diff --git a/notebooks/NFU1/NFU1_SPG93_individuals.ipynb b/notebooks/NFU1/NFU1_SPG93_individuals.ipynb new file mode 100644 index 00000000..c9f73b46 --- /dev/null +++ b/notebooks/NFU1/NFU1_SPG93_individuals.ipynb @@ -0,0 +1,289 @@ +{ + "cells": [ + { + "cell_type": "markdown", + "metadata": {}, + "source": [ + "# NFU1\n", + "[autosomal recessive spastic paraplegia-93 (SPG93) ](https://omim.org/entry/620938) is caused by homozygous or compound heterozygous mutation in the NFU1 gene." + ] + }, + { + "cell_type": "code", + "execution_count": 1, + "metadata": {}, + "outputs": [ + { + "name": "stdout", + "output_type": "stream", + "text": [ + "Using pyphetools version 0.9.115\n" + ] + } + ], + "source": [ + "from pyphetools.creation import TemplateImporter, Moi\n", + "from pyphetools.visualization import IndividualTable, QcVisualizer\n", + "from IPython.display import display, HTML\n", + "import pyphetools\n", + "print(f\"Using pyphetools version {pyphetools.__version__}\")" + ] + }, + { + "cell_type": "code", + "execution_count": 2, + "metadata": {}, + "outputs": [], + "source": [ + "template = \"input/NFU1_SPG93_individuals.xlsx\"\n", + "created_by = \"0000-0002-0736-9199\"" + ] + }, + { + "cell_type": "code", + "execution_count": 28, + "metadata": {}, + "outputs": [ + { + "name": "stdout", + "output_type": "stream", + "text": [ + "HPO version 2024-12-12\n", + "Created encoders for 63 fields\n", + "Importing OMIM:620938, Spastic paraplegia 93, autosomal recessive, HGNC:16287, NFU1, NM_001002755.4\n", + "We output 21 GA4GH phenopackets to the directory phenopackets\n" + ] + } + ], + "source": [ + "timporter = TemplateImporter(template=template, created_by=created_by)\n", + "individual_list, cvalidator = timporter.import_phenopackets_from_template()" + ] + }, + { + "cell_type": "code", + "execution_count": 29, + "metadata": {}, + "outputs": [ + { + "data": { + "text/html": [ + "
Errors found with 17 of 21 phenopackets.
\n", + "Level | Error category | Count |
---|---|---|
WARNING | REDUNDANT | 21 |
A total of 21 issues were fixed and no individual was removed from the cohort.
" + ], + "text/plain": [ + "Individual | Disease | Genotype | Phenotypic features |
---|---|---|---|
F1-II:1 (FEMALE; P17Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.721G>C (homozygous) | Developmental regression (HP:0002376): onset ; Strabismus (HP:0000486): onset ; External ophthalmoplegia (HP:0000544): onset ; Brisk reflexes (HP:0001348): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Global developmental delay (HP:0001263): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: Ptosis (HP:0000508): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P9M14D)): Spastic paraplegia (HP:0001258): onset age {\n", + " iso8601duration: \"P9M14D\"\n", + "}\n", + " |
F1-II:2 (FEMALE; P10Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.721G>C (homozygous) | Delayed ability to walk (HP:0031936): onset ; Strabismus (HP:0000486): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Global developmental delay (HP:0001263): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Developmental regression (HP:0002376): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: Ptosis (HP:0000508): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P6M)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P6M\"\n", + "}\n", + " |
F2-II:1 (MALE; P25Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) |
| Developmental regression (HP:0002376): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Clonus (HP:0002169): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Global developmental delay (HP:0001263): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P1Y\"\n", + "}\n", + " |
F3-II:1 (FEMALE; P12Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Global developmental delay (HP:0001263): onset ; Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Developmental regression (HP:0002376): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P1Y\"\n", + "}\n", + " |
F3-II:2 (MALE; P9Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Global developmental delay (HP:0001263): onset ; Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Developmental regression (HP:0002376): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P1Y\"\n", + "}\n", + " |
F4-II:4 (FEMALE; P2Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Developmental regression (HP:0002376): onset ; Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; Ankle clonus (HP:0011448): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P2Y9M)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P2Y9M\"\n", + "}\n", + " |
