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Alajouanine Syndrome


Presentation

  • At current presentation, he was unable to stand, even with support. Bilateral foot drop was present. Right lower limb power was grade 3/5 in hip flexors and knee extensors and 2/5 in knee flexors, ankle plantar flexors, and hip extensors.[ncbi.nlm.nih.gov]
  • […] osteosclerosis with abnormalities of the nervous system and meninges Specialty Neurology Foix–Alajouanine syndrome, also called subacute ascending necrotizing myelitis, [1] is a disease caused by an arteriovenous malformation of the spinal cord. [2] The patients present[en.wikipedia.org]
  • The skin lesions of Cobb syndrome typically are present as port wine stains or angiomas, but reports exist of angiokeratomas, angiolipomas, and lymphangioma circumscriptum.[dixion-healthcare.com]
  • Owing to the rarity of this syndrome and the varied clinical presentations, multidisciplinary teams may best serve the patient.[emedicine.medscape.com]
Unable to Stand
  • At current presentation, he was unable to stand, even with support. Bilateral foot drop was present. Right lower limb power was grade 3/5 in hip flexors and knee extensors and 2/5 in knee flexors, ankle plantar flexors, and hip extensors.[ncbi.nlm.nih.gov]
Leg Pain
  • Case Report A 54-year-old male presented with gradually progressive history of walking difficulty, weakness, and decreased sensation in lower limbs, diffuse gluteal, and leg pain and bowel and bladder incontinence of 3 years duration.[ncbi.nlm.nih.gov]
Buttock Pain
  • This affliction of the lower part of the cord leads to the commonly observed symptoms of dysesthesias, non-dermatomal sensory loss, walking difficulty, wasting, weakness of lower limbs, buttock pain, and sphincter dysfunction.[ 1, 2, 3 ] Often this condition[ncbi.nlm.nih.gov]
Incontinence
  • Case Report A 54-year-old male presented with gradually progressive history of walking difficulty, weakness, and decreased sensation in lower limbs, diffuse gluteal, and leg pain and bowel and bladder incontinence of 3 years duration.[ncbi.nlm.nih.gov]
  • […] than ocular Anatomical cases Clinical cases 66 56 55 38 22 17 10 9 8 150 96 82 52 42 35 20 14 19 Dysarthria Hyperactive deep tendon reflexes, spasticity Dysphagia Babinski sign Jaw-jerk Explosive crying, exaggerated emotional responses Glabellar reflex Incontinence[slideheaven.com]
Tremor
  • CN 3, 4) Weber's syndrome ventral peduncle, PCA Benedikt syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Nothnagel's syndrome Claude's syndrome Other Alternating hemiplegia Cerebellum lateral ( Dysmetria Dysdiadochokinesia Intention tremor[en.wikipedia.org]
  • Absolute incidence o f cerebellar signs Anatomical cases Ataxia absent Dysmetria absent Romberg sign absent Adiadokokinesis absent Tremor 214 cases 13 12 10 11 6 2 4 1 1 Clinical cases 7 2 16 13 8 2 11 9 130 99 41 4 67 73 41 29 35 8 20 8 15 9 1 detected[slideheaven.com]
Dysmetria
  • […] ophthalmoplegia One and a half syndrome Midbrain (CN 3, 4) Weber's syndrome ventral peduncle, PCA Benedikt syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Nothnagel's syndrome Claude's syndrome Other Alternating hemiplegia Cerebellum lateral ( Dysmetria[en.wikipedia.org]
  • Absolute incidence o f cerebellar signs Anatomical cases Ataxia absent Dysmetria absent Romberg sign absent Adiadokokinesis absent Tremor 214 cases 13 12 10 11 6 2 4 1 1 Clinical cases 7 2 16 13 8 2 11 9 130 99 41 4 67 73 41 29 35 8 20 8 15 9 1 detected[slideheaven.com]
Dystonia
  • […] syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Nothnagel's syndrome Claude's syndrome Other Alternating hemiplegia Cerebellum lateral ( Dysmetria Dysdiadochokinesia Intention tremor ) medial ( Cerebellar ataxia ) Basal ganglia Chorea Dystonia[en.wikipedia.org]
  • (In Greek). [10] ANASTASOPOULOSG., ROUTSONIS K., COCCHINI D., KONSTm G.: Oculo-facio-cervical dystonia. (Progressive Supranuclear palsy). Proc. 4th Panhellenic Cong. Greek Neurologists and Psychiatrists, Salonica 1968, pp. 205-208.[slideheaven.com]
Neglect
  • Dysdiadochokinesia Intention tremor ) medial ( Cerebellar ataxia ) Basal ganglia Chorea Dystonia Parkinson's disease Cortex ACA syndrome MCA syndrome PCA syndrome frontal lobe : Expressive aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect[en.wikipedia.org]
Expressive Aphasia
  • aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect Gerstmann syndrome Astereognosis occipital lobe : Bálint's syndrome Cortical blindness Pure alexia temporal lobe : Cortical deafness Prosopagnosia Thalamus Thalamic syndrome Other[en.wikipedia.org]

