous fistula: a misdiagnosed and treatable cause of non-compressive myelopathy

A 50-year-old woman, previously healthy, presented with a 9-month history of progressive paraparesis with sphincter disturbances and hypoesthesia in lower legs. Several weeks before admission, her paraplegia and urinary dysfunction rapidly deteriorated. She was unsuccessfully treated 4 months prior with analgesics and corticosteroids drugs. Spinal magnetic resonance imaging (MRI) (A) showed central myelopathy of the conus medullaris with posterior dilated perimedullary veins (arrows), suggestive of a spinal vascular malformation. Selective spinal angiogram of the right T11 segmental artery (B) demonstrated a right sided fistulous connection between the segmental artery and the perimedullary venous plexus (dotted oval circle). A thoracic T10-T12 laminectomy and durotomy was performed, which showed engorged venous vessels on the posterior surface of the spinal cord (C). An arterialized draining vein was identified arising from the inner aspect of the dura of the right T11 nerve root. This vessel was completely clipped (arrow). Postoperatively, neurologic examination remained unchanged at the time of discharge. However, at 4-month follow-up, she was able to ambulate with a walker. Postoperative angiography performed 2 months later showed total disappearance of the vascular fistula (D).

Epidural anesthesia masking pain from spinal epidural hematoma

Spinal epidural hematoma (SEDH) without pain due to epidural anesthesia is a rare but serious complication. We report the successful treatment of a patient with progressive paraparesis after the development of SEDH during the administration of epidural anesthesia. An 84-year-old woman underwent laparoscopy with epidural anesthesia. To prevent deep vein thrombosis, the delivery of low molecular weight heparin was started 16.5 hours after laparoscopy. On the 1st postoperative day she reported painless leg paralysis and on the 2nd day she manifested severe paraplegia and urinary retention. Magnetic resonance imaging revealed an SEDH compressing the spinal cord at the Th12 - L1 and we performed emergent laminectomy. After the laminectomy her symptoms disappeared completely. SEDH is a rare but serious complication related to epidural anesthesia. As its excessive analgesic effect may elicit motor weakness and mask pain, SEDH must be ruled out in patients with paraparesis with continuous epidural anesthesia.

Mutational Spectrum of Spast (Spg4) and Atl1 (Spg3a) Genes In Russian Patients With Hereditary Spastic Paraplegia

Hereditary spastic paraplegia (HSP) comprises a heterogeneous group of neurodegenerative disorders, it share common symptom - of progressive lower spastic paraparesis. The most common autosomal dominant (AD) forms of HSP are SPG4 (SPAST gene) and SPG3 (ATL1 gene). In the current research we investigated for the first time the distribution of pathogenic mutations in SPAST and ATL1 genes within a large cohort of Russian HSP patients (122 probands; 69 famillial cases). We determined the frequencies of genetic abnormalities using Sanger sequencing, multiplex ligation-dependent probe amplification (MLPA), and Next Generation Sequencing (NGS) of targeted gene panels. As a result, SPG4 was diagnosed in 30.3% (37/122) of HSP cases, where the familial cases represented 37.7% (26/69) of SPG4. In total 31 pathogenic and likely pathogenic variants were detected in SPAST, with 14 new mutations. Among all detected SPAST variants, 29% were gross deletions and duplications. The proportion of SPG3 variants in Russian cohort was 8.2% (10/122) that were all familial cases. All 10 detected ATL1 mutations were missense substitutions, most of which were in the mutational hot spots of 4, 7, 8, 12 exons, with 2 novel mutations. This work will be helpful for the populational genetics of HSP understanding.

Case report: SPG11 hereditary spastic paraplegia with no thinning in the corpus callosum

Autosomal recessive hereditary spastic paraplegia with thin corpus callosum (ARHSPTCC) is a type of complicated hereditary spastic paraparesis, which caused by SPG11 mutations. SPG11 ARHSPTCC is characterized by the thin corpus callosum, progressive spastic paraparesis, and cognitive decline. In addition to other neurological symptoms such as seizures, cerebellar ataxia, speech and swallowing problems, extrapyramidal signs and skeletal deformities may also occur. The neuroradiological findings typically include thinning of the anterior corpus callosum (TCC), periventricular white matter changes and cortical atrophy. We herein report two Saudi Arabian siblings with genetically confirmed SPG11 ARHSP with no thinning in the corpus callosum. The brothers, aged 20 and 34 years, presented with a typical presentation of SPG11 ARHSP; including bilateral progressive lower limbs weakness, spasticity, and unsteadiness. Brain MRI of the younger sibling showed mild periventricular deep white matter T2 hyperintensity changes, mainly at the parietooccipital lobe. And diffuse cortical gray and white matter atrophic changes. While the older sibling had a normal brain MRI. Autosomal recessive hereditary spastic paraparesis with SPG 11 mutations may rarely have absence of corpus collosal involvement. This may perhaps be due to the timing of the MRI or other genetic features. Longitudinal MRI studies are required to determine if the collosal involvement develops later in the disease course.

High-frequency spinal cord stimulation (HF-SCS) improves motor as well as cognitive performances in a case of hereditary spastic paraplegia

We report a case of complicated hereditary spastic paraplegia treated with spinal cord stimulation. A 40 year-old man with slowly progressive spastic paraparesis,moderate cognitive deficit,was submitted to the implant of a cervical epidural electrode for the neuromodulation of spasticity.After trial with 80Hz stimulation,patient referred some motor improvement,but unpleasant paraesthesiae.We tested 10000Hz stimulation that does not induce paraesthesiae.Before and after15days:neurological status (Ashworth scale,Albert scale,VAS,Hamilton depression scale),gait analysis,quality of life (SF-36),neuropsychological evaluation,brain single photon emission computer tomography(SPECT)with HMPAO to assess regional cerebral perfusion. The patient during HF-SCS referred a better endurance, walk and could lay down and stand up faster, with more fluent language,and a better vescico-sphincterial control.Ashworth scale decreased from 20 to 17,Albert scale improved from 143 to 149.Increase of gait speed by 26%, increased step length (by 40.8%),decreased double support(by 43.5%).Kinematic analysis showed hip and knee decreased flexion.The Hamilton scale showed no signs of depression.The SF-36 improved,particularly the Physical Health Index (from 33 to 43).The neuropsychological studies showed an improvement of dysarthria,marked improvement in the test of word reading,in the visual comprehension of names,denomination of names,constructive praxia tests along with a mild improvement in MMSE and short term memory.The brain SPECT during SCS showed an increased perfusion in the right frontal and temporal cortex and in the left fronto-temporal cortex. The patient was submitted to chronic stimulation.The motor and cognitive improvement was confirmed at the 12 months follow up visit. HF-SCS improves motor as well as language and cognitive performances without inducing disturbing paraesthesiae in a case of complicated hereditary spastic paraplegia.