F4-II:5 (FEMALE; P6Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; Ankle clonus (HP:0011448): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Global developmental delay (HP:0001263): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Developmental regression (HP:0002376): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P11M)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P11M\"\n", + "}\n", + " |
F4-II:6 (FEMALE; P2Y6M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Global developmental delay (HP:0001263): onset ; Developmental regression (HP:0002376): onset ; Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; Ankle clonus (HP:0011448): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P1Y\"\n", + "}\n", + " |
F5-II:1 (FEMALE; P6M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Developmental regression (HP:0002376): onset ; Brisk reflexes (HP:0001348): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Hypertonia (HP:0001276): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P5M)): Fever (HP:0001945): onset age {\n", + " iso8601duration: \"P5M\"\n", + "}\n", + "; Hypotonia (HP:0001252): onset age {\n", + " iso8601duration: \"P5M\"\n", + "}\n", + "; Respiratory failure (HP:0002878): onset age {\n", + " iso8601duration: \"P5M\"\n", + "}\n", + "; Acidosis (HP:0001941): onset age {\n", + " iso8601duration: \"P5M\"\n", + "}\n", + " |
F5-II:2 (MALE; P4Y7M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Developmental regression (HP:0002376): onset ; Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y2M)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P1Y2M\"\n", + "}\n", + " |
F6-II:2 (MALE; P3Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Lactic acidosis (HP:0003128): onset ; Developmental regression (HP:0002376): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; Ankle clonus (HP:0011448): onset ; Babinski sign (HP:0003487): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Strabismus (HP:0000486): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: Ptosis (HP:0000508): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y)): Gait disturbance (HP:0001288): onset age {\n", + " iso8601duration: \"P1Y\"\n", + "}\n", + "; Progressive spastic paraplegia (HP:0007020): onset age {\n", + " iso8601duration: \"P1Y\"\n", + "}\n", + " |
F6-II:3 (MALE; P2Y3M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.362T>C (homozygous) | Lactic acidosis (HP:0003128): onset ; Developmental regression (HP:0002376): onset ; Brisk reflexes (HP:0001348): onset ; Knee clonus (HP:0011449): onset ; Ankle clonus (HP:0011448): onset ; Babinski sign (HP:0003487): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Strabismus (HP:0000486): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: Ptosis (HP:0000508): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P10M)): Lower limb spasticity (HP:0002061): onset age {\n", + " iso8601duration: \"P10M\"\n", + "}\n", + " |
F7-II:1 (MALE; P10Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.295C>G (homozygous) | Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; Talipes equinovarus (HP:0001762): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Developmental regression (HP:0002376): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P3M)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P3M\"\n", + "}\n", + " |
F7-II:2 (MALE; P6Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.295C>G (homozygous) | Delayed ability to walk (HP:0031936): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; Talipes equinovarus (HP:0001762): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Developmental regression (HP:0002376): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P3M)): Paraplegia (HP:0010550): onset age {\n", + " iso8601duration: \"P3M\"\n", + "}\n", + " |
F8-II:5 (MALE; P7Y7M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.263T>C (homozygous) | Developmental regression (HP:0002376): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Strabismus (HP:0000486): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: Ptosis (HP:0000508): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y6M)): Frequent falls (HP:0002359): onset age {\n", + " iso8601duration: \"P1Y6M\"\n", + "}\n", + " |
F8-II:6 (MALE; P6Y6M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.263T>C (homozygous) | Developmental regression (HP:0002376): onset ; Hypertonia (HP:0001276): onset ; Brisk reflexes (HP:0001348): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Strabismus (HP:0000486): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: Ptosis (HP:0000508): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y6M)): Frequent falls (HP:0002359): onset age {\n", + " iso8601duration: \"P1Y6M\"\n", + "}\n", + " |
F9-II:1 (MALE; P12Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) |
| Developmental regression (HP:0002376): onset ; Ptosis (HP:0000508): onset ; Peripheral neuropathy (HP:0009830): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Strabismus (HP:0000486): onset ; excluded: Nystagmus (HP:0000639): onset ; excluded: Optic atrophy (HP:0000648): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset |
F10-II:1 (MALE; P12M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) |
| Global developmental delay (HP:0001263): onset ; Developmental regression (HP:0002376): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Peripheral demyelination (HP:0011096): onset ; Enlarged cisterna magna (HP:0002280): onset ; Encephalopathy (HP:0001298): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Hypertonia (HP:0001276): onset ; excluded: Brisk reflexes (HP:0001348): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P6M)): Hypotonia (HP:0001252): onset age {\n", + " iso8601duration: \"P6M\"\n", + "}\n", + "; Generalized-onset seizure (HP:0002197): onset age {\n", + " iso8601duration: \"P6M\"\n", + "}\n", + "; Status epilepticus (HP:0002133): onset age {\n", + " iso8601duration: \"P6M\"\n", + "}\n", + " |
F10-II:2 (FEMALE; P2Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) |
| Global developmental delay (HP:0001263): onset ; Developmental regression (HP:0002376): onset ; Nystagmus (HP:0000639): onset ; Optic atrophy (HP:0000648): onset ; Reduced cerebral white matter volume (HP:0034295): onset ; Hypoplasia of the corpus callosum (HP:0002079): onset ; Enlarged cisterna magna (HP:0002280): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Strabismus (HP:0000486): onset ; excluded: External ophthalmoplegia (HP:0000544): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P3M)): Hypotonia (HP:0001252): onset age {\n", + " iso8601duration: \"P3M\"\n", + "}\n", + " TimeElement(element=Age(iso8601duration=P2Y)): Lower limb spasticity (HP:0002061): onset age {\n", + " iso8601duration: \"P2Y\"\n", + "}\n", + " |
Clinical data (MALE; P30Y) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) |
| Global developmental delay (HP:0001263): onset ; Spastic paraplegia (HP:0001258): onset ; Brisk reflexes (HP:0001348): onset ; Peripheral neuropathy (HP:0009830): onset ; Scoliosis (HP:0002650): onset ; Hypoesthesia (HP:0033748): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Knee clonus (HP:0011449): onset ; excluded: Ankle clonus (HP:0011448): onset ; excluded: Babinski sign (HP:0003487): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset TimeElement(element=Age(iso8601duration=P1Y6M)): Developmental regression (HP:0002376): onset age {\n", + " iso8601duration: \"P1Y6M\"\n", + "}\n", + "; Spastic tetraplegia (HP:0002510): onset age {\n", + " iso8601duration: \"P1Y6M\"\n", + "}\n", + " TimeElement(element=Age(iso8601duration=P30Y)): Corpus callosum atrophy (HP:0007371): onset age {\n", + " iso8601duration: \"P30Y\"\n", + "}\n", + "; Reduced brain N-acetyl aspartate level by MRS (HP:0012708): onset age {\n", + " iso8601duration: \"P30Y\"\n", + "}\n", + "; Elevated brain choline level by MRS (HP:0012706): onset age {\n", + " iso8601duration: \"P30Y\"\n", + "}\n", + "; Elevated brain lactate level by MRS (HP:0012707): onset age {\n", + " iso8601duration: \"P30Y\"\n", + "}\n", + " |
patient (FEMALE; P3Y6M) | Spastic paraplegia 93, autosomal recessive (OMIM:620938) | NM_001002755.4:c.565G>A (homozygous) | Global developmental delay (HP:0001263): onset ; Developmental regression (HP:0002376): onset ; excluded: Gait ataxia (HP:0002066): onset ; excluded: Lactic acidosis (HP:0003128): onset ; excluded: Reduced cerebral white matter volume (HP:0034295): onset ; excluded: Hypoplasia of the corpus callosum (HP:0002079): onset ; excluded: Peripheral demyelination (HP:0011096): onset ; excluded: Enlarged cisterna magna (HP:0002280): onset ; excluded: Encephalopathy (HP:0001298): onset ; excluded: Talipes equinovarus (HP:0001762): onset ; excluded: Peripheral neuropathy (HP:0009830): onset TimeElement(element=Age(iso8601duration=P1Y11M)): Loss of ambulation (HP:0002505): onset age {\n", + " iso8601duration: \"P1Y11M\"\n", + "}\n", + " |