Treatment

  • Table 3 Summary of cases of filum terminale AV fistulas Both surgical and endovascular treatment options are mentioned in literature.[ncbi.nlm.nih.gov]
  • Surgical treatment can be tried in some cases. If surgical intervention is contraindicated, corticosteroids may be used. The condition is named after Charles Foix and Théophile Alajouanine.[en.wikipedia.org]
  • A possible complication if treatment is delayed is Foix-Alajouanine disease or subacute necrotic myelopathy due to thrombosis in the spinal angioma. Cutaneous lesions may be distributed anywhere in the dermatome, from midline back to abdomen.[dixion-healthcare.com]
  • Treatment with L-dopa, Can. J. Ophthalmol., 7, 368-374, 1972. [139] REn -Iz J.J., KOLODNYE.H., RICHARDSONE.P., jr.: Corticodentatonigral degeneration with neuronal achromasia. Arch.[slideheaven.com]

Prognosis

  • Prognosis likely depends on the severity of the spinal pathology and the timeliness of diagnosis and intervention. Patients who present with rapidly progressing neurological deficits may have a worse prognosis. Early diagnosis is critical.[emedicine.medscape.com]
  • Factors determining prognosis are said to be duration of illness, sphincter involvement at presentation and location of fistula however, there is no consensus on any of these.[ 1 ] Before our case, only one such has been published in Indian literature[ncbi.nlm.nih.gov]

Etiology

  • No classification has been possible in the face of ignorance of etiologic factors; pathogenesis has been equally uncertain, and the interpretation of pathologic material has produced no uniformity of opinion regarding the nature of the morbid changes.[jamanetwork.com]
  • Etiology It has been suggested that Cobb syndrome, Sturge-Weber syndrome, and PHACE syndrome (posterior fossa, hemangioma or other vascular birthmark present either on the outside or inside, arterial defect in the head and or neck area, cardiac problems[emedicine.medscape.com]
  • Drugs are used in neurological recovery depending on the etiological classification of FCMS.[en.wikipedia.org]

Epidemiology

  • The timing of this mutation during development may determine which syndrome develops. [4] Epidemiology Frequency Cobb syndrome is rare; fewer than 100 cases have been reported. Race Cobb syndrome is reported more commonly in whites.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Spinal intradural arteriovenous fistulas acquired in late adulthood: Absent spinal venous drainage in pathogenesis and pathophysiology. Report of two cases. J Neurosurg Spine. 2005; 3 :488–94. [ PubMed ] [ Google Scholar ][ncbi.nlm.nih.gov]
  • Report of a case and study on the pathophysiology of the eye movement disturbance. Ciin. Neurol. (Tokyo), 15, 535-540, 1975. (Abstract in English). [86] KHUNADORN N., KALUMPAH R., S, JP -TARA K.: Blepharospasm in progressive supranuclear palsy.[slideheaven.com]

Prevention

  • The spinal vascular lesions can cause neurologic deficits, including paralysis. [2, 3] Early recognition of the association between the vascular skin lesions and associated spinal lesions may prevent or minimize neurological sequela.[emedicine.medscape.com]